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DISEASES 


NERVOUS  SYSTEM 


A  TEXT-BOOK  OF 


NEUROLOGY  AND  PSYCHIATEY 


BY 

SMITH  ELY  JELLIFFE,  M.D.,  Ph.D. 

FORMERLY,    FROFESSOR    OF    PSYCHIATRY,    FORDHAM    UNrVERSITY,    NEW    YORK,    AND    FORMERLY 

ADJUNCT    PROFESSOR    OF    DISEASES    OF   THE    MIND    AND    NERVOUS    SYSTEM, 

NEW  YORK    POST-GRADUATE   MEDICAL   SCHOOL  AND   HOSPITAL 


WILLIAM  A.  WHITE,  M.D. 

SUPERINTENDENT    OF   ST.    ELIZABETH'S   HOSPITAL,  WASHINGTON,  D.  C. ;     PROFESSOR  OF  NERVOUS 

AND  MENTAL  DISEASES,   GEORGETOWN  UNIVERSITY;     PROFESSOR   OF  NERVOUS 

AND   MENTAL   DISEASES,   GEORGE   WASHINGTON   UNIVERSITY,   AND 

LECTURER    ON  PSYCHIATRY,  U.  S.  ARMY  AND  U.  S.  NAVY 

MEDICAL  SCHOOLS 


THIRD  EDITION,  REVISED,  REWRITTEN  AND  ENLARGED 


ILLUSTRATED  WITH  470  ENGRAVINGS  AND  12  PLATES 


LEA  &  FEBIGER 

PHILADELPHIA    AND    NEW    YORK 


Copyright 

LEA  &  FEBIGER 

1919 


TO 

HELENA  LEEMING  JELLIFFE 

WHOSE   LOFTY   PURPOSE, 

IDEAL   STRIVING,  AND   NEVER-FAILING   COOPERATION, 

HAVE   BEEN   A   CONSTANT   STIMULUS  TO   PROGRESSIVE   ENDEAVOR, 

THIS  BOOK   IS   DEDICATED 

AS  A  TOKEN  OF   LOVE   AND  ESTEEM 


PREFACE. 


The  present  edition  has  been  remodelled  and  largely  rewritten.  New 
data  in  the  fields  of  vegetative  neurology  and  of  the  endocrinopathies 
have  accumulated  in  large  volume  in  the  past  two  years;  we  have 
tried  to  do  justice  to  these  acquisitions  in  knowledge  by  a  careful 
selection  of  material  which  will  best  serve  the  practical  purposes  of  the 
student  and  practitioner;  at  the  same  time  we  have  so  endeavored  to 
arrange  these  chapters  that  the  student  may  see  the  trend  of  the 
development  in  this  rapidly  enlarging  field  which  underlies  a  dynamic 
understanding  of  all  physiological  and  pathological  processes  going  on 
not  only  within  the  nervous  structures  themselves,  but  in  all  of  the 
organs  of  the  body. 

The  chapters  on  sensorimotor  neurology  have  been  carefully  revised 
to  accord  with  many  new  observations  which  the  Great  War  has 
afforded,  and  the  many  confirmations  of  existing  hypotheses  relative 
to  this  large  sphere  in  nervous  diseases. 

In  the  third  part  the  enlargements  have  been  mostly  along  the  lines 
of  an  interpretative  presentation  of  the  psychoses,  with  an  increased 
emphasis  on  a  description  of  the  mechanisms  involved  rather  than 
upon  the  grouping  of  certain  s^tinptom-complexes  under  conventional 
captions. 

Throughout  the  book  we  have  continued  the  endeavor,  projected  in 
our  original  plan,  to  build  up  a  conception  of  that  interrelation  and 
interdependence  between  the  several  divisions  of  the  subject  which  is 
their  characteristic  in  nature,  but  which  for  the  practical  purposes  of 
presentation  we  have  artificially  separated  under  three  headings. 

S.  E.  J. 
W.  A.  W. 
New  York  and  Washington,  1919. 


CONTENTS. 


Introduction:    On  Some  Principles  Underlying  a  Classification  of  Diseases 
of  the  Nervous  System 17 


CHAPTER  I. 

Methods  of  Examination  of  the  Nervous  System. 

Questionnaire 22 

Family  History 24 

History  of  patient 27 

Adult  Diseases 29 

Present  Illness 31 

General  Observations 32 

Physical  Examination 32 

Vegetative  System  Examination 33,  38 

Endocrine  Status 40 

Sensorimotor  Examination 42 

Cranial  Nerves 42 

Head  and  Neck 75 

Upper  Extremities 77 

The  Muscles  of  the  Trunk     . 88 

The  Lower  Extremities 89 

Reflexes  of  Upper  Extremities 91 

Reflexes  of  the  Lower  Extremities 92 

Types  of  Vegetative  Reflexes 95 

Tremors 97 

Examination  of  Sensory  Nervous  System 98 

Epicritic  Sensibility 99 

Protopathic  Sensibility 101 

Deep  Sensibility 101 

Vasomotor  and  Tropliic  Disturbances 103 

Scheme  for  Testing  Sensibihty 103 

Status  Corporis 105 

Mental  Examination  Methods 110 

Psychoanalysis 114 

The  Complex 114 

Technic 117 

(ix) 


viii  CONTENTS 

PART  I. 
THE  PHYSICOCHEMICAL  SYSTEMS. 

THE  NEUROLOGY  OF  METABOLISM. 


CHAPTER  II. 


Vegetative  or  Visceral  Neurolooy. 

The  Autonomic  and  Sympathetic  Nervous  Systems — ^The  Endocrine  System  121 

Vegetative  Receptors 124 

Spinal  Synapses 125 

Midbrain  Synapses  of  the  Vegetative  Pathways 131 

Cortical  Stations  of  the  Vegetative  Pathways 132 

Sympathetic  and  Autonomic  Divisions 134 

Special  Pathology 138 

Eye  Sympathetic 138 

Glaucoma 141 

Tear  Glands 141 

Mucous  and  SaUvary  Glands 141 

Neck  Sympathetic 143 

Cervical  Sympathetic 143 

Gastro-intestinal  Syndromes 145 

Esophagus 147 

Stomach  and  Intestines 148 

Rectum 151 

Geni to-urinary  System 153 

Respiratory  Apparatus 157 

Vascular  Apparatus 159 

Heart 159 

Bloodvessels:    Vasomotor  Neuroses .'      .  160 

Tonic  Hyperemias 161 

Erythromelalgia 161 

Spastic  Anemic  Group 162 

Raynaud's  Disease 162 

Intermittent  Claudication 164 

Ophthalmic  Migraine      .      .  .      .      .      .      .      .      .      .      .  165 

Periodic   Palsies:     Ophthalmoplegic,    Facioplegic,    Hemiplegic 

Migraine 182 

Vasomotor  IrritabiUty  Group 184 

Angioneurotic  Edema  ....  184 

Vegetative  Skin  Syndromes 191 

Scleroderma 191 

Multiple  Neurotic  Gangrene  of  the  Skin 193 

Sweat  Secretory  Mechanisms 193 

Pilomotor  System 194 

Bony  Syndromes 195 

Blood  Syndromes 196 

Chlorosis 196 

EosinophiUa 196 


CONTENTS  ix 

CHAPTER  III. 

The  Endocrinopathies. 

Internal  Secretions.     General  Considerations 197 

The  Thyreopathies;  Th^Teoses— The  Thyroid      .           204 

HypothjTeoses 205 

Myxedema 205 

Cretinism 211 

Sporadic  Cretinism 213 

Cretinoid  Degeneration  ....            215 

Goiter 216 

Goiter  Heart 216 

Endemic  Cretinism    ....                        216 

Endemic  Deaf-mutism 219 

Mild  and  Mixed  Hypothyroid  States 219 

Hyperthyreoses 225 

Exophthalmic  Goiter 225 

Parathyroid  Sjmdromes 234 

Tetany 234 

Diseases  of  the  Hypophysis — Pituitary 241 

Hyperpituitarism 242 

Acromegaly  and  Gigantism ....  242 

Hypopituitarism 246 

Dyspituitarism 249 

Infundibular  Syndromes 252 

Diseases  of  the  Pineal  Organ 252 

Pineal  Syndrome .      .  252 

Diseases  of  the  Suprarenal  Body                                                    2-53 

Suprarenal  SjTidromes 253 

Hypoadrenalemia 255 

Addison's  Disease 256 

Hyperadrenalemia 256 

Suprarenal  Pseudohermaphroditism 256 

Suprarenal  Virilism 257 

Disease  of  the  Gonadal  Systems                      258 

Genital  Syndromes 258 

Agenitalism,  Hypergenitalism,  Hypogenitalism 258 

Hermaphroditism ...  259 

Agenitalism:    Eunuchs 259 

Dysgenitahsm 261 

Chlorosis 261 

Dysgonadal  Syndromes 262 

Status  Thymolymphaticus .      .  263 

Diseases  of  the  Pancreas 266 

Pancreatic  Syndromes 266 

Diseases  of  the  Muscles  ...           267 

Muscle  Syndromes 267 

Myasthenia  Gravis 267 

Thomsen's  Disease — Myotonia  Congenita 269 

Myatonia  Atrophica 271 

The  Muscular  Dystrophies  or  Myopathies 271 


X  CONTENTS 

Fatty  Syndromes .  279 

Obesity .279 

Adiposis  Dolorosa 279 

Bony  and  Ligamentous  Syndromes:     Osteopathies,  Arthropathies       .      .      .  280" 

Achondroplasia         282 

Osteomalacia 283 


PART  II. 
SENSORIMOTOR  SYSTEMS. 
SENSORIMOTOR  NEUROLOGY. 


CHAPTER  IV. 

Cranial  Nerves. 


I.  Diseases  of  the  Olfactory  Tract 287 

Olfactory 287 

II.  Diseases  of  the  Visual  Apparatus 293 

Retinitis ...  296 

Optic  Nerve 297 

Diseases  of  Optic  Nerve 297 

(a)  Axial  Neuritis 298 

{b)  Interstitial  Peripheral  Neuritis 301 

(o)  Diffuse  Neuritis 303 

Disease  at  or  about  the  Chiasm 304 

Thalamus  Disease 305 

Cortex  Diseases 307 

III,  IV,  VI.  Diseases  of  the  Oculomotor  Nerves 309 

Third  Nerve  Palsies 310 

Syndromes 312 

Chronic  Progressive  Eye  Palsies 315 

Fourth  Nerve  Palsy 315 

Sixth  Nerve  Palsy 315 

V.  Diseases  of  the  Trigeminal  Nerve 322 

Motor  Part 322 

Sensory  Part 324 

Progressive  Facial  Hemiatrophy 328 

VII.  Diseases  of  the  Facial  Nerve 329 

Cortical  Palsies 329 

Pontine  Facial  Lesions 332 

Peripheral  Facial  Palsies 332 

VIII.  Diseases  of  the  Auditory  and  Vestibular  Pathways 336 

Auditory  Nerve 336 

Vestibular  Nerve 339 

Nervus  Vestibularis 340 

Vestibular  Vertigoes 342 


CONTENTS  XI 

IX.  Disorders  of  the  Taste  Apparatus 347 

Glossopalatine  and  Glossopharyngeal  Nerves 347 

X.  Diseases  of  the  Larj'ngeal  Nerves 350 

Laryngeal  Disorders 350 

XI,  XII.  Diseases  of  the  Spinal  Accessory  and  Hypoglossal  Nerves   .           .  352 

Accessorius 352 

Hypoglossus 354 

Speech  Disturbances 357 

Aphasias 361 

Motor  Aphasia 366 

Auditory  Aphasia 366 

Visual  Aphasia 367 

Writing  Disturbances 367 


CHAPTER  V. 

Affections  of  the  Peripheral  Netjbons:  Sensory  and  Motor. 

Neuralgias 369 

Special  Locahzed  Forms  of  Neuralgias 378 

Trigeminal  Neuralgia 379 

Tic  Douloureux 379 

Cervico-occipital  Neuralgia                                   , 382 

Diaphragmatic  Neuralgia .      .  383 

Brachial  Nevualgia ...            ...  384 

Intercostal  Neuralgia .            .  387 

Lumbar  Plexus  Neuralgia 387 

The  Sciaticas    ....           ...           387 

Lumbo-abdominal  Neuralgia 393 

Testicular  Neuralgia      ...            -          .                  .  393 

Crural  Neuralgia 393 

Femoral  Neuralgia         394 

Obturator  Neuralgia 394 

Neuralgias  of  the  Pudendal  Plexus 394 

Neuralgias  of  the  Coccygeal  Plexus 395 

Herpes  Zoster;  Shingles;  Zona.     RadicvdogangUonic  Syndrome  (Acute  Pos- 
terior Poliomyelitis) 395 

Radiculitis 397 

Neuritis 401 

Polyneuritis,  Multiple  Neuritis     ...                 402 

Alcoholic  Multiple  Neuritis 404 

Lead  Neuritis                 407 

Arsenical  Neuritis 407 

Other  Intoxications 408 

Infectious  Disease  Types 408 

Plexus  Palsies 409 

Brachial  Plexus  Palsies 409 

Total  Brachial  Palsy 410 

Inferior  Root  Type 412 

Supciior  Brachial  Plexus  Palsy    ....            412 

Mixed  Types .  413 

Cervical  Rib  414 


xii  CONTENTS 

Plexus  Palsies — 

Lumbosacral  Syndromes 415 

Lumbar  Root  Syndromes 415 

Sacral  Root  Syndromes 416 

Peripheral  Palsies 417 

Defects,  Congenital  and  Acquired 417 

Peripheral  Palsies  due  to  War  Injuries 418 

Injuries  to  Facial  Nerve 426 

Spinal  Accessory  Nerve 427 

The  Long  Thoracic  Nerve 427 

The  Circumflex  Nerve 427 

Ulnar  Nerve 427 

Musculocutaneous  Nerve 429 

Median  Nerve 429 

Radial  or  Musculospiral  Nerve 431 

Sciatic  Nerve 434 

External  PopUteal  Nerve 434 

Internal  PopUteal  Nerve 436 

CHAPTER  VI. 

Lesions  of  the  Spinal  Cord. 

Acute  Pohoencephalomyelitis 441 

Spinal  Forms 448 

Acute  Ascending  Form — Landry's  Paralysis  452 

Bulbar  and  Pontine  Forms 452 

EncephaUc  or  Cerebral  Form 453 

Cerebellar  Forms 453 

Meningitic  Forms 454 

Polyneuritic  Forms 454 

Vegetative  Types 454 

Incomplete  or  Minor  Forms 454 

Primary  Progressive  Muscular  Atrophies 459 

Group  1.  The  Progressive  Nuclear  Atrophies:     (1)  Spinal,  (2)  Bulbar 

and  (3)  MesencephaUc  Forms 460 

Spinal 460 

Chronic  PoUomyeUtis 461 

Aran-Duchenne  Type.     Progressive  Muscular  Atrophy    .  462 

Infantile  Hereditary  Forms 463 

Bulbopontine  Types.     Chronic  Progressive  Bulbar  Palsies      .  464 
Pontomesencephalic  Forms — Chronic  Progressive  Ophthalmo- 
plegia      465 

Group  2.  The  Neural,  Neuritic  or  Spinal  Neuritic  Atrophies  .  465 

Peroneal-forearm  Type 466 

Tabetic  Type 469 

Amyotrophic  Lateral  Sclerosis 469 

Fracture  and  Dislocation  and  Concussion  Syndromes 473 

Concussion  Syndromes 484 

Compression  of  the  Cord 485 

Bone  Hypertrophies 485 

Tuberculosis  (Caries) 485 

Spinal  Cord  Tumors 486 


CONTENTS  xiii 

Lateral  Sclerosis  Group        488 

Combined  Scleroses.     Combined  Degeneration 490 

Combined  Sclerosis 491 

Combined  Sclerosis  in  Paresis " 491 

Toxic  Anemic  Forms 491 

Senile  Forms 491 

Syringoencephalomyelia 492 

Multiple  Sclerosis 506 

CHAPTER  VII. 

Lesions  at  the  Level  of  the  Medulla,  Pons,  Brain-stem  or  Midbrain. 

Medullary  Sjoidromes 524 

Lesions  of  the  Pons 531 

Inferior  Alternate  Paralysis 537 

Peduncle  Syndromes 542 

Corpora  Quadrigemina  Syndromes 546 

Rabies 550 

Tetanus 551 

CHAPTER  VIII. 

Paralysis  Agitans,  Lenticular  Degeneration,  Chorea,  and  Related 

Syndromes. 

Paralysis  Agitans  Group 557 

Progressive  Lenticular  Degeneration 567 

Dystonia  Musculorum  Deformans 568 

The  Choreas 570 

Chorea  Minor 571 

Huntington's  Chorea 575 

CHAPTER  DC. 

Cerebellab  Syndromes. 

Chief  Syndromes 590 

Inferior  Cerebellar  Peduncle — Corpus  Restiforme 590 

Lesions  of  the  Middle  Cerebellar  Peduncle 591 

Lesions  of  the  Superior  Cerebellar  Peduncles .      .  591 

Lesions  within  the  Cerebellum  Itself 591 

Cerebellar  Aplasia 591 

OUvo-ponto-cerebellar  Atrophy         593 

Cerebellum  and  Cord  Atrophies  or  Aplasias 593 

Primary  Parenchymatous  Degeneration 595 

Cysts  of  Cerebellum 595 

Tumors  of  Cerebellum 595 

Dyssynergia  Cerebellaris  Progressiva — Chronic  Progressive  Cere- 

beUar  Tremor 598 

Wounds  of  the  Cerebellum 599 

Hemorrhage  of  Cerebellum 599 

Abscess  of  Cerebellum 600 

Associated  Posterior  Fossa  Complications 601 


xlv  CONTENTS 


CHAPTER  X. 

Diseases  of  the  Meninges. 

Dural  Disease 604 

Meningeal  Apoplexy 604 

Inflammation  of  Dura 608 

Pachymeningitis  Externa 608 

Pachymeningitis  Interna  Simplex 609 

Pachymeningitis  Interna  Hemorrhagica 609 

Diseases  of  the  Arachnoid  and  Pia.     Leptomeningitis 611 

Acute  Leptomeningi tides 611 

Infectious  Meningitis 615 

Epidemic  Cerebrospinal  Meningitis 615 

Tuberculous  Meningitis 617 

Serous  Meningitis 618 

Syphilitic  Meningitis 619 

Chronic  Leptomeningitis 620 

Hydrocephalus 621 

Sunstroke 623 

CHAPTER  XI. 

Diseases  of  the  Brain. 

EncephaUtis — Abscess  of  the  Brain 624 

Acute  EncephaUtis 625 

Abscess  of  the  Brain 629 

CHAPTER  XII. 

Diseases  of  the  Brain  {Continued), 

Vascular  Disturbances — Cerebral  Apoplexies 633 

Vascular  Instability 635 

Anemia 635 

Hyperemia 635 

Cerebral  Arteriosclerosis 636 

Cerebral  Apoplexies 637 

Hemorrhage,  Thrombosis,  Embohsm 637 

The  Apoplectic  Attack 638 

The  Thalamic  Syndrome 642 

Sensory  Changes  in  Cortical  Lesions,  Suprathalamic  Pathways      ....  646 

Cortical  Sensory  Disturbances 651 

Apraxia 653 

Sinus  Thrombosis 662 

CHAPTER  XIII. 

Tumors  of  the  Brain. 

True  Tumors 665 

Infectious  Tumors 667 


CONTENTS  XV 

Parasitic  Cystic  Tumors 668 

Aneurismal  Tumors .      .  668 

Frontal  Lobe  Tumors 673 

Central  Convolution  Tumors 675 

Parietal  Lobe  Tumors 677 

Temporal  Lobe  Tumors 677 

Occipital  Lobe  Tumors 679 

Corpus  Callosum  Tumors 680 

Optic  Thalamus  Tumors 681 

CHAPTER  XIV. 

Neurosyphilis. 

Syphilis  of  the  Cranial  Bones  Causing  Nervous  Symptoms 699 

Syphilitic  Meningitis  of  the  Base 700 

Syphilitic  Meningitis  of  the  Convexity 703 

Cerebral  Syphilis 705 

Vascular  Types 705 

Parenchymatous  or  Central  Types  (General  Paresis) 708 

Dementing  Forms 722 

Depressed  Forms 722 

Expansive  Forms 723 

Agitated  Forms 726 

Irregular  Forms 727 

Juvenile  Paresis 728 

Syphilitic  Psychoses 72& 

Tabes 734 

Syphihtic  Meningomyelitis 753 

Congenital  or  Hereditary  Syphihs 757 


PART  III. 
PSYCHICAL  OR  SYMBOLIC  SYSTEMS. 

NEUROSES,  PSYCHONEUROSES,  PSYCHOSES. 
Introduction 773 


CHAPTER  XV. 


The  Psychoneuroses  and  Actual  Neuroses. 

The  Psychoneuroses 788 

Hysteria 788 

Compulsion  Neurosis 796 

Anxiety  Hysteria 805 

"Shell  Shock" 806 


XVi  CONTENTS 

The  Actual  Neuroses 806 

Anxiety  Neurosis 806 

Neurasthenia 814 

Mixed  Neuroses 817 

CHAPTER  XVI. 

Manic-depressive  Psychoses. 

Manic  Phase 829 

Depressive  Phase 835 

The  Periodical  Types 837 

The  Cyclothymias 840 

The  Mixed  States 842 

Involution  Melancholia 843 

CHAPTER  XVII. 

The  Paranoia  Group. 

Paranoia  of  Kraepehn 854 

Mixed  and  Aberrant  Forms 855 

Paraphrenias 856 

CHAPTER  XVIII. 

Epilepsy  and  Convulsive  Types  of  Reaction. 

Classical  Epilepsy 872 

The  Epileptic  Constitution 873 

The  Seizure 874 

Attenuated  Forms.     Affect  Epilepsies 875 

Epilepsies  of  Gross  Brain  Disease 876 

CHAPTER  XIX. 

Dementia  Precox  (Schizophrenia)  Group. 

Dementia  Simplex 894 

Hebephrenia 895 

Catatonia 898 

Paranoid  Forms 901 

Mixed  and  Atypical  States 902 

CHAPTER  XX. 
Infection — ExhausIuon  Psychoses. 

Prefebrile,  Febrile  and  Postfebrile  Psychoses 918 

Infection  and  Initial  Delirium 919 

Fever  Dehrium 919 

Postfebrile  Psychoses 920 

Exhaustion  Psychoses 920 

Collapse  Dehrium 920 

Acute  Hallucinatory  Confusion  (Amentia) 921 

Typhoid  Fever 922 


CONTENTS 


xvii 


CHAPTER  XXI. 

The  Toxic  Psychoses. 

Alcoholism 925 

Drunkenness 927 

Chronic  Alcoholism 927 

Delirium  Tremens 929 

Korsakow's  Psychosis 930 

Alcoholic  Hallucinosis 932 

Alcohohc  Pseudoparesis 933 

Alcohohc  Pseudoparanoia 933 

Alcohohc  Epilepsy 934 

Dream  States 934 

Dipsomania 934 

Opium 934 

Cocain 935 

Miscellaneous  Intoxicants 935 

Bromides 935 

Carbon  Monoxide 936 

Lead 936 

Mercury        ....  936 

Uremia 937 

Diabetes  MeUitus 937 

Gastro-intestinal  Diseases 937 

PeUagra   .' 938 

CHAPTER  XXII. 

Psychosis  Associated  with  Organic  Diseases. 

Apoplexy 940 

Traumatism 940 

Acute  Chorea 943 

Chronic  Chorea 944 

Paralysis  Agitans 945 

Multiple  Sclerosis 945 

Polyneuritis 946 

Heart  Disease 946 


CHAPTER  XXIII. 


Presenile,  Senile,  and  Arteriosclerotic  Psychoses. 


The  Presenile  Psychoses 
Involution  Melancholia 
Other  Psychoses  of  this  Period 

The  Senile  Psychoses 

Intermediate  Conditions 
Normal  Senile  Involution  . 
Simple  Senile  Deterioration 
Senile  Dehrium 
Presbyophrenia  .... 


947 
947 
951 
953 
953 
953 
954 
955 
955 


xviii  CONTENTS 

Alzheimer's  Disease 958 

Arteriosclerotic  Psychoses 958 

Arteriosclerotic  Brain  Atrophy 959 

Subcortical  Encephalitis 959 

Perivascular  GUosis 959 

Senile  Cortical  Devastation 959 


CHAPTER  XXIV. 

Idiocy,  Imbecility,  Feeble-mindedness,  and  Characterological 
Defect  Groups. 

Feeble-mindedness 967 

Imbecility 967 

Moral  ImbeciUty 967 

Idio-imbeciUty 967 

Idiocy '   .      .  967 

Amaurotic  Family  Types 969 

Sclerotic  Types 969 

Cretinism 971 

Mongolism 973 

HydrocephaUc  Types 975 

Microcephalic  Types 975 

Paralytic  Types        :.      .  978 

Traumatic  Types !      .  979 

Epileptic  Types A^  ^^^ 

Inflammatory  Types m'.,  980 

Sensorial  Types 9    ■  980 

SyphiUtic  Types '     .  980 

Idiot-savants ■  982 

Mild  Grades  of  Defect 982 

Psychopathic  Constitution 984 

AnomaUes  of  the  Sexual  Instinct 987 

Quantitative  Anomalies 987 

QuaUtative  Anomalies 987 

Masturbation 987 

Active  Algolagnia 987 

Passive  Algolagnia //  •      "      ■  ^^^ 

Homosexuality /  987 

Narcissism /      .      .      .  987 

Fetichism '/....  988 

BestiaUty '  ....  988 

Exhibitionism 988 

Necrophiha 988 


DISEASES  OF  THE  NERVOUS  SYSTEM. 


INTRODUCTION. 

OX  SOME  PPJNXIPLES  UNDERLYING  A  CLASSIFICATION 
OF  DISEASES  OF  THE  NERVOUS  SYSTEM. 

Classifications  have  value  as  offering  a  condensed  resume  of 
existing  attitudes  of  mind  with  reference  to  the  nature  and  relations 
of  the  things  classified.  They  also  serve  as  a  concrete  setting  forth  of 
newer  concepts,  which,  but  for  the  effort,  would  tend  to  linger  indefi- 
nitely in  older  settings. 

For  a  long  time  the  nervous  system  has  been  considered  as  a  thing 
apart,  a  sort  of  consecrated  territory,  where  only  the  initiated  could 
enter.    It  was  a  place  for  mystery  and  for  bewilderment. 

The  mind  was  more  consecrated  and  mysterious  still,  and  was  dealt 
with  metaphysically  until  its  relations  to  the  nervous  system  were 
much  more  vague  than  the  relations  of  the  nervous  system  to  the  rest 
of  the  body.  This  state  of  affairs  has  been  crystallized  by  the  titles 
of  our  text-books,  which  set  forth  that  they  deal  with  nervous  and 
mental  diseases,  inferring  that  these  two  groups  have  little  relation, 
the  one  to  the  other,  and,  by  the  same  token,  fail  to  indicate  that  they, 
either  or  both,  have  any  relation  to  the  rest  of  the  body. 

All  this  is  faulty.  The  human  individual  is  a  biological  unit,  his 
several  parts  are  parts  of  that  unity,  and  he  himself  is  still  part  of  a 
greater  whole,  a  particular  instance  of  the  manifestation  of  life.  Any 
system  of  classification,  no  matter  how  far  it  attempts  to  go  in  formulat- 
ing distinct  disease  trends,  should  not  fail  altogether  to  permit  this 
broader  view  of  the  interrelations  to  show  through. 

From  the  point  of  view  of  the  nervous  system,  how  is  this  to  be  done? 
Recent  developments  in  physiology  and  in  internal  medicine  point  the 
way.  Reference  is  made  to  the  development  in  knowledge  of  the 
vegetative  nervous  system  and  of  the  endocrine  glands.  These  develop- 
ments have  served  the  twofold  purpose  of  bringing  the  several  functions 
of  man  into  a  closer-knit  harmony  and  at  the  same  time  correlating 
that  harmony  with  the  manifestations  of  the  organic  activities  as 
found  in  lower  animals.  The  last  has  been  the  result  because  the 
develo])ments  have  had  to  do  with  what  are  called  lower,  simpler  or, 
more  ])roperly,  ])hylogenetically  older  forms  of  activity,  or  modes  of 
reaction. 

2  (17) 


18  INTRODUCTION 

Right  here,  in  the  term  " phylogenetically  older,"  is  the  key  to  the 
principle  of  classification — a  principle  that  has  long  governed  in  the  bio- 
logical sciences,  the  simpler  and  older  merging  by  insensible  gradations 
into  the  more  complex  and  more  recent.  It  has  taken  long  periods  of 
time  and  the  accumulation  of  great  masses  of  detailed  observations 
before  those  facts  of  wider  bearing  have  emerged  that  have  served  to 
bring  all  this  material  together  under  wider  generalizations.  The 
older  hypotheses  have  become  more  and  more  inadequate;  in  their 
prime  they  were  useful,  but  as  with  all  hypotheses,  which  are  solely 
functions  of  thinking,  their  present  aspect  of  staleness  is  a  sign  of 
possible  progress. 

For  a  long  time  it  has  been  asserted  that  the  nervous  sytem  is  the 
means  by  which  all  of  the  several  parts  of  the  human  unit  are  integrated 
by  a  species  of  complicated  adjustments  to  given  ends.  It  can  be 
perceived  how  this  integration  is  actually  brought  to  pass  by  means 
of  the  vegetative  nervous  system  and  the  chemical  regulators  of 
metabolism,  at  a  physicochemical  level,  and  how  by  the  successive 
compounding  of  reflexes  at  the  sensorimotor  level,  the  human  unit 
is  further  integrated,  so  that  it  can  as  a  whole  work  more  consistently 
toward  broadly  defined  goals,  the  integration  manifesting  itself  at  suc- 
cessively higher  and  higher  levels  in  the  history  of  the  individual  and 
of  the  species. 

Viewed  in  this  way  the  individual  is  seen  struggling  along  the 
path  of  evolution  in  constant  conflict  with  an  inherent  inertia  that 
would  keep  it  at  a  given  level,  but  gradually  advancing  by  a  series  of 
give-and-take  compromises  that  finally  bring  it  to  better  adjustment 
with  its  environment  at  ever  higher  levels  of  integration. 

Sherrington  has  beautifully  illustrated  this  integrative  action  of  the 
nervous  system  in  the  simple  reflex  with  its  innervation  of  agonists 
on  the  one  hand  and  antagonists  on  the  other,  and  the  channelling  of 
final  common  pathways  for  nervous  discharge.  This  law  of  conflicting 
tendencies,  pathways  of  opposites — ambivalence — where  the  final 
issue  for  higher  integrations  is  made  possible  at  the  sensorimotor 
level  by  the  tension  of  reciprocal  innervations,  is  found  also  to  be  the 
rule  in  the  vegetative  nervous  system,  with  its  double  set  of  pharmaco- 
dynamically  demonstrated  balancing  elements,  mediated,  at  least  in 
part,  by  equally  opposed,  stimulogenic  chemical  substances  secreted 
by  the  endocrinous  glands,  the  hormones. 

Finally  an  analogous  ambivalent  mechanism  is  seen  working  at  the 
highest,  the  most  complex  level,  the  psychic,  which  determines  conduct 
with  the  assistance  of  the  phenomenon  called  consciousness,  in  which  a 
psychological  symbolism  is  found  replacing  sensory  and  motor  neurons, 
and  exciting  and  inhibiting  hormones. 

For  practical  purposes,  then,  the  nervous  system  may  be  divided 
into  these  three  levels  of  activity,  the  vegetative,  the  sensorimotor 
and  the  psychic. 

This  threefold  division  of  the  reactions  of  the  nervous  system  is  the 
fundamental  basis  on  which  a  classification  mav  be  founded.     The 


CLASSIFICATION  OF  DISEASES  OF  THE  NERVOUS  SYSTEM     19 

biological  activities  which  maintain  life  at  the  lowest  level  are  physical 
and  chemical,  and  thus  that  portion  of  the  nervous  system  which  has 
direct  controlling  influences  over  these  activities  is  properly  designated 
as  the  vegetative  nervous  system,  and  that  part  of  neurology  which 
has  to  do  with  a  consideration  of  these  physicochemical  systems 
because  it  deals  with  the  nervous  control  of  the  viscera  and  of  metab- 
olism, is  properly  designated  as  visceral  or  vegetative  neurology. 

In  this  region  of  vegetative  neurology  a  rich  variety  of  disturbances 
is  found,  involving  the  glandular,  gastro-intestinal,  genito-urinary, 
vascular,  respiratory,  muscular,  cutaneous  and  bony  systems.  In 
addition  there  are  certain  complex  clinical  groups  involving,  for  the 
most  part,  the  glands  of  internal  secretion,  the  endocrinopathies. 
Here  is  a  rich  field  which  has  been  unequally  cultivated  since  Brown- 
Sequard  first  tried  to  discover  the  fountain  of  youth  in  the  testicular 
extracts. 

\Miile  the  symptomatology  of  the  neurological  disturbances  of  the 
tear,  mucous  and  salivary  glands  is  a  comparatively  limited  one,  a 
very  rich  symptomatology  has  grown  up  about  the  vascular  system 
in  the  group  of  vasomotor  neuroses.  There  are  many  strictly  ne  irolog- 
ical  problems  among  the  gastro-intestinal  and  in  the  cutaneous  dis- 
orders, which,  however,  are  for  the  most  part  taken  over  by  the 
specialties  dealing  with  these  respective  systems,  but  nevertheless 
many  of  these  disorders  will  receive  an  adequate  explanation  only 
through  the  understanding  of  visceral  neurology.  Some  portions  of  the 
field  are  as  yet  too  little  known  to  offer  much  that  is  of  value,  as,  for 
example,  the  neurology  of  the  bony  system  and  the  nervous  mechanism 
underlying  the  regulation  of  the  blood  cells  and  the  relations  of  the 
^'egetative  nervous  system  to  immunity  and  anaphylaxis,  while  in 
other  systems  the  disturbances  are  known  only  as  contributing  symp- 
toms in  fairly  well-defined  clinical  groups,  as,  for  example,  myas- 
thenia gravis  as  a  disturbance  of  the  muscle  vegetative  mechan- 
isms. 

The  endocrinopathies  naturally  form  a  considerable  part  of  visceral 
neurology,  and  many  of  the  disturbances  of  the  several  systems  are 
still  best  included  in  the  various  clinical  groups  that  are  considered  as 
due  to  disturbances  in  one  or  more  of  the  endocrinous  glands.  This 
field  today  occupies  the  main  focus  of  attention  of  the  medical  prac- 
titioner, and  much  that  is  false  is  being  taught;  but  out  of  the  diver- 
gent trends  sound  harmonies  will  be  evolved. 

If  the  vegetative  nervous  system  has  for  its  function,  in  the  main, 
the  maintenance  of  the  vegetative,  that  is,  the  metabolic  processes  of 
life,  such  as  nutrition,  growth,  development  and  involution,  the  next 
higher  level,  the  sensorimotor,  has  as  its  function,  in  the  main,  further 
integration  by  })r<)viding  the  means  for  the  balanced  interrelations  of 
the  various  motor  organs  of  the  body.  It  has  to  provide  that  all  the 
various  parts  of  the  machine  work  harmoniously  together,  that  the 
functions  of  the  various  organs  are  not  only  properly  timed  in  relation, 
one  to  tile  others,  but  a^o  adequately  related  on  the  basis  of  the 


20  INTRODUCTION 

functional  demands  made  on  them  to  carry  out  the  necessary  motor 
activities  to  satisfy  1  he  vegetative  system  needs. 

This  field  of  sensorimotor  neurology,  including  the  disorders  of  the 
cranial  and  peripheral  nerves,  the  spinal  cord,  medulla,  pons,  brain- 
stem, midbrain,  cerebellum  and  cerebrum,  is  that  portion  of  neurology 
to  which  the  term  "neurology"  is  generally  thought  of  as  applying, 
to  all  intents  and  purposes,  exclusively.  Here  the  field  is  pretty  well 
cultivated,  and  classifications  based  on  anatomical  and  on  etiological 
criteria  are  pretty  well  accepted. 

The  third,  the  highest,  the  psychic  level  is  the  most  complex.  Its 
function  is  no  longer  simply  one  of  integration  of  the  various  parts  of 
the  individual  but  at  its  highest,  conscious  level  it  has  to  do  not  only 
with  the  relation  of  the  individual  as  a  whole  to  his  environment  but 
more  especially  to  his  social  environment. 

At  this  level  it  has  been  the  prevalent  custom  to  think  only  of 
consciousness,  and  of  conduct  consciously  regulated  by  intelligence. 
Ideas  are  symbols;  they  are  symbols  of  the  contemplated  action  on 
things,  through  w^hich  the  individual  comes  to  an  efficient  adjustment 
with  his  environment  by  controlling  them.  The  symbol  therefore 
becomes  a  carrier  of  energy  which  is  translated  into  conduct. 

The  ways  in  which  these  psychic  symbolizations  work  at  the  highest 
conscious  levels  are  pretty  well  formulated  in  current  psychology,  and 
these  ways  work  very  well  so  long  as  there  is  nothing  unusual  the 
matter  with  the  whole  machine.  The  great  error  of  the  psychologist, 
however,  has  been  to  suppose  that  the  matter  stopped  here.  The 
lower  animals  exhibit  most  complex  forms  of  behavior  without  its 
being  thought  necessary  to  ascribe  conscious  motives  (intelligence) 
to  them  in  explanation.  Very  complicated  activities  low  down  in  the 
biological  scale  are  ascribed  to  tropisms,  while  for  man  it  has  been 
supposed  that  what  he  did  he  consciously  intended.  Recent  studies 
in  psychopathology  have  shown  the  inadequacy  of  this  conception, 
and  it  is  thoroughly  well  established  that  lying  back  of  consciousness 
is  a  much  larger,  a  much  more  important  territory  which  furnishes  a 
psychic  motivation  of  conduct,  and,  in  fact,  that  conscious  processes 
as  they  are  known  to  the  individual  are  largely,  if  not  altogether 
determined  by  what  lies  in  this  region — the  unconscious. 

Psychic  symbols — ideas,  feelings — must  therefore  be  traced  farther 
back  than  the  conscious  level  at  which  the  individual  is  acquainted 
with  them  in  order  to  understand  their  real  meanings.  Psychoanalysis 
is  as  important  for  the  understanding  of  the  construction  of  the  psyche 
as  dissection  is  for  the  understanding  of  the  structure  of  the  body,  or 
chemical  analysis  for  the  understanding  of  the  constitution  of  the 
molecule. 

The  greatest  deficiency  in  the  psychology  of  the  nineteenth  century 
relative  to  the  understanding  of  human  conduct  has  been  the  neglect 
of  the  unconscious. 

For  centuries  man  has  marvelled  and  speculated  and  gathered 
observations  concerning  the  exquisite  subtleties  of  adaptation  of  plant 


CLASSIFICATION  OF  DISEASES  OF  THE  NERVOUS  SYSTEM     21 

structures  to  their  environment.  Students  of  nature  have  recorded 
in  encyclopedic  proportions  the  intricacies  of  Nature's  story  of  the 
conduct  of  the  lower  animals  from  protozoa  to  highest  ape.  These 
activities  have  been  relegated  to  tropisms  and  to  instincts.  Man 
alone  has  supposed  that  he  could  explain  his  owti  conduct  by  reference 
to  that  which  appears  in  his  consciousness,  unmindful  of  the  millions 
of  years  of  evolution  preceding  that  which  he  has  designated  as  his 
conscious  activities. 

With  the  help  of  the  hypothesis  of  the  unconscious,  however,  it  has 
come  to  be  recognized  that  the  psyche  has  its  embryology  and  its 
comparative  anatomy — in  short,  its  history — just  as  the  body  has 
and  in  precisely  the  same  way  as  in  the  case  of  the  body  this  history 
has  to  be  utilized  before  it  can  be  understood. 

So  long  as  the  unconscious  failed  to  be  recognized,  just  so  long  was 
the  gap  between  so-called  body  and  so-called  mind  too  wide  to  be 
bridged,  and  so  there  arose  the  two  concepts,  body  and  mind,  which 
gave  origin  to  the  necessity  of  defining  their  relations.  Consciousness 
covered  over  and  obscured  the  inner  organs  of  the  psyche  just  as  the 
skin  hides  the  inner  organs  of  the  body  from  vision.  But  just  as  a 
knowledge  of  the  body  first  became  possible  by  the  removal  of  the  skin 
and  the  revealing  of  the  structures  that  lay  beneath,  so  a  knowledge 
of  the  psyche  first  became  possible  when  the  outer  covering  of  con- 
sciousness was  penetrated  and  what  lay  at  greater  depth  was  revealed. 
As  soon  as  this  was  done,  the  wonderful  history  of  the  psyche  began 
to  give  up  its  secrets,  and  the  distinction  between  body  and  mind  began 
to  dissolve,  until  now  it  has  come  to  be  considered  that  the  psyche  is 
the  end-result  in  an  orderly  series  of  progressions  in  which  the  body 
has  used  successively  more  complex  tools  to  deal  with  the  problems 
of  integration  and  adjustment. 

The  hormone  is  the  tj-pe  of  tool  at  the  physicochemical  level,  the 
reflex  at  the  sensorimotor  level,  and  finally,  the  symbol  at  the  psychic 
level. 

In  the  phylogenetic  history  of  that  development  which  culminates 
in  man,  the  symbol  has  been  developed  after  trying  and  laying  aside 
a  great  variety  of  tools,  because  it  offered  the  means  of  unlimited 
development  of  man's  control  over  nature.  The  hormone,  the  reflex, 
are  confined  in  their  capacities  for  reaction  within  relatively  narrow 
limits  of  possibilities.  The  symbol  is  capable  of  infinite  change  and 
adjustment,  and  so  has  grown  out  of  the  necessity  created  by  ever 
increasing  demands.  The  growth  from  the  lowest  to  the  highest,  from 
the  youngest  to  the  oldest,  from  the  simplest  to  the  most  complex  has 
been  here,  as  everywhere  in  nature,  without  gaps. 


CHAPTER  I. 
METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM. 

The  student  of  diseases  of  the  nervous  system,  be  they  vegetative, 
sensorimotor  or  mental,  should  have  at  his  command  a  practical,  sys- 
tematic series  of  methods  for  examining  his  patients.  Such  a  scheme  of 
case-examination  should  above  all  be  orderly,  and  sufficiently  extensive 
to  enable  him  to  make  a  rapid  si  zing-up  of  the  character  of  the  dis- 
turbance under  investigation.-  It  should  not  be  burdened  with  details 
for  which  an  application  will  be  found  in  only  the  rarest  of  disorders. 

A  comprehensive  neurological  history  should  include  a  careful 
study  of  the 

I.  Family  History. 
II.  History  of  Patient. 

III.  History  of  the  Present  Illness. 

IV.  General  Observations. 
V.  Physical  Examination. 

VI.  Vegetative  Examination. 
VII.  Sensorimotor  Examination. 
VIII.  Mental  Examination. 

For  the  sake  of  convenience  the  chief  factors  to  be  covered  are 
here  given  in  the  form  of  printed  questionnaires.  Such  printed  schemes 
have  their  advantages  and  their  disadvantages.  But  if  carefully 
and  thinkingly  followed  the  advantages  far  outweigh  the  disadvan- 
tages. For  the  beginner  in  neurology,  to  whom  this  book  is  addressed 
especially,  such  questionnaires  well  grounded  in  the  mind,  are  the  first 
step  in  the  development  of  a  technic  of  case-examination  which  will 
prove  invaluable.  It  is  not  thought  that  other  forms  may  not  prove 
as  satisfactory,  but  some  definite  scheme  is  indispensable. 

Questionnaires. — The  heading,  size,  form  of  binding,  of  ruling,  etc., 
will  be  determined  by  local  conditions  and  by  special  needs.  The 
definite  facts  which  are  noted  in  the  questionnaires  are  not  by  any 
means  all  of  the  facts  to  be  collected,  and  such  printed  question  blanks 
are  given  purely  as  guides,  rather  than  as  finished  products.  As 
practical  blanks  for  out-patient  dispensary  work  they  are  invaluable 
and  also  for  note-taking  in  private  work.  In  hospital  work  with 
nervous  disorders,  where  it  is  hoped  that  autopsy  material  may  put 
opportunities  for  anatomical  research  in  one's  hands,  such  blanks  are 
naturally  too  didactic  and  inelastic.  The  main  outlines,  however,  are 
still  desirable. 

A  general-status  blank  is  often  advisable.    Inasmuch  as  this  country 
has  been  put  on  a  war  basis  for  some  years  facts  concerning  military 
service  should  be  included.    Such  a  blank  is  here  given  in  short  form : 
(22) 


GENERAL-STATUS  QUESTIONNAIRE 


23 


NAME:               Age:               Rank:               Co.:  Corps: 

Birthplace:                Date:                      In  U.  S.:  Relig. : 

Occupations:          $:          M.S.W.D.:        Enlist:  Draft: 
Home  Address :                Nearest  Relative : 

Admitted:               Referred:               Transfer:  Examiner: 


Heredity : 

Military  History : 

Parents  related : 

Birthplace: 

, 

F. 

Mental: 

M. 

Nervous : 

Children : 

Epilepsy: 

TBC: 

Diabetes : 

Alcohol : 

Goitre : 

Syphilis: 

Birth:  Early  History: 

Walk:                   Talk 

:                   Read : 

Children's  Dis. : 

Enuresis: 

Nail  bite: 

Sleep  Walk: 

Stammer: 

Other  child  traits : 

Education: 

Adult  Status: 

- 

Syphilis: 

Treatment : 

Present  Illness: 

Shocks : 

Tropics : 

Habits:              Ale: 

Tob.: 

Mast: 

Sex  Experience : 

Ardor : 

Frigidity : 

Accident : 

Gunshot : 

Occupation  Toxemias 

Convulsions  (injury — 

tongue — urine) : 

Constitution : 

Wt.:               Ht.: 

Marriage : 

Menses : 

Children : 

Mis.: 

Dead: 

24     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 


I.  Family  History. — Informant  (name,  relationship  to  patient, 
address.) 

Grandparents;  parents  (uncles  and  aunts);  Siblings. 

Children  (with  abortions  and  miscarriages). 

Note. — In  securing  the  family  history  it  must  be  remembered  that 
it  is  equally  important  to  get  a  record  of  all  the  well  members  of  the 
family  as  well  as  'he  sick  ones  and  not  stop  with  securing  the  latter 
as  is  often  done.  The  patient's  relation  to  hereditary  tendencies  can 
only  be  determined  by  securing  the  fullest  information  about  his 
ancestors. 


O 


DvJ»  C    .  I 


Fig.  1. — Pedigree  chart  illustrating  how  two  delective  parents  may  have  only  defective 
children.  A,  alcoholic;  C,  criminalistic;  D,  infant,  died  in  infancy;  F,  feeble-minded; 
N,  normal;  T,  tuberculous.     (Goddard,  1910.) 

It  is  desirable  to  get  a  family  history  as  well  as  an  objective  history 
both  from  the  patient  and  from  other  members  of  the  family. 

The  first  factor  to  be  investigated  is  heredity.  The  usual  facts 
gathered  concerning  heredity  are  frequently  worthless.  Better  no 
facts  concerning  the  extremely  complicated  question  of  heredity  than 
false  ones.  In  ordinary  investigation  the  research  need  not  be  exhaus- 
tive. A  complete  consideration  of  heredity  is  best  left  to  special 
eugenic  students  whose  business  it  has  become  to  properly  collate 
and  weigh  the  mass  of  material  needed  to  bring  out  salient  factors 
in  the  heredity  problem.  The  main  facts  noted  in  the  questionnaire 
are  sufficient,  save  in  the  consideration  of  certain  disorders,  in  which 
hereditary  factors  are  known,  such  as  Huntington's  chorea,  Thomsen's 
disease,  diabetes  mellitus,  deaf-mutism,  certain  optic  atrophies,  etc. 

Consanguinity  in  the  parents  is  first  to  be  inquired  into;  the  degree 
of  relationship  should  be  explicitly  expressed.  Hough  charts  con- 
structed on  the  plan  of  Fig.  1  should  be  used  to  show  the  relationships. 

Consanguinity  may  or  may  not  have  any  relation  to  nervous  dis- 
orders; it  much  depends  on  the  stock.  Bad  factors  present  in  one 
or  another  may  be  absolutely  missed  in  the  descendants,  for  Men- 
delian  laws  seem  fairly  well  established.  With  the  presence  of  dominant 
neuropathic  traits  of  the  same  kind  in  both  ascendants,  the  chances 
are  less  hopeful  in  the  descendants.  In  human  fecundity  but  few  of  the 
ova  are  impregnated,  and  each  ovum  undoubtedly  contains  its  own 


FAMILY  HISTORY  25 

individualistic  grouping  of  hereditary  factors.  The  chance  element 
seems  too  great  to  estimate.  One  feature  of  consanguinity  not  to  be 
overlooked  is  that  many  relatives  (first  cousins,  second  cousins)  marry 
each  other  because  of  a  neuropathic  tendency.  It  is  not  impossible 
then  that  the  evil  results  of  consanguinity  sometimes  seen  is  a  direct 
transmission  of  the  neuropathic  traits  that  were  primarily  responsible 
for  the  marriage.  At  all  events  the  studies  of  Woods/  Cox,^  Lund- 
borg,^  Punnett/  Bateson/  Karplus,^  and  the  numerous  papers  of  Karl 
Pearson/  all  give  hope  of  new  and  more  definite  outlooks  in  the  study 
of  the  relationship  of  heredity  to  disease  in  general,  and  of  disorders 
of  the  nervous  system  in  particular. 

Abraham^  and  others  have  given  some  suggestive  discussions  con- 
cerning the  tendency  for  related  neurotics  to  marry. 

Organ  Inferiority. — The  concept  of  inferior  organs,  recently  set 
forth  by  Adler,  must  needs  be  taken  into  consideration  in  the  matter 
of  heredity  in  a  much  broader  sense  than  heretofore.  Inferior  organs, 
in  their  phylogenetic  relationships  have  to  be  considered  and  the 
indirect  results  noted  rather  than  attempting  to  follow  out  heredity 
along  the  concrete  lines  heretofore  in  evidence.  For  example,  it  has 
recently  been  shown  that  in  the  ascendants  of  pellagrines  there  was  a 
noticeable  tendency  to  diseases  of  the  skin  and  of  the  gastro-enteric 
tract.  In  the  interpretation  of  such  a  fact  the  evident  relationship  of 
the  skin  and  mucous  membrane  of  the  stomach  and  intestines  has  to  be 
correlated  with  the  presumed  etiological  factor  of  the  disease  as  worked 
out  by  Goldberger  and  his  associates.  His  work  indicates  quite  clearly 
that  it  is  a  metabolism  disorder  belonging  in  the  group  with  beriberi 
and  scurvy  and  dependent  upon  a  deficient  diet.  The  toxemia  result- 
ing produces  the  symptomatology  which  largely  manifests  itself  as  a 
peculiar  form  of  dermatitis  associated  with  a  gastro-enteritis.  The  lack 
of  some  phosphorus  compound  is  a  necessary  and  specific  factor, 
while  the  toxin  which  results  produces  its  effect  upon  the  inferior 
organs,  the  skin  and  mucous  membrane.  Viewing  the  facts  of  heredity 
in  this  larger  way  will  undoubtedly  broaden  our  concept  of  various 
diseases. 

Menial  Disorder. — The  presence  of  mental  disorder  in  the  direct 
ancestry  is  of  moment,'  not  as  indicating  that  mental  disorder  as  such 
is  inherited  but  as  an  aid  to  an  understanding  of  the  characteristics 
of  the  stock  with  which  the  individual  comes  to  take  up  his  life  prob- 
lems. The  evidence  drawn  from  aunts  and  uncles  is  of  value,  while 
that  from  cousins  is  open  to  the  influence  of  another  stock.    It  is  useless 

•  Heredity  in  Royalty,  1906.  2  Psychiatrische  Neurologische  Bladen,  1907. 

'  Ueber  DeReneration,  1901.  *  Mendclism  in  Relation  to  Disease. 

»  Brain.  1906. 

'  Zur  Kenntniss  der  Variabilitat  und  Vererbung  am  Zentralnervensystem,  1907. 

'  Biometrica. 

'  Die  Stellung  der  Verwandtenehe  in  der  Pssychologie  der  Xeurosen,  Jahrbuch  f. 
Psychoan.  u.  Psychopath.  Forschungen,  1909. 

'  Bleuler:  Mendelismus  bei  psyehosen.  speciel  liei  der  Schizophrenie,  Arch,  suisses 
de  Neurol.,  No.  1,  vol.  i.  Riidin:  Studien  iiljer  Vererbung  und  Entstehuug  geistiger 
Storungen,  1916. 


26     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

to  record  the  fact  "insane"  without  any  definite  knowledge  of  the 
particular  mental  disturbance.  It  is  important  to  reiterate  that  the 
old  Morel-Zeller  view,  that  all  mental  disturbances  are  one  disease  with 
different  stages  is  absurd.  Insanity  as  a  disease  entity  has  no  existence. 
One  should  be  as  specific  as  possible,  for  there  is  a  distinct  tendency  for 
special  types  to  be  passed  down  as  dominants.  Mental  peculiarities 
should  be  noted:  great  avarice,  originality,  queerness,  eccentricities, 
great  capability,  marked  incapacities,  temperamental  peculiarities,  ina- 
bility of  husband  and  wife  to  get  along,  suicides  (valuable  as  evidence 
only  if  cause  be  known),  all  these  may  be  of  considerable  service  in 
offering  a  clue  to  many  different  cases,  in  psychoneuroses  and  psychoses 
particularly.  The  occurrence  of  the  hysterical  type  of  reaction  in  the 
parents  or  in  the  brothers  or  sisters  should  not  be  overlooked,  but 
deductions  therefrom  should  be  founded  on  precise  criteria,  not  hap- 
hazard guessing. 


II 


-I       -   .    .1  n  n   71    n  rmi.  I 


i'i46^n 


21  3|    4       5i    6 

III       all  N  allN  all  N 

"  ^—  ^—      -     '    •     ■■  numerous 

decendants 
all  normal 

IV 


Fig.  2. — Pedigree  of  a  family  showing  Huntington's  chorea.  Affected  persons  (indi- 
cated by  black  symbols)  are  always  derived  from  affected  parents.  From  original  data 
furnished  by  Dr.  S.  E.  Jelliffe;  Smi  family.     (Davenport.) 

Definite  nervous  disorders  that  need  investigation  in  the  ancestry 
are,  so  far  as  known,  but  comparatively  few.  The  more  important 
are  tics,  myoclonias,  Huntington's  chorea,  Thomsen's  disease,  myop- 
athies, certain  congenital  brain  defects,  as  cerebellar  defects,  speech 
defects,  skin  defects,  optic  defects  (color-blindness).  Careful  history- 
taking  will  undoubtedly  reveal  other  defect  neiu-oses,  for  the  very 
fundamental  study  of  Karplus  (loc.  cit.)  has  shown  that  brain  form, 
brain  cytotectonic,  cord  form,  cord  myelotectonic,  etc.,  are  directly 
transmitted.  Certain  occupation  neuroses,  some  migraines,  many 
eye-strains,  etc.,  are  possibly  due  to  the  continuous  transmission  of 
structural  variations.  ^ 

Epilepsy. — The  occurrence  of  epilepsy  in  the  ascendants,  as  epilepsy, 
means  little.  When  both  parents  are  known  to  have  the  syndrome  the 
chances  of  transmission  of  a  defect  are  great.  Epilepsy,  like  many 
another  disease  so-called,  is  resolvable  into  many  different  disorders. 
The  convulsive  seizure  is  only  an  end-result  of  a  vast  variety  of  ante- 

'  Compare  Adier:  Die  Minderwertigkeit  der  Organen,  Organ  Inferiority,  Nerv.  and 
Ment.  Monograph  Series,  No.  24. 


HISTORY  OF  PATIENT  27 

cedent  events  in  only  a  few  of  which  can  hereditary  factors  be  said  to 
be  important. 

AJcohoHsm. — Alcoholism,  if  excessive,  should  never  be  overlooked. 
If  possible  the  cause  should  be  recorded,  for  in  the  last  analysis  alcohol- 
ism is  to  be  regarded  as  the  individual's  attempt  to  escape  certain 
difficulties.  Alcoholism  may  mean  hysterical  or  compulsive  reactions. 
It  may  be  a  schizophrenic  symptom.  Periodicity  in  drinking  should 
be  carefully  inquired  into,  particularly  in  its  relation  to  the  cyclothymic 
constitution  (manic-depressive  psychosis). 

Migraine .—T\\Q  hereditary  factor  in  migraine  is  much  exaggerated. 
The  frequency  of  the  disease  has  served  to  bring  about  this  confusion.^ 

Tuberculosis  and  Diabetes. — Tuberculosis,  diabetes,  tendency  to 
arthritic  disturbances  are  factors  in  heredity,  the  exact  significance 
of  which  it  is  hard  to  estimate;  tuberculosis  also  especially,  because  of 
its  extreme  frequency.  The  presence  of  diabetes  in  a  parent  should 
not  be  overlooked.  It  seems  to  play  a  large  role  in  nervous  and  mental 
disorders. 

Syphilis. — Syphilis  as  an  antecedent  factor  should  never  be  disre- 
garded. Not  only  does  one  meet  with  congenital  tabes,  paresis,  hydro- 
cephalus, optic  atrophies,  deafness,  etc.,  but  evidence  is  accumulating 
that  reinforces  the  belief  that  a  syphilitic  heritage  is  responsible  for 
much  nervous  and  mental  disorder,  of  a  less  tragic  though  perhaps 
more  annoying  character  than  those  just  mentioned.  Objective  evi- 
dences of  a  syphilitic  inheritance  should  be  looked  for  in  the  teeth, 
bony  formation,  pelvic,  chest,  and  cranial  contours,  etc.  Wassermann 
tests  of  the  blood  of  suspected  parents  may  even  be  necessary  to  clear 
up  a  diagnosis,  say  of  a  meningeal  disturbance  of  hidden  etiology  in  an 
infant,  child,  or  even  young  adult.  The  evidence  of  congenital  Wasser- 
mann reactions  is  still  too  undecided  at  this  date  to  permit  one  to  be 
satisfied  with  the  results,  especially  if  negative,  obtained  from  the  blood 
of  the  patient. 

II.  History  of  Patient. —  Birth:  when  learned  to  walk,  talk  and  read. 
Childhood  diseases,  particularly  with  reference  to  constitutional  dis- 
turbances following — mumps  and  testicles,  ovaries,  or  other  endocrine 
involvement — childish  habits,  enuresis,  nail-biting,  thumb-sucking, 
sleep-walking,  stammering. 

Endocrinopathic  inheritance  is  of  much  moment,  which  is  discussed 
later  in  the  endocrinopathy  questionnaire  (see  later).  • 

Diseases  (especially  convulsions,  delirium,  head-injury,  gonorrhea, 
syphilis,  rheumatism,  neuritis). 

Habits  (alcohol,  drugs,  masturbation,  character  of  erotic  impulses). 

Accidents   (gunshot  injuries,  tropical  diseases,  poisonings). 

Marriage:  Menstruation;  gynecological. 

Previous  attacks  (special  attention  to  so-called  hysterical,  to  nervous 
breakdown,  and  to  melancholic  periods). 

Crimes  and  misdemeanors. 

'  See  chapter  on  Migraine  in  Osier:  Modern  Medicine,  1915,  second  edition,  vol.  v. 


28     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 


Note. — The  history  of  the  early  life  of  the  patient  is  especially 
important  in  order  to  obtain  a  comprehensive  idea  of  the  sort  of 
person  the  patient  was  before  becoming  ill.  A  given  disorder  of  the 
nervous  system  cannot  be  fully  understood  without  understanding, 
not  only  the  circumstances  that  gave  rise  to  it,  but  the  other  and  more 
important  factor,  the  make-up  of  the  individual  in  whom  the  disorder 
occurs. 

The  objective  examination  passes  on  to  the  birth  of  the  patient. 
Was  it  normal,  or  instrumental,  or  of  excessive  length?  Hemorrhage, 
accidents,  or  pressure  palsies  may  thus  receive  their  interpretation. 

Did  the  child  learn  to  ivalk  at  an  average  period,  i.  e.,  from  nine  to 
fifteen  months  (Preyer),  and  if  not  were  there  definite  facts — excessive 
weight,  intercurrent  disease — to  explain  the  tardiness?  Was  there 
precocity?     Endocrinous  disorder  usually  underlies  great  precocity 


Fig.  3. — Congenital  syphilis  "unto  the  third  generation."  Syphilitic  father  of  first 
generation.  Second,  three  premature  births,  two  dead  children,  and  one  dying  at 
sixteen  months.  Congenital  syphilitic  next,  with  Hutchinson  triad.  This  patient 
married,  and  had  a  child  with  snuffles,  who  died  at  age  of  six  weeks.  Remaining  sister 
had  infantile  hemiplegia,  Hutchinson  teeth,  and  keratitis.     (Mott.) 

in  body  development.  Was  speech  acquired  early  or  late?  and  did 
the  child  learn  to  read  at  the  usual  period  (five  to  eight  years,  according 
to  opportunities  afforded)?  These  facts  are  of  much  importance, 
especially  in  Estimating  mental  capacity,  and  for  young  children  the 
probabilities  concerning  development  and  the  need  for  special  training. 
Children  who  learn  to  walk  and  talk  as  late  as  twenty- six  to  thirty- 
eight  months  respectively  often  remain  very  backward.  The  relations 
of  speech  to  mental  development  are  extremely  close.  Special  tests, 
as  the  Binet- Simon  or  other  special  scales,  are  essential  in  placing  the 
intellectual  age  of  the  child.  Emotional  difficulties  are  often  causes  of 
psychogenic  blocking,  which  are  not  revealed  with  intelligence  tests 
and  are  misinterpreted. 

Difficulties  in  teething,  especially  when  attended  with  convulsions, 
should  be  noted.    The  rachitic  chest  form  should  not  be  overlooked. 


ADULT  DISEASES  29 

Concerning  children  s  diseases,  much  can  be  said.  Severe  measles, 
or  scarlet  fever,  or  other  disorders  may  lay  the  foundation  of  a  later 
developing  epilepsy;  diphtheria  may  cause  various  forms  of  neuritis 
with,  at  times,  permanently  weakened  powers.  Tuberculosis  plays  a 
very  important  role.  The  importance  of  influenza  is  often  overlooked, 
while  the  relation  of  the  acute  streptococcic  infections  and  their 
resulting  joint  involvements  to  chorea  is  well  established.  Excessive 
fatigue  or  exhaustion,  either  from  an  infectious  disease,  apart  from 
its  toxemia,  or  from  overexercise  or  strain  in  rapidly  growing  children, 
bears  a  close  relation  also  to  choreic  reactions.  The  infections  also 
affect  the  endocrinous  glands,  thyroiditis,  hypophyseal  cysts,  etc.,  and 
thus  ma^•  exert  a  great  influence  in  the  metabolism  of  the  growing 
child. 

Very  close  attention  should  be  paid  to  the  aural  affections  of  children, 
and  the  nasopharyngeal  cavities  should  be  scrutinized  for  adenoids  or 
other  foreign  bodies  that  interfere  with  free  respiration,  sound  sleep 
or  the  proper  hygiene  of  the  nasopharyngeal  mucous  membranes. 

Intestinal  worms  should  not  be  overlooked.  They  may  be  the  cause 
of  infantile  convulsions  or  of  milder  neurotic  disturbances. 

Enuresis  should  never  be  overlooked.  If  continuing  past  the  third 
or  fourth  year  it  aflFords  valuable  evidence  of  a  neurotic  predisposition. 
Thumb-sucking,  nail-biting,  and  other  little  habits  may  be  included  in 
this  place.  Stammering  should  be  carefully  inquired  into,  sleep-walking 
also. 

The  education  of  the  patient,  especially  if  a  mental  disorder  or  a 
psychoneurosis  is  under  investigation,  should  be  very  thoroughly 
gone  into.  A  knowledge  of  the  earliest  impressions  gained,  the  picture 
books  used,  the  principal  childish  associations  formed,  special  tastes, 
animosities  or  dislikes  are  essential  to  the  understanding  of  the  obses- 
sional, hysterical  or  allied  reactions.  The  ideals  inculcated,  the 
religious  and  ethical  training  gained  in  the  early  years  usually  give  a 
definite  stamp  to  the  personality  and  must  be  known  if  the  adult 
personality  is  to  be  understood.  The  grosser  factors  of  the  classes 
passed,  and  the  schooling  received  are  absolutely  essential  in  estimating 
the  grade  of  later  mental  capacity,  and  the  application  of  intelligence 
tests  in  the  study  of  the  psychoses  or  psychoneuroses.^ 

Adult  Diseases. — Syphilis  stands  in  the  first  rank.  Care  sho  ild  be 
exercised  in  obtaining  a  syphilitic  history.  Did  you.  ever  have  a 
chancre?  is  the  usual  method  of  asking  the  question.  The  query, 
How  old  were  you  when  you  had  a  sore  on  the  penis?  although  perhaps 
more  abrupt,  will  give  a  higher  percentage  of  positive  answers,  espe- 
cially in  those  cases  where  its  previous  existence  is  largely  inferred,  as 
in  general  paresis  or  tabes.  If  the  direct  question  is  to  be  avoided, 
as  in  the  case  of  many  women,  married  or  otherwise,  the  questions 
concerning  syphilitic  symptoms  are  desirable — the  presence  of  symp- 
toms such  as  continuous  sore  throat,  hair-falling,  etc.    A  physical  ex- 

'  HuR-Helmuth:  The  Sexual  Life  of  the  Child,  Nervous  and  Mental  Disease,  Mono- 
graph series.  No.  29,  New  York  and  Washington,  1919. 


30     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

amination  for  mucous  plaques,  leukoplakia  of  the  mouth,  and  scars  on 
the  penis  should  never  be  omitted.  In  women  the  mucous  membranes 
of  the  cheeks  and  sides  of  the  tongue  should  always  be  examined. 

Further,  the  Wassermann  blood  reaction  should  be  obtained  in  all 
cases  where  any  doubt  exists.  Although  the  percentage  of  unknown 
syphilitic  infections  is  low,  nevertheless  such  exist.  A  single  Wasser- 
mann test,  positive  or  negative,  is  not  conclusive,  and  great  care  should 
be  taken  in  the  choosing  of  a  proper  serologist.  The  subject  of  technic 
is  a  complicated  and  immensely  important  one.^ 

The  subsequent  history  of  the  syphilitic  infection  is  desirable  and 
the  character  and  length  of  time  of  treatment  should  be  recorded. 

Gonorrhea  is  not  unimportant.  It  is  of  special  relevancy  in  all 
arthritic  disorders,  in  choreas  (vaginal  discharge)  in  young  children, 
and  in  meningeal  excitements.    Gonorrheal  neuritis  is  known. 

Arthritis  in  its  various  forms  calls  for  careful  observation.  Here 
one  would  best  record  observations,  and  not  attempt  a  diagnosis  of 
the  joint  conditions.  Tooth  infection  from  the  Streptococcus  viridans 
is  of  importance  as  a  chronic  infection  in  producing  several  neurological 
or  psychotic  syndromes. 

The  role  of  the  infections  in  mental  pathology  is  very  marked. 
Typhoid  fever  and  influenza^  both  constitute  severe  infections  with 
marked  influence  on  nervous  tissues. 

Under  shocks  is  included  sudden  mental  and  moral  influences 
tending  to  disturb  the  emotional  life.  Loss  of  money,  of  parents, 
husband,  children,  or  loved  ones,  interference  with  one's  hopes  of  a 
career,  unfortunate  entanglements,  all  call  for  investigation.  The 
great  importance  of  emotional  disturbance  in  all  nervous  reactions 
should  be  borne  constantly  in  mind. 

As  to  habits,  particular  attention  should  be  directed  to  alcoholism. 
It  plays  a  most  important  role  in  diseases  of  the  nervous  system. 
There  is  much  divergence  of  opinion  as  to  what  may  constitute  alcohol- 
ism. Accurate  recording  of  the  exact  amounts  consumed  will  afford 
the  student  the  best  criteria  by  which  he  later  can  judge  for  himself 
from  his  own  carefully  kept  records.  The  use  of  other  narcotics,  as 
opium  and  its  allies,  cocaine,  the  alcohol  h\^notics,  bromides,  etc.,  call 
for  record. 

Occupation  toxemias  should  not  be  overlooked.  They  are  daily 
assuming  increased  importance  in  America  by  reason  of  the  increasing 
niunber  of  dangerous  occupations.  Workers  in  lead,  arsenic,  mercury, 
copper,  pewter,  pottery,  dyes,  sulphur  compounds,  and  others  suffer 
often  from  obscure  symptoms,  due  to  chronic  poisonings.  Occupation 
fatigues  explain  many  neurasthenias. 

The  sexual  habits  should  be  inquired  into.  Liberal  indulgence  in 
masturbation,  etc.,  while  usually  self-corrective,  at  times  works  havoc 
with  the  nervous  system.    Sexual  abstinence  in  the  married  as  well 

•  Plant:  Wassernumn  Reaction  in  Neurology  and  Psychiatry,  Nervous  and  Mental 
Disease  Monograph  Scries,  No.  .5,  New  York,  1910.  Kaplan:  Serology  in  Nervous 
Disease.s.  Philadelphia,   1914. 

2  Jelliffe:  Influenza  and  the  Nervous  System,  New  York  Med.  Jour.,  1918. 


PRESENT  ILLNESS  31 

as  the  unmarried  is  at  times  an  important  element  in  the  neuroses 
(anxiety  neurosis).  The  rarer  anomalous  sexual  manifestations  call  for 
investigation  in  some  cases.  Genital  masturbation  is  not  the  only 
type  of  masturbation. 

Special  attention  should  be  paid  to  convulsive  seizures,  either  occur- 
ring in  the  young  or  in  adult  life.  Certain  facts  about  such  convulsions 
should  never  be  omitted.  These  are  the  presence  of  dizziness,  or  of 
objects  revolving  (direction  to  be  noted) ;  of  the  state  of  consciousness; 
whether  there  is  injiuy  to  the  body  during  such  attacks;  if  the  tongue 
be  injured;  if  urine  or  feaes  be  voided  during  the  attack;  and  if  there 
be  amnesia,  complete  or  partial,  following  the  attack. 

The  influence  of  violence  or  injury  to  the  body  is  often  of  extreme 
importance.  If  there  is  accompanying  mental  shock  the  fact  should 
not  be  omitted. 

The  general  constitution  of  the  patient — his  or  her  general  capacity 
for  work  and  fatigue — is  to  be  noted.  The  question  of  general  tem- 
perament, of  outlook  on  life,  may  be  tentatively  entered  in  this  place. 

In  the  case  of  women,  special  attention  should  be  directed  to  the 
menstrual  history.  The  number  and  character  of  the  births,  the 
health  of  the  children,  the  number  of  miscarriages  with  causes  should 
be  recorded. 

III.  Present  Illness. — Onset;  cause;  physical  pains;  disabilities;  mental 
and  moral  changes;  emotional  conditio?!;  hallucinations  and  delusions; 
judgment;  memory;  suicide  and  homicide;  insight. 

Note. — Under  this  head  an  inquiry  is  made  into  all  the  circumstances 
surrounding  and  conditioning  the  onset  of  the  disorder,  the  patient's 
attitude  toward  it  and  his  insight. 

Subjective  History. — The  patient's  own  account  of  his  illness  can 
either  precede  or  follow  the  outlines  of  the  family  history  and  his 
general  previous  condition.  The  history  is  recorded  in  the  question- 
naire on  the  side  of  the  blank,  thus  keeping  it  separate  from  the 
preceding  and  after-coming  facts. 

It  is  advisable  for  the  patient  to  fix  as  nearly  as  possible  the  date 
of  the  onset  of  his  illness.  Certain  facts  which  may  or  may  not  have 
had  any  connection  with  the  malady  under  study  ma>'  aid  in  fixing 
such  a  period  of  transition  between  health  and  sickness.  The  nature 
of  its  onset,  whether  acute  and  progressive,  or  acute  and  regressive, 
insidious  and  irregular,  or  very  severe  and  immediate.  What  did  the 
patient  notice  at  that  time?  Then  gradually  trace,  step  by  step, 
hour  by  hour,  day  by  day,  week  by  week,  or  year  by  year,  the  develop- 
ment of  the  disorder.  What  new  symptoms  have  been  added  to  the 
first — what  have  disappeared;  has  the  picture  remained  the  same,  or 
has  it  gradually  or  suddenly  altered? 

A  methodical  going-over  of  the  locomotor,  sensor^',  emotional,  intel- 
lectual, skin,  digestive,  respiratory,  and  secretory  systems  should 
follow  the  patient's  own  account  of  the  difficulty.  Pains  felt  should 
be  roughly  charted  and  carefully  located.  If  certain  symptoms  have 
disappeared,  attention  should  be  paid  to  the  mode  of  their  onset  and 
of  their  departure.     It  is  important  to  ascertain  just  what  effect  the 


32     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

illness  has  upon  the  social,  familial,  physical  or  psychical  life  in  order 
to  estimate  the  severity  of  certain  symptoms.  Also,  has  the  patient 
consulted  other  physicians,  or  has  he  visited  institutions  or  other  than 
trained  medical  men  of  various  kinds?  What  was  the  treatment? 
Its  effect? 

A  thorough  subjective  anamnesis  is  one  of  the  most  difficult,  and  at 
the  same  time  the  most  important  features  of  a  neurological  or  mental 
examination,  particularly  the  latter.  If  the  gradual  evolution  of  the 
disorder  is  carefully  elucidated,  there  is  little  danger  of  going  wrong. 
If  one  hops  from  one  thing  to  another,  however,  mistakes  will  be 
frequent. 

IV.  General  Observations.  —  Facial  expression;  appearance  and 
demeanor;  movements;  disabilities;  gaits;  anomalies  of  innervation; 
speech;  mental. 

Note. — The  general  observd,tion  of  the  patient  is,  of  course,  always 
important:  whether  he  appears  silly,  resentful,  indifferent;  whether 
he  has  mannerisms,  etc.  It  is  particularly  important,  however,  in 
stuporous  and  delirious  patients  who  either  will  not  speak  or  are  not 
responsive  and  therefore  not  accessible.  These  patients  should  be 
observed  particularly  as  to  their  general  attitude  of  body  and  limbs, 
the  expression  of  the  face,  the  reflexes,  and  the  reactions — volitional 
emotional,  and  organic  (hunger,  sexual,  responding  to  calls  of  nature 
etc.). 

The  Objective  Examination.  —  During  the  subjective  examination 
many  facts  concerning  the  general  attitude  of  the  patient  have  been 
gained.  His  expression  and  carriage,  in  bed  or  able  to  be  about, 
the  character  of  his  intelligence  and  the  responsiveness  of  his  answers. 
Is  his  mind  clear  and  is  he  oriented? 

V.  Physical  Examination. — Form;  nutrition;  weight;  height;  shin; 
bones  and  joints. 

Decubitus;  scars  (especially  penis  and  mouth). 

Respiratory  system. 

Circulatory  system;  heart  position,  size  and  sounds;  blood-pressure. 

Genito-urinary  system. 

G astro-intestinal  tract;  stomach  content  (if  indicated) ;  glands;  abdomen. 

Sputum  (if  indicated). 

Blood  composition  (if  indicated). 

Cerebrospinal  fluid  (if  indicated). 

Urine  (always). 

Note. — It  is  hardly  necessary  to  insist  upon  the  necessity  for  a 
thorough  physical  examination  in  every  case.  It  is  especially  impor- 
tant in  the  deliria  in  which  the  mental  disorder  may  be  the  expression 
of  an  obscure  physical  condition. 

As  has  already  been  noted,  a  scheme  is  of  great  advantage,  for 
beginners  particularly.  With  increasing  experience,  one  may  depart 
from  a  hard  and  fast  method  of  case-taking,  but  in  the  beginning 
the  student  should  habituate  himself  to  a  rigid  and  exhaustive  method 
if  he  would  avoid  careless  work. 

The  cranium  should  first  be  inspected.     Is  it  regularly  shaped,  or 


VEGETATIVE  SYSTEM  EXAMINATION  33 

is  there  assymmetry?     Measurements  of  the  vertical,  binauricular, 
and  horizontal  diameters  should  be  taken. 

The  position,  general  form,  and  character  of  the  ears  should  be 
noted,  and  the  arch  of  the  palate  observed.  The  occurrence  of  isolated, 
or  even  several,  so-called  signs  of  degeneration  may  be  recorded. 
There  is  no  necessary  connection  between  such  anomalies  and  nervous 
or  mental  disease.  They  are  found  in  superior  as  well  as  inferior 
deviates.  The  departure  from  the  average  is  worthy  of  record,  but 
the  hasty  generalizations  of  the  Lombroso  school  should  be  avoided. 
These  deviations  from  the  average  structures  will  be  discussed  later. 

Careful  and  thorough  percussion  of  the  skull  may  reveal  local 
points  of  tenderness  (brain  tumor),  etc.  The  presence  of  cicatrices 
(epilepsies),  depressions  (fractures),  or  abnormal  elevations  is  to  be 
noted.  In  special  cases,  .r-ray  examination  of  the  skull  is  of  great 
value,  and  should  always  be  made  for  suspected  fractures,  for  many 
brain  tumors  (acromegaly),  etc. 

VI.  Vegetative  System  Examination. — A  systematic  presentation  of 
methods  for  examining  the  vegetative  reactions  is  only  just  being 
formulated.  The  vegetative  system  consists  of  two  more  or  less 
opposed  systems,  the  autonomic,  or  extended  vagus  system,  and  the 
sympathetic  proper.  A  hyperactive  autonomic  type  of  reaction  has 
been  termed  by  Eppinger  and  Hess,  vagotonic;  of  the  sympathetic, 
sympathicotonic.  These  two  contrasting  types  show  a  number  of  char- 
acteristic anomalies  which  are  fairly  constant  and  capable  of  objective 
examination.  Not  only  are  there  a  variety  of  anomalies  of  the  auto- 
nomic and  sympathetic  reactions  to  be  observed  but  a  host  of  clinical 
signs  are  known  which  are  dependent  upon  disorder  of  the  metab- 
olism of  different  parts  of  the  body  due  to  disturbances  of  the  glands 
of  internal  secretion,  endocrinopathies.  ^lany  of  these  are  very  marked 
as  in  myxedema,  or  acromegaly  for  instance;  others,  however,  are  less 
striking,  but  close  observation  will  reveal  a  great  many  metabolic 
variations  due  to  a  uniglandular  or  polyglandular  defect. 

It  should  always  be  held  in  mind  by  the  observer  that  this  poly- 
glandular interaction  may  have  produced  marked  anomalies  of  struc- 
ture and  }et  may,  at  the  time  of  observation,  be  no  longer  operative; 
that  is  compensation  may  have  become  efl'ective  and  the  structural 
anomaly  or  anomalies  remain  as  a  witness  to  a  struggling  series  of  inter- 
relating compensations.  On  the  contrary,  and  this  is  even  more 
important,  a  polyglandular  disturbance  may  be  operating  and  yet  not 
sufficiently  advanced  to  produce  structural  anomalies.  In  all  events 
the  minute  testing  out  of  each  endocrine  function  is  imperative. 
Another  extremely  important  consideration  should  always  be  in  mind. 
It  is  not  always  the  most  obviously  overstrained  glandular  activity 
that  may  be  primarily  at  fault.  Thus  an  obviously  hyperthyroid 
disturbance  may  only  be  an  over-com))ensat()ry  activity  for  an  under- 
functioning  thymus,  or  other  glandular  defect.  Many  a  thyroid  has 
been  foolishly  removed  because  of  failure  to  recognize  the  interaction- 
ism  which  is  constantly  going  on  in  the  human  body. 

We  here  append  an  endocrine  status. 
3 


34     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

ENDOCRINE  STATUS. 


Heredity,  Endocrinopathic : 

Descriptive  details : 

Giants : 

Dwarfs : 

Goitres : 

Diabetes: 

Structural  Peculiarities : 

Familial  Diseases: 

Skeletal  Type: 

6°  +: 

5°5'-: 

Arm  Leg  Ratio : 

Trunk  Leg  Ratio : 

Hands :                 Fingers : 

Toes: 

Lordosis: 

Kyphosis: 

Growth:    Curve: 

Bony  Consistency. 

A'-ray 

Facial  Types : 

Acromeg : 

Acromic : 

Cretin : 

Myxedem : 

Gravian : 

Addison : 

Gerodermic : 

Eunuchoid : 

Thymic: 

Lvmphat : 

Mongolian : 

Ovarian : 

Head  Segments: 

1 

Hydroceph : 

Microceph : 

Jaw  Shape: 

Prognath : 

U: 

L: 

Teeth :                 Spaced : 

Absent : 

Lat.  Licisors: 

Canines: 

Color : 

Gingivitis: 

Dentition  Dates: 

VEGETATIVE  SYSTEM  EXAMINATION 
ENDOCRINE  STATUS— Continued 


35 


Skin.     Subcutaneous  Tissue: 

Color : 

Texture : 

Tonus :                 Pigm : 

Nevus : 

Atrophies : 

H^^ertrophies: 

Infilt: 

Fat: 

Eruptions :           Acne : 

Psor:           Ecz: 

Distribution : 

(Chart) 

Stroking  React : 

Gooseflesh : 

Urticaria : 

Blanching: 

Mottling: 

Hair:     Nails: 

Type:                  Color: 

Lustre : 

Texture :       Rough :       Fine 

:       Curly:       Dry: 

Distrib :                  Face : 

Body: 

Age: 

Develop : 

Eyebrows : 

Eyelashes : 

Ridging :                 Brittle : 

Spots: 

Growths: 

Mucous  Membranes: 

Nasal : 

Mouth: 

Atrophy : 

Hypertrophy : 

Pigment : 

Blanching: 

Aplasia : 

Hyper  pi: 

Tonsil  Persist : 

Tongue  Papilhe : 

Hay  Fever: 

Leucorr : 

Colitis : 

Asthma: 

Descriptive  details : 


36     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 
ENDOCRINE  STATES— Continued 


Muscles :    Ligaments : 

Massive : 

Sparse : 

Atrophies : 

Hypertrophies : 

Aplasia: 

Hyperplasia : 

Tonus : 

Carriage : 

Fatigability : 

Flat  Feet: 

Extensible  Joints 

Ptoses : 

Contractures : 

Sex  Character  Development : 

Male :    Size : 

Shape  Penis: 

Implant; 

Prepuce : 

Scrotal  Tonus: 

Testicle: 

Pubes : 

Pelvis: 

Hair  Male: 

Hair  Fem.: 

Beard: 

Body  Hair: 

Puberty  Age : 

Voice  changes : 

Breast : 

Erotism:                Ardor: 

Frigid : 

Homo  vs.  Hetero  Trends: 

Friends : 

Fecundity : 

Familial : 

Female:         Genitals  Size: 

Character : 

Clitoris: 

Labia: 

Uterus : 

Ovary : 

Hair  Male: 

Hair  Fem.: 

Beard: 

Body: 

Menst.  Time: 

Character : 

Voice  Variations: 

Erotism : 

Homo  vs.  Hetero  Trends: 

Friends : 

Fecundity : 

Familial : 

Descriptive  details: 


VEGETATIVE  SYSTEM  EXAMINATION 
ENDOCRINE  STA.T\]S~Continued 


37 


Cardiovascular : 

Descriptive  details : 

Blood  P.essu.e(°- 150^ 

IZHlo 

Rest: 

Exertion : 

Pulse  Pressure: 

Blush: 

Blood  Picture: 

Mottling: 

Aschner : 

Vessel  Palpat: 

Pharmacodynamic  Reaction  to 

Atropine : 

Pilocarp: 

Cocaine : 

Adrenalin : 

Mercury :           ^ 

Idiosyncrasies : 

Metabolism : 

Hypothermia: 

HjTperth : 

Frilosity : 

Hot  Flashes: 

Weather  Reactions : 

Cataract : 

Glaucoma; 

Blood  Sugar : 

Sugar  Tolerance : 

CO2  Quotient: 

X-ray  Examinations : 

Sella  Turcica: 

Pineal  Region : 

Sinuses : 

Teeth: 

Thymus : 

Thyroid: 

Palpation : 

P^piphyses : 

Bony  Structures: 

38    METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

In  this  section  attention  will  first  be  briefly  centered  upon  some  of 
the  objective  signs  to  be  looked  for  resulting  from  disturbances  of  the 
vegetative  reflexes,  and  secondly  to  those  more  closely  related  to  endo- 
crinous modifications.  The  two  series  of  observations  are  very  closely 
related  at  times.  More  thorough  discussion  of  the  syndromes,  or 
diseases  are  found  in  the  first  section  of  this  book  on  diseases  of  the 
vegetative  nervous  system  and  the  endocrinopathies. 

Vegetative  System  Examination. — ^\"agotonia  and  sympathicotonia.^ 
Mild  grades  or  trends  of  these  have  been  termed  the  vagotonic  and 
sympathicotonic  constitutions.  Speaking  generally,  they  are  the 
cold-blooded  and  the  warm-blooded.  The  vagotonic  (vagus  tonus  or 
sympathetic  paralysis)  shows  relative  insusceptibilit}'  to  sympathetic 
stimuli;  hence  cold  skin,  compressible  slow  pulse,  deep-set  eyes,  con- 
tracted pupils.  The  skin  is  apt  to  sweat  readily,  at  times  in  patches 
or  blotches ;  the  palms  are  apt  to  be  thick.  They  are  usually  under- 
nourished. Short-sightedness  is  frequent.  Acneiform  eruptions,  par- 
ticularly of  the  back,  usual.  They  frequently  swallow  as  they  talk, 
and  seem  to  have  much  saliva  in  the  mouth.  Sore  throats  are  frequent. 
The  faucial  reflexes  are  usually  diminished.  Increase  of  hair  about 
nipples  and  masculine  distribution  of  the  pubic  hair  is  frequent  in  the 
vagotonic  woman. 

Pilocarpine  test  shows  marked  hypersensitiveness  and  is  a  useful 
mode  of  examination.  This  is  to  be  given  hypodermically,  0.01  to 
0.003  gm.  Higier  recommends  0.0001  per  kilo  of  body  weight.  These 
tests  are  to  be  interpreted  like  all  others  solely  as  monosymptomatic 
and  weighed  with  other  signs.  Careful  records  should  be  made  of  the 
blood-pressure,  perspiration  and  pupillary  reactions,  every  10  minutes 
for  an  hour. 

The  trend  of  the  svTupathicotonic  (sympathetic  tonus  or  autonomic 
paralysis)  is  toward  the  warm-blooded  type.  Vivaciousness,  dilated 
pupils,  glistening  eye,  pulse  rapid,  skin  warm  and  dry.  There  is 
relative  insusceptibility  to  pilocarpine  and  also  to  atropine  (0.001  to 
0.00065  gm.),  while  adrenalin,  0.001  gm.  increases  all  of  the  signs 
present.  Cocaine  in  hypodermic  form  should  also  be  tested  out  in  the 
same  manner. 

It  is  to  be  borne  in  mind  these  are  trends  rather  than  fixed  types, 
and  that  an  individual  may  show  vagotonic  trends  in  one  or  more 
vegetative  segments  and  sympathicotonic  reactions  in  others.  These 
variabilities  are  pronounced  in  the  so-called  neurotic,  i.  e.,  the  indi- 
vidual with  marked  emotional  (psychogenic)  response. 

Pathological  reactivity  of  the  vegetative  systems  shows  itself  in  the 
eyes,  nose,  mouth,  skin,  respiration,  circulatory,  digestive,  urogenital, 
cutaneous,  and  metabolic  systems.     They  result  from  constitutional 

1  Eppinger  and  Hess:  Vagotonia,  translated  by  Jeliiffe  and  Kraus,  Nervous  and 
Mental  Disease  Monograph  Series,  No.  20,  New  York.  Also  consult  Barker  and  Slade: 
Tr.  Assn.  Am.  Phys.,  1912,  p.  471.  Higier:  Vegetative  Nervous  System,  Nervous  and 
Mental  Disease  Monograph  Series,  No.  29.  Laignel  Lavastine:  Internal  Secretions  and 
Nervous  System,  ibid.,  No.  30.  Rosenbloom:  Methods  of  Endocrine  Testing:  Interstate 
Med.  Jour.,  November,  1918  et  seq. 


VEGETATIVE  SYSTEM  EXAMINATION  39 

variations  (endocrinous  anomalies),  chiefly  iri  their  response  to  body 
cravings  which  come  partially  to  consciousness  as  emotional  reactions, 
/.  e.,  psychic  disturbances  (symbolic  systems,  unconscious). 

1.  Metabolic  Sign.s. — Variations  in  fat  and  sugar  tolerance,  eosino- 
philia,  pigmentations,  lymphocytosis. 

2.  Cutaneous  Signs. — Horripilation  (painful  hair  raising),^  goose 
flesh,  contractions  of  testicle  and  of  the  nipple,  seborrhea,  hypertri- 
chosis, baldness,  h^peridrosis,  bromidrosis,  local  syncopes,  acrocya- 
nosis, purpura,  pruritus,  pallor,  dermographism,  erythema,  urticaria. 


Fig.  4. — Lanugo  hair.     (Endocrinous  disturbances.)     (A.  Josefson.) 

3.  Respiratory  Signs. — Asthmatic  attacks,  laryngeal  spasms,  Asch- 
ner's sign  (oculocardiac  reflex),  pressure  upon  the  eyeball  leading  to 
slowing  of  the  pulse  with  stopping  of  respiration  in  expiratory  phase, 
coryzas,  bronchitides. 

4.  Circulatory  Signs. — Bra^lycardia,  tachycardia,  irregular  extra- 
systolic  pulse,  dromotropia,  vasomotor  anginas,  peripheral  anemia 
and  hyperemias,  acrocyanosis,  intermittent  claudication,  high  tension. 

5.  Digestive  Signs. — Colicky  diarrhea  (sympathicotonic),  spastic 
constipation  (vagotonic),  hyperchlorhydria,  achylia,  gastric  atony, 
pylorospasm,  gastrosuccorrhea,  esophagism,  hypersalivation,  entero- 
colitis. 

'  Andr6  Thomaa:  Reactions  anserines,  Presse  Med.,  July  6,  1918. 


40     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

6.  Genito-urinary . — ^Retention,  incontinence,  menstrual  anomalies, 
ejaculatory  disturbances,  tenesmus,  renal  colic,  priapism,  frigidity, 
loss  of  power. 

7.  Cranial  Autonomic  Signs. — Eyes:  Mydriasis  or  rayosis,  glaucoma, 
irregular  lid  slits,  spasms  of  accommodation,  ^^on  Graef 's  Moebius  sign, 
exophthalmos,  enophthalmos,  dryness  of  eyeballs,  Loewi's  test  (adrena- 
lin mydriasis — adrenalin  by  conjunctiva,  1  to  1000  solution).  Irregular 
pupils,  Stelwag's  sign  (irregular  and  infrequent  winking) ;  nose  signs 
are  dry  nose,  or  excessive  secretion  (hay  fever),  frequent  spitting  or 
very  dry  mouth. 

The  influence  of  various  drugs  upon  different  branches  of  the  two 
systems  is  shown  in  page  136. 

These  various  anomalies  should  be  looked  for  in  summing  up  the 
vegetative  reactivities. 

Endocrine  Status. — The  chief  observations  which  are  to  be 
recorded  are  given  in  the  endocrine-status  questionnaire  (see  pp.  34  to 
38).  The  various  syndromes  are  described  later.  The  morphological 
examination  includes  the  endocrine  inheritance,  observation  of  skeletal 
type,  characteristic  facial  developments,  variations  in  development  of 
the  head  segments,  special  attention  being  paid  to  the  teeth  develop- 
ment, skin  anomalies,  hair,  nails,  mucous  membranes,  muscles,  liga- 
ments, sex-character  development,  cardiovascular  reactions,  pharma- 
codynamic reactions,  general  metabolic  changes  and  .x-ray  examinations 
of  the  head  and  special  glands.  These  are  all  systematically  grouped 
on  pages  34  to  38,  a  more  detailed  consideration  following. 

Fades. — Acromegalic,  cretinistic,  myxedematous,  exophthalmic, 
Addisonian,  eunuchoid,  gerodermic,  mongoloid,  infantile,  juvenile, 
lymphatic,  chlorotic,  adenoid,  ovarian  face  of  Spencer  Wells. 

General  Develojjment. — Weight  and  stature,  excessive  obesity  or 
thinness.  Microsomia,  macrosomia,  disproportionate  development  of 
upper  and  lower  extremities,  of  the  distal  and  proximal  limb  lengths. 
Epiphyseal  calcifications,  cervicodorsal  kyphosis,  genu  valgum. 

Genital  Development. — (a)  In  male:  External  genitals,  descent  of 
te!Sticles,  beard,  distribution  of  pubic  hair  and  hair  of  body,  voice, 
fecundity  or  sterility,  psychosexual  characters,  ardor,  eroticism,  signs 
of  feminism,  overarching  scrotal  folds  simulating  clitoris  implanta- 
tion of  penis,  precocious  or  retarded  puberty,  (b)  In  female:  External 
and  internal  genitals,  breasts,  pubic  hair  formation,  male  type  rising 
in  middle  line,  female  type  straight  across,  menstruation,  ardor 
eroticism,  satisfaction  in  sexual  act,  psychosexual  characters,  fecundity 
or  sterility,  masculine  signs,  precocious  or  tardy  menstruation,  meno- 
pause, character  of  pregnancy. 

Examination  of  trophic  changes  or  disturbances. 

(a)  In  Skin. — Myxedematous  infiltrations,  scleroderma,  pigmen- 
tation (dyschromia),  circumscribed  or  dift'used,  activity  of  vesicants, 
hypertrophies,  atrophies,  and  precocious  senile  changes,  anidrosis, 
hyperidrosis,  eruptions,  chronic  ulcerations,  circumscribed  lipomatosis, 
adiposis. 


VEGETATIVE  SYSTEM  EXAMINATION 


41 


(b)  Hair  and  Nails. — Character  of  hair:  rough,  fine,  curly,  dry,  color; 
eyelashes  and  eyebrows.  General  hairiness — distribution.  Crescents 
in  nails,  character  of  nail  developments,  splits,  spots,  and  ridges. 

(c)  Teeth. — Primary  and  secondary  dentition,  dental  forms,  dental 
resistances,  ridges,  early  caries;  color,  chalkv-white  or  yellowish. 
Wide  implantation,  lateral  incisors,  canine,  shape  of  palatal  arch 
high  (thyroid?)  broad  (h\T)ophyseal?). 

(d)  Mucous  Membranes. — Pigmentation,  hyperplastic,  atrophic  or 
catarrhal,  lingual  hypertrophy,  ridges,  etc. 

(e)  Lens. — Early  cataract,  opacities,  floating  bodies  in  vitreous. 

(/)  Ligaments,  Muscles,  Bones. — Atrophies,  abnormal  fragility, 
osseous  growths,  hypertrophy  of  bone,  joints,  muscular  atrophy,  hyper- 
trophy, rheumatismal  swellings,  epiphyseal  anomalies. 


Infantile  genitals  from  hypo-ovarism.     (A.  Josefson.) 


(g)    Blood. — Blood  count,   eosinophilia,  lymphatic  hyperplasise. 

(h)  Chemoregulatory. — Albumin,  phosphorus,  iodin,  sugar  toler- 
ance, calcium  and  magnesium  metabolism,  respiratory  gases,  alveolar 
carbon-dioxide  tension.     Hyperthermia,  hypothermia. 

Vegetative  nervous  system,  see  pp.  38,  39  and  40. 

Direct  examination  of  endocrinous  glands: 

Hyjmphysis:  Sella  turcica  by  .r-rays,  signs  of  hypophyseal  tumor, 
results  of  hypophyseal  extract.  The  character  of  the  sella  turcica  is  of 
great  importance  in  determining  the  hypophyseal  capacity.  Erosion 
of  the  clinoid  processes  to  permit  enlargement  of  the  hypophysis  is  a 
frequent  finding. 

Thyroid:  Size  (20  to  30  gms.  average),  goiter  and  its  character, 
retrosternal  goiter,  thyroid  antibodies,  thyroidine  sensibility. 

Thymus:  Radioscopy  of  region,  tumor  of  superior  mediastinum, 
th\Tnic  antibodies. 


42     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

Suprarenal :  Circulating  adrenalin  dosage,  pain  in  suprarenal 
region,  tumor,  sensibility  to  adrenalin,  adrenalin  mydriasis,  pigmen- 
tation. Sergents  white  line.^  This  is  best  tested  by  marking  out  with 
the  finger  tips,  an  oblong  or  square  about  the  umbilicus;  light  pressure 
being  used.  These  first  results  a  slight  goosefleshing  in  from  ten  to 
twenty  seconds,  this  is  followed  by  a  blanching  of  the  area  which  is 
wider  than  the  pressure  area.  This  white  line  persists  in  depressed 
suprarenal  states  and  from  a  variety  of  exhausting  causes — from  two 
to  five  minutes  or  more.  The  usual  reaction  is  a  pinkish  coloration, 
which  in  some  white  lines,  marks  their  border.^ 

Genital  Glands:  Modification  in  size,  neoplasms,  effect  of  extracts. 

Pineal:  Signs  of  tumor.     Pineal  shadow  on  a:-ray. 

Pancreas:  Sugar  in  urine. 

VII.  Sensorimotor  Examination. — Cranial  Nerves. — These  should  be 
systematically  tested.  (See  questionnaire  made  out  for  a  patient  with 
tabes,  pp.  44  to  47.) 

I.  Smell. — There  are  no  satisfactory  quantitative  tests  for  smell 
apart  from  special  physiological  psychological  tests  that  are  of  service 
in  research  work  only.  The  smell  in  each  nostril  should  be  tested 
separately,  preferably  by  some  well-known  substance  (oil  of  turpen- 
tine) and  by  a  substance  resembling  well-known  foods  (asafetida, 
onions)  or  bodily  excretion  (feces) .  The  nostril  of  one  side  is  stopped, 
and  with  the  eyes  closed  the  patient  is  asked  to  smell  from  a  bottle 
containing  the  odorous  substance;  the  other  side  is  then  tested  with 
the  same  or  different  substance  and  comparisons  are  made.  Variations 
in  smell  are  very  frequent,  and  much  care  must  be  exercised  in  drawing 
conclusions  from  smell  anomalies.  Influenza  interferes  with  smell  tests. 
Local  conditions,  empyema  of  the  antrum,  etc.,  must  be  excluded. 
Irritating  substances,  like  ammonia,  etc.,  should  not  be  used.  A  search 
for  subjective  smell  disturbances  may  be  made  at  the  same  time. 
Anosmia,  unilateral  or  bilateral,  is  often  present  in  fractures  of  the 
skull,  in  frontal  lobe  brain  tumors  and  in  certain  epilepsies. 

II.  Eyes. — Sight  should  be  tested  by  the  Snellen  or  other  tj'pe  cards. 
These  should  be  well  illuminated  and  the  patient  should  stand  with 
the  window  light  behind  him.  Each  eye  should  be  tested  separately. 
Patients  unable  to  distinguish  the  largest  letters  should  be  tested  as 
to  their  ability  to  see  the  fingers,  determine  light  and  dark.  Some 
patients  see  better  in  a  dim  light  than  in  bright  light  (hemeralopia) 
(see  Plate  V,  p.  252,  for  family  tree  of  patients  who  become  blind  in 
dim  light — night-blindness,  nyctalopia).  Myopias  and  astigmatisms 
are  important  to  bear  in  mind  in  testing  the  sight  functions. 

Color  vision  is  important.  Colored  w^ools  are  matched  as  to  shades 
in  the  full  daylight.  In  color-blindness,  if  of  the  red-green  variety, 
gray-  or  straw-colored  wools  are  selected.     In  total  color-blindness  the 

1  Sergent:  fitudes  cliniques  sur  I'insufEence  surrenale,  Collected  Papers,  Maloine, 
1914. 

*  Massalongo:  La  linea  bianca  di  Sergent  nella  insufficenza  surrenale,  La  Riforma 
Medica,  August  24,  1918,  xxxiv. 


SENSORIMO  TOR  EX  A  MIX  A  TIOX 


43 


individual  confuses  all  colors;  comparative  degrees  of  brightness  alone 
are  distinguished.  A  number  of  ridiculous  blunders  may  be  made  in 
testing  for  color-blindness  with  the  wool  tests. 


Lf/l  Eye 


Bi'j'^  ^  I « 


Fig.  6. — Crossed  amblyopia  in  a  case  of  hysteria.     (Stewart.) 

The  field  of  vision  should  be  tested  with  avoidance  of  suggestive 
factors.  This  may  be  done  with  a  perimeter,  which  frequently  intro- 
duces the  error  of  suggestion,  or  the  rapid  finger  test  employed. 
The  patient  sits  about  three  feet  from  the  examiner.  Each  eye  is 
tested  separately.  The  patient  puts  his  hand  over  his  left  eye,  and 
looks  fixedly  at  the  examiner's  left  eye,  the  right  eye  being  closed. 
With  the  left  hand  held  midway  between  patient  and  examiner  some 
distance  to  one  side,  it  is  then  gradually  brought  toward  the  middle 


Riifht  Sj,t 


Fig.  7. — Right  homonymous  hemianopsia  in  a  case  of  softening  of  the  left  occipital  lobe. 

(Stewart.) 

line,  the  fingers  being  waved  slightly.  The  patient  is  directed  to  say 
"now"  as  soon  as  he  catches  sight  of  the  slightly  waving  fingers. 
P^our  axes  should  be  tested.     Variations  from  the  examiner's  own 


44     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SY'STEM 


No.  14^50. 


Diagnosis:  Tabes. 


NAME,  J.  Jones.  Age,  4£. 

Residence,  444  Spring  St. 
Date,  Jan.  5,  1910. 
Referred  by  Dr.  X. 


Occup.:  Clerk.  M.S.W.[D.] 
Race,  U.  S.  In  U.  S.  42  yrs. 
Examined  by  Dr.  J. 


Parents  related :  0 
Mental:  0 
Nervous :  0 
Epilepsy :  0 
Diabetes :  0 
Syph.:0 
Eruptions:  f 
Read :  6  years. 


Heredity: 

F.,  d.  70;  apoplexy. 

M..,  d.  64;  cancer. 

Children:  Only  child 

TBC:0 

Alcohol:  0 
Birth:  Normal. 
Walk:  N.     Speak:  N. 
Children's  Diseases :  Measles. 
Enuresis:  0    Thumb :0     Nail-biting,  etc. :  0 
Sleep-walking:  0  Stammering:  0 

Other  Childish  Traits:  Cheek-biting. 
Education:  Pub.  Sch.,  High  Sch.,  to  17  years. 
Adult  Diseases: 

Syphilis:  26  years.  Treatment:  1  month, 

Shocks :  0  Internal:  Hg. 

Habits:  Ale:  +      Tob.: +  + 

Sex:  Moderate  Indulgence. 

Trauma:  0 

Occupation  Toxemias:  0 

Convulsions  (injury,  tongue,  urine) :  0 
Constitution:  Healthy.  Weight:  150. 
Marriage:  At  32. 

Menses: 

Children:  1;  d.  in  infancy  (convulsions). 
Mk.:2;  4mos.  Dead:  0 


History : 

Chief  complaint: 
Slight  unsteadi- 
ness in  gait  and 
severe  "rheumatic" 
neuralgic  pains  in 
lower  extremities 
for  past  four  years. 
Five  years  ago  had 
a  transitory  attack 
of  dizziness,  with 
double  vision  and 
an  eye  palsy,  cross- 
eye,  which  lasted 
two  months. 

Occasional  weak- 
ness of  the  bladder, 
dribbling.  Pares- 
thesia occasional. 


First  page  of  Sensorimotor  Questionnaire:    Made  out  from  exami- 
nation of  a  patient  with  Tabes. 

0  =  negative.     +  =  present,  positive.     N  =  normal  or  average. 


SENSORIMOTOR  QUESTIONNAIRE 
SENSORIM OTOR  STATUS— Continued. 


45 


I.  Smell: 


11.  Sight: 


Bin.,  N.     Vert.,  N. 
Deformity:  0 


Subjective:  0 
O.K. 

Position  of  Eyes:  O.K. 


Cranium:    Hor.,  N. 
Sensibility:  0.  A'. 
Asymmetry :  0 
Cranial  Nerves : 

01.  ter. :  0.  K 
Asafet, 
R.  20/40 
L.  20/100 
Hemianopsia :  0       Scotomata :  0 
Fundus:  0  Fields:  Limited;  con. 

III.,  IV.,  VI.  Eye  movements:  0.  K. 
Nystagmus :  0         Palp.  fiss. :  R  =  L. 
Diplopia:  0;  5  years  ago.     Ptosis:  + 
Pupils:  R  ~>  L,  9  and  Jf.  mm. 

Light:  Lost  L;  dim.  R. 
Accom. :  0.  K. 
Symph. :  Dim.  R  ^  L. 
[  Consensual:  Lost  L-^R. 
Oculocardiac  reflex:  0.  K. 
V.  Motor:  0.  K. 

Sensory:  0.  K.        Jaw-jerk:  0.  K. 
Tender  spots :  0       Cornea :  0.  K. 

Conjunctiva:  0.  K. 
VII.  At  rest:  0.  A.     R  =  L. 

Forehead:  O.K.    Eyes  closed:  0.  K. 
Teeth:  O.K.        Whistle:  0.  A'. 
Involuntary:  0.  K.     Rosenbach:  0.  K. 
VIII.  Hearing:  Dim.         Equilibrium:  0.  K. 
Rinne:  +  Weber:  + 

Tinnitus:  0  Vertigo:  f 


Reflexes : 


History — Contin'd. 


0.  K.  =  average. 


46     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 
SENSORIIMOTOR  STAT\]S— Continued. 


Cranial  Nerves  (continued): 

IX.,  X.,  XL,  XII.  Fauces:  0.  K. 


Speech:  0.  K. 
Tongue :  Straight. 
Scars:  0 
Tremor:  0 
Taste:  0.  K. 
Shoulders:  0.  K. 
Upper  Extremity : 
Atrophy:  0 
Hypertrophy :  0 
Spasm:  0 
Hypotonus:  -\ — \- 
Muscular  power:  Dim. 
Dynamom. : 
Nerve  trunks :  Not  tender. 


Stammering:  0 
Swallow:  0.  K. 


Respiratory:  0.  K. 
Cardiac:  0.  K. 
Neck:  0.  K. 

Malformations :  0 
Symmetries:  0.  K. 

Twitching:  0 
R  =  L. 


History — Contin'd. 


Triceps  Rx.:  Dim. 
Tremor:  0         Rest:  0 
Ataxia:  +  F.  N.T.: 
Stereognosis :  0.  K. 
Light  touch:  0.  K. 
Position:  0.  K. 
Pain:  0.  K. 
Vasomotor:  0 
Hair,  pigmentation,  etc, 
Trunk: 
Power:  0.  K. 
Spine:  0.  K. 
Epigastric  Rx. :  + 
Cremaster  Rx. :  + 
Bladder:  Shiggish. 
Light  touch :  0.  K. 
Deep  Sens.:  Dint. 
Pain:  0.  K. 


Radial  Rx. :  Dim. 

Static :  0 
Atax.  F.  F.T. -.Atax. 

Adiadokok.:  0 
Diapason:  Dim. 
Thermal:  0.  K. 
Trophic:  0 


Deformity:  0 
Malposition : 
Abdom.  Rx.:  + 
Anal.  Rx. :  + 
Rectum:  Sluggish. 
Localization:  0.  K. 
Diapason:  Dim. 
Thermal:  0.  K. 


F.  N.  T.  =  finger-nose  test. 


F.  F.  T.    =  finger-finger  test. 


SENSORIMOTOR  QUESTIONNAIRE 
SENSORIMOTOR  STATUS— Continued. 


47 


Trunk  (continued) : 

History — C  ontin'd. 

Equilib.:  Unsteady. 

Asynergia:  +.    Slight. 

Vasomotor:  0 

Trophic : 

Dermographia :  0 

Pigmentation:  0.  K. 

Lower  Extremity: 

Deformity:  0 

Treatment : 

Atrophy:  +;  legs  flabby 

Intraspinous    in- 

Hypertrophy: 0 

Asymmetries :  0 

jections   of   salvar- 

Spasm :  0 

Kernig:  0 

saniz.ed   serum. 

Hypotonus:  +  + 

Tremor:  0 

(Swift-Ellis.) 

Muscular  power :  Bimmished. 

Course  of  mercurial 

Synergistic  tests :  R  =  L 

inunctions. 

Patellar:  0        Achilles: 

0        Clonus:  0 

Babinski:0       Chad:0 

Opp:0 

Nerve:  Tender. 

Lasegue:  Tender. 

L.  touch:  Dim. 

Pain:  0.  K. 

Deep:  Dim. 

Therm.:  0.  K. 

Localization:  0.  K. 

Position:  Impaired. 
Diapason:  Impaired. 

K.B.T.'.Atax. 

Romberg:  +  +  + 

Gait:  Markedly  ataxic. 

Closed  eyes:  Made  worse.     Sidewise: 
On  heels:  ++  On  toes:  +  + 

Asynergia:  0.  K. 

Vasomotor:  0  Trophic:  0 

Status  Corporis :  Medium  nutrition. 


Heart:  0.  K. 
Blood:  0.  K. 
Lungs:  0.  K. 
Abdomen:  0.  K. 
Liver:  O.K. 
Urine:  0.  K. 
Cerebrospinal  fluid: 
Wass. :  +  +        Cells :  50.      Globulin :  +  + 

A'.  H.  T.  =  knee-heel  test. 


Murmurs:  0 
Wassermann:  -\ — |- 
Arteries:  0.  K. 
Blood-pressure:  160. 
Skin:  0. /v. 
Joints:  0.  K. 


EXPLANATION  OF  PLATE  I. 

The   Pyramidal   Tract   in   its    Corticospinal   and    Cortico- 
nuclear Portions. 

The  corticomedullary  (corticospinal)  Co.M,  is  colored  light  red;  the  corticonuclear 
portion,  Co.N,  darker  red;  the  reticular  substance  (S.R.)  of  the  tegmentum  in  yellow 
and  the  motor  nuclei  of  the  cranial  nerves  orange.  The  corticopontine  portions  and 
the  cerebellar  paths  of  the  tegmentum  are  omitted. 

Abbreviations:  Aq,  aqueduct  of  Sylvius;  Cia,  anterior  segment;  Ci(g),  knee;  Cip, 
posterior  segment;  CirP,  retrolenticular  segment  of. the  internal  capsule;  CoM,  cortico- 
spinal si'stem  (pmk) ;  CON,  corticonuclear  system  (red)  of  the  pyramidal  tract,  VP; 
Ftp,  posterior  longitudinal  fasciculus;  FPba,  FPop,  anterior  and  posterior  pontine 
fibers;  FPyc,  crossed  pyramidal  tract;  FPyd,  direct  pyramidal  tract;  fPyh,  homolateral 
pyramidal  fibers;  fab,  medullary  aberrant  fibers;  fap,  pontine  aberrant  fibers;  fabp, 
medullary,  pontine  aberrant  fibers;  fasih,  subthalamic  or  superior  pontine  aberrant 
fibers;  fcnc,  fend,  crossed  and  direct  cervical  corticonuclear  fibers;  Ln,  locus  niger; 
NC,  caudate  nucleus;  NC,  tail  of  caudate  nucleus;  NCp,  nuclei  of  posterior  columns 
(Goll  and  Burdach);  NLi,  NLi,  NLz,  the  three  segments  of  the  lenticular  nucleus;  Np, 
pontine  nuclei;  PLp,  deep  pes  lemniscus;  PLs,  superficial  pes  lemniscus  or  aberrant 
pontine  fibers;  a,  b,  c,  the  three  modes  by  which  the  pontine  aberrant  fibers  enter  the 
third  nerve  nuclei;  Pul,  pulvinar;  Qa,  Qp,  anterior  and  posterior  corpora  quadrigemina; 
RgRm,  region  of  the  median  lemniscus;  Rm,  the  median  lemniscus;  Sgc,  subependymal 
gray  substance;  SR,  reticular  formation,  colored  yellow;  Th,  thalamus;  V^,  fourth 
ventricle;  VP,  pyramidal  tract;  ///,  IV,  nuclei  and  root  fibers  of  the  oculomotorius 
and  trochlearis  nerves;  Vm,  nucleus  and  root  fibers  of  the  trigeminus  (masticators); 
VII,  IX,  XI,  XII,  nuclei  and  root  fibers  of  the  facial  (VII),  glossopharyngeal  (IX), 
spinal  accessory  (XI),  and  hypoglossal  (XII),  nerves;  Xa,  anterior  root  of  the  spinal 
vagus;  X-XI,  root  fibers  of  the  spinal  vagus. 

The  encephalic  trunk  and  its  three  great  divisions,  cerebral  peduncles  (P),  the  pons 
(PO),  the  medulla  (B),  is  shown  in  sagittal  section  with  its  connections  with  the  internal 
capsule  (Cia.  Cig,  Cip,  Cirl)  and  the  thalamus  (Th)  in  part,  and  with  the  spinal  cord 
(M)  for  the  other  part.  The  anterior  segmerit  contains  the  pyramidal  tract  (V.P)  (and 
is  made  up  from  above  below)  of  the  foot  composed  of  the  fibers  passing  through  the 
knee  (Cig)  and  the  posterior  segment  (Cip)  ol  the  internal  capsule,  the  anterior  segment 
of  the  pons  with  the  pontine  nuclei  (Np)  and  the  anterior  (FPoa)  and  posterior  (FPop) 
pontine  fibers  and  the  anterior  pyramid  of  the  medulla  (Py) ;  and  the  posterior  segment 
or  tegmentum,  is  separated  above  by  the  locus  niger  (LN),  below  it  abuts  the  posterior 
pontine  fibers  (FPop.),  and  the  pyramid  of  the  medulla  (Py). 

The  tegmentum  is  made  up  of  (1)  a  gray  substance  the  reticular  formation  (S.R) — 
colored  yellow — which  extends  from  the  subthalamic  region  (RsTh)  to  the  lateral  columns 
of  the  cord  and  contains  the  motor  nuclei  of  the  cranial  nerves — colored  orange — disposed 
in  two  longitudinal  columns;  the  anterior  column  includes  the  motor  nuclei  of  the  trigem- 
inus (Vm),  the  facial  (VII),  the  nucleus  ambiguus  or  the  anterior  spinal  vagus  nucleus, 
(Xa);  the  posterior  column  includes  the  nuclei  of  the  (///)  and  (IV)  pair,  the  nuclei 
of  the  (VI),  the  long  nucleus  of  the  hypoglossal  (XII)  and  the  long  inferior  nuclei  or 
spinal  accessory  (XI) ;  (2)  of  the  longitudinal  fibers  of  which  a  part  group  themselves  in 
fascicles  more  or  less  compact  to  form  the  posterior  loiigitudinal  fasciculus  (Flp)  and  the 
median  lem7iiscus  (Rm).  The  median  lemniscus,  represents  an  important  sensory  path- 
way which  terminates  in  the  thalamus  (Th)  and  takes  part  of  its  origin  from  the  posterior 
column  nuclei  (Goll  and  Burdach).  The  po-'sterior  longitudinal  fasciculus  consists  of 
an  important  association  fiber  system  between  the  nuclei  of  the  oculomotorius,  the  spinal 
nuclei,  the  facial,  to  serve  the  important  functions  of  lateral  movements  of  the  head, 
eyes,  and  trunk  for  maintaining  the  static  equilibrium  of  the  body.     (See  Plate  VII.) 


PLATE    I 


Pyramidal 
tract 


III.  VI.  XI 
sal  aber 
ons  fibe 


Homolateral  pyramidal  fibers 


Pyrmnidal  Tract. — ^The  corticospinal  poi-tion  (CoM)  of  the  pyramidal  tract  (pink) 
takes  its  origin  in  the  ascending  frontal  convolutions  (Fa),  descends  in  the  posterior 
segment  of  the  internal  capsule  (Cip)  and  in  the  anterior  portion  of  the  cerebral  peduncle. 
The  corticonuclear  (COX)  poition  (red)  arises  from  the  Rolandic  operculum  (OpR) 
at  the  foot  of  the  ascending  frontal  convolution  and  the  second  frontal,  descends  through 
the  knee  (Cig)  of  the  internal  capsule  and  along  the  lenticular  border  of  the  posterior 
limb  of  the  internal  capsule  (Cip),  then  to  the  cerebral  peduncle,  where  it  divides.  One 
portion  of  the  corticonuclear  contingent  follows  the  course  of  the  corticospinal  fibers 
to  the  anterior  segments  of  the  pons  and  the  anterior  pyramidal  region  of  the  medulla. 
Another  portion,  the  system  of  the  aberrant  fibers  of  the  pyramidal  tract,  breaks  up  at 
different  levels,  penetrating  the  tegmentum,  descending  in  the  median  lemniscus  (Rm) 
and  the  interolivary  portion  of  the  medulla  to  make  up  the  fibers  entering  the  motor 
nuclei  of  the  cranial  nerves. 

In  the  medulla  portions  of  the  corticonuclear  fibers  fuse  more  or  less  ^vith  the  aberrant 
fibers,  pass  by  the  inferior  hypoglossal  and  spinal  accessory,  and  behave  like  the  cortico- 
spinal fibers.  One  part  descends  in  the  direct  pyramidal  (FPyd),  another  traverses 
the  middle  line  with  the  crossed  pyramidal  (FPyc) ,  a  third  descends  as  the  homolateral 
pjTamidal  (FPyh)  and  the  homolateral  cord.  All  of  these  fibers  end  finally  in  the 
motor  nuclei  of  the  anterior  horns  of  the  cord,  functioning  the  movements  of  rotation, 
flexion,  and  bending  of  the  head  on  the  neck  (Cir  to  Civ). 

The  aberrant  fibers  of  the  pyramidal  tract  which  detach  themselves  from  the  cortico- 
nuclear contingent  are:  (1)  Some  inconstant  fibers  in  the  subthalamic  region,  the  sub- 
l}iala7nic  aberrant  fibers  (fasth)  destined  for  the  oculomotor  nuclei  (Ilia)  and  to  the 
anterior  corpora  quadrigemina.  (2)  In  the  region  of  the  cerebral  peduncles  the  aberrant 
pyramidal  fibers  proper,  deep  pes  lemnisci  (PLp),  and  superficial  (PLs),  which  leave  the 
nuclei  of  the  oculomotor  nerves  (6  and  e),  then  descend  in  the  median  lemniscus  (Rm) 
and  contain  fiberg  for  the  (VI)  and  (XI)  nucleocortical  oculorotary  and  cortical  cephalo- 
rotary  fibers.  (3)  In  the  pons  regions  the  pontine  aberrant  fibers  (fap)  which  reinforce 
the  preceding  in  the  median  lemniscus  to  enter  the  motor  nuclei  of  the  trigeminus  (Vm), 
the  hypoglossal  (XII)  and  the  spinal  vagus  (Xa).  (4)  In  the  neighborhood  of  the 
pontomedullary  sulcus,  the  medullopontine  aberrant  fibers,  which  are  constant  and 
well  developed,  reinforcing  the  preceding  and  destined  to  the  nuclei  of  the  facial  ( VII) , 
the  mixed  nuclei  of  the  glossopharyngeal  (IX)  and  the  spinal  vagus  (Xa),  and  the 
hypoglossus  nucleus  (XII).  (5)  In  the  medulla,  medullary  aberrant  fibers,  homologues 
of  the  homolateral  pjTamidal  fibers,  inconstant  fibers  which  descend  the  length  of  the 
peripherj'  of  the  homolateral  anterolateral  cord  and  terminate  in  the  superior  cervical 
cord  nuclei  of  the  rotators  and  flexors  of  the  head  and  of  the  neck. 

These  aberrant  fibers  present  great  individual  variation  in  their  development — -as 
seen  in  the  different  degeneration  pictures  studied  by  the  Marchi  method.  Their  chief 
characteristic  is  their  descending  pathways  in  the  tegmentum,  particularly  in  the  median 
lemniscus  and  the  anterolivary  space  in  the  medulla.  This  disposition  explains  why  these 
fibers  can  be  affected  or  spared  by  peduncular  lesions,  according  to  the  site  above  or 
below,  or  whether  it  involves  the  anterior  or  posterior  (tegmental)  regions.  The  mode 
of  termination  of  these  corticonuclear  fibers,  possibly  direct,  possibly  by  intercalated 
neurones,  in  the  motor  nuclei  of  the  cranial  nerves  has  not  yet  been  elucidated  (Dejerinc) . 


50     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

fields  can  be  noted.  A  square  of  white  paper  (1  cm.)  on  a  gray  card 
may  be  used  instead  of  the  fingers.  For  a  general  test  of  the  color 
fields  squares  of  different  colors  may  be  used.  The  most  striking 
features  to  be  sought  for  are  hemianopsia,  temporal  or  nasal,  concen- 
tric limitations,  irregular  limitations,  quadrant  hemianopsias,  psychic 
blindness  and  scotomata.  Hemianopsia  should  always  be  searched 
for.  It  may  be  done  rapidly  by  use  of  the  usual  finger-sight  test  and  if 
there  are  any  anomalies  careful  chartings  by  a  perimeter  should  be 
made.  Scotomata  are  sought  for  in  the  same  manner.  Single  peri- 
metric studies  are  to  be  warned  against.  Frequent  examinations, 
especially  for  the  different  color  fields  are  necessary,  especially  to 
trace  advancing  changes.  For  careful  perimetric  work  the  methods 
of  Bjerrum  should  be  followed  out.^  A  useful  method  of  making 
perimetric  charts  is  by  means  of  a  modified  Bjerrum  screen.  A  large 
sheet  of  white  paper  12  to  15  inches  is  covered  with  a  thin  black 
cloth  or  black  paper  and  both  fastened  to  a  board.  A  small  thumb- 
tack in  the  center  can  serve  as  a  fixation-point  for  the  patient's  eye. 
The  test  object  is  carried  on  the  blunt  end  of  a  steel  pen,  0.5  cm.  in 
length  which  is  fixed  transversely  across  the  dark  end  of  a  metal  bar 
so  that  its  point  projects  about  1  mm.  on  the  opposite  side.  The  test 
object  thus  fixed  on  the  end  of  the  rod  is  moved  slowly  from  the  blind 
region  across  the  screen  until  the  patient  sees  it,  then  the  carrier  is 
simply  pressed  against  the  board  so  that  the  pin  penetrates  the  cloth 
and  marks  the  paper  beneath  it.  A  large  number  of  observations 
can  thus  be  quickly  made  and  they  can  be  easily  transferred  to  a 
perimeter  chart. 

In  all  cases  the  fundus  should  be  examined.  A  knowledge  of  the 
eye-ground  changes  is  essential  for  good  neurological  investigation. 
Works  on  ophthalmology  must  be  consulted  for  the  many  anomalies, 
but  the  most  important  to  be  observed  are  signs  of  pressure,  of  atrophy, 
of  retinal  hemorrhage  or  congestion,  and  irregular  pallors  (temporal 
pallor  in  multiple  sclerosis),  edema,  choroiditis,  retinitis,  venous 
congestion,  arterial  occlusions,  etc.  Optic  atrophy  may  exist  without 
bhndness.     (See  Fig.  2,  Plate  VI,  page  257.) 

Ocular  Malifigering.'^ — The  accurate  examination  of  the  eye  calls  for 
a  good  psychological  acumen  as  well  as  a  skilled  ophthalmological 
technic,  for  there  is  room  here  for  intentional  malingering  or  for 
unintentional  hysterical  falsification  on  the  part  of  the  subject.  The 
neurologist  must  have  a  ready  appreciation  of  what  an  eye  ought  to 
do  and  should  be  able  to  rapidly  vary  his  tests  so  that  the  subject  is 
taken  as  much  as  possible  oft'  his  guard.  Of  course  previous  history 
of  employment  or  other  use  of  the  eyes  is  important. 

Refractive  defects  because  of  their  universality  must  be  given  no 
more  than  a  limited  valuation.     There  is  a  reserve  of  accommodation 

1  See  Walker:  Arch,  of  Ophthalmology,  1915,  p.  369.  Cushing  and  Walker:  Brain, 
1915,  p.  341. 

^  Alger:  Ocular  Malingering,  New  York  State  Jour.  Med.,  December,  1918.  Jones 
and  Llewellyn:  Malingering,  Blakiston,  1917. 


SENSORIMOTOR  EXAMINATION  51 

which  may  not  be  apparent  at  once  if  the  subject  has  worn  correcting 
glasses,  but  which  may  be  tested  by  approximating  the  Hds,  the 
vision  being  thus  appreciably  sharpened.  There  should  normally  be 
a  reserve  of  accommodation  sufficient  to  make  a  hyperopia  of  four  or 
five  dioptres  compatible  with  20/100  vision.  A  myopia  in  which 
the  error  is  not  greatier  than  three  dioptres,  in  which  the  reading  of 
No.  1  txpe  proves  the  macula  sound,  has  room  for  considerable  improve- 
ment under  good  hygienic  conditions  even  though  distant  vision  is 
below  20/200. 

The  routine  examination  may  begin  with  the  momentary  use  of  a 
flashlight  which  will  reveal  important  external  disease,  opacities  of 
the  cornea  and  the  pupillary  reaction.  It  can  be  easily  and  inadver- 
tently determined  whether  the  patient  customarily  fixes  with  one  eye 
or  both.  A  preliminary  retinoscopy,  which  can  be  performed  merely 
by  having  the  patient  look  off  into  the  distance,  gives  an  idea  of  what 
the  patient's  unaided  eye  is  capable  of  and  the  kind  of  lens  that  may 
be  needed.  It  is  important  to  watch  the  patient  rather  than  the  test 
cards.  The  manner  of  conducting  the  examination  is  important  in 
order  to  provide  the  unexpected,  allow  the  test  to  proceed  rapidly 
and  to  keep  the  details  of  the  test  from  the  patient's  knowledge,  at 
the  same  time  that  one  maintains  an  attitude  of  friendliness  and 
apparent  carelessness.  Ignorance  of  the  visual  angle  on  the  part  of 
most  subjects  should  be  made  use  of.  A  mirror  may  be  used  to  reflect 
a  reversed  test-card  so  that  the  distance  is  increased  M-ithout  the 
subject's  knowledge.  Insistence  upon  some  one  point  may  throw^  the 
subject  off  the  track  and  lead  him  to  a  much  freer  and  more  correct 
report  upon  other  far  more  important  details. 

A  flashlight  presented  to  both  eyes  will  prove  whether  one  eye  is 
totally  blind  or  not  because  of  the  presence  or  absence  of  squint. 
Then  a  5-flegree  prism  interposed  before  the  supposedly  good  eye 
will  move  with  the  good  eye  if  the  other  is  blind  but  will  not  move  at 
all  if  binocular  fixation  is  usual.  Binocular  vision  may  also  be  proved 
by  the  Maddox  double  prism.  This  may  be  put  before  the  good  eye 
so  that  tAvo  lights  are  seen  one  above  the  other  and  if  the  other  eye  is 
good  three  lights  will  be  seen  when  this  is  uncovered.  Then  a  card 
may  be  passed  gradually  in  front  of  the  double  prism,  while  the  patient 
states  the  disappearance  of  either  flame.  The  seeing  of  two  flames 
when  the  prism  is  half  covered  proves  the  use  of  both  eyes.  An 
attempt  to  state  that  one  light  disappears  before  the  other  as  the 
card  is  carried  over  the  whole  prism  proves  the  subject's  statements 
incorrect.  The  overlapping  of  two  cards  caused  by  a  4  degree  prism 
will  be  confusing  if  the  eye  is  good  but  otherwise  cause  no  incon- 
venience. 

A  more  positive  form  of  testing  is  to  block  out  the  gotKl  eye  with  a 
l)lus  glass  before  the  i)atie]it  is  aware  of  it.  The  patient  may  read 
the  test  card  through  the  Stevens  phronometer  with  both  eyes  open 
when  he  will  read  one  card  up  and  one  down.  Then  his  attention  may 
be  distracte<l  and  tiie  prisms  reversed  when  he  will  try  to  read  as 


52     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

before  without  realizing  that  he  is  using  the  other  eye.  The  red  and 
green  glass  test  is  useful  only  if  conditions  are  just  right.  More  simply 
a  red  glass  may  be  put  before  the  good  eye  and  the  impression  given 
that  the  subject  should  see  red  with  this  eye  and  not  with  the  other. 
Then  his  ability  to  read  red  letters  on  a  white  card  may  be  tested, 
which  would,  however,  not  be  possible  tln-ough  the  red  glass  and  could 
be  done  only  with  the  other  eye.  The  ordinary  stereoscope  can  also 
be  used  over  the  patient's  distant  glasses,  care  being  taken  to  detect 
the  possible  closing  of  the  alleged  bad  eye. 

To  test  the  relative  quality  of  both  eyes  fine  type  bar  reading  is 
useful  with  a  pencil  held  vertically  four  or  five  inches  in  front  of  the 
patient.     Unless  both  eyes  are  reading  there  will  be  hesitation  and 


Fig.  8. 


-Disturbance  of  vision  from  bullet  wound  of  posterior  end  of  the  calcarine 
fissure.     (Lister  and  Holmes.) 


twisting  of  the  head.  This  test  can  also  be  applied  to  distant  vision 
by  means  of  an  ui^right  to  conceal  one  or  more  letters  in  each  line 
from  each  eye  while  all  are  visible  to  both.  The  diploscope  of  Remy 
permits  a  row  of  letters  to  be  seen  apparently  with  both  eyes  but  in 
fact  the  letters  of  either  side  are  seen  only  with  the  eye  of  the  opposite 
side.  The  unsuspecting  patient  will  insist  upon  the  contrary.  Several 
devices  have  been  used  to  make  this  test  applicable  to  distant  vision 
as  well.  The  author  uses  one  consisting  of  two  short  adjustable 
cylinders  which  the  patient  can  hold  to  his  eyes.  The  aperture  before 
the  good  eye  may  be  closed  while  the  patient  is  led  to  suspect  that  the 
poor  eye  has  been  blocked  and  the  sensation  will  be  that  he  is  using 
the  good  eye.  If  the  two  trial  cards  used  are  exactly  alike  they  will 
usually  be  fused  and  appear  as  only  one  card.     Then  extra  letters 


CRANIAL  NERVES 


53 


may  be  interpolated  at  different  places  on  each  card  and  these  will 
often  be  read  as  if  on  one  card  thereby  proving  not  only  binocular 
vision  but  its  degree. 

Third,  Fourth,  and  Sixth  Cranial  Nerves. — The  functions  of  the  third, 
fourth,  and  sixth  cranial  nerves  are  conveniently  tested,  first  together 
and  later  separately.  Ability  to  move  both  eyes  outwardly  (external 
rectus)  indicates  integrity  of  the  sixth  cranial  nerve.  If  the  eyes  can 
be  moved  freely,  amply  and  equally  upward,  downward,  and  inward, 
the  third  and  fourth  cranial  nerves  are  usually  intact. 

Having  seen  if  the  eyes  move  freely  in  all  directions,  nystagmus  is 
tested  for  under  the  same  conditions.  It  consists  of  a  slow  movement 
of  the  bulb  in  one  direction,  with  a  rapid  jerk  back  in  the  opposite 


Fig.  9. — Paralj.sis  of  the  fourth  nerve. 
Inability  to  look  down.  It  will  be  noted 
that  as  the  eyeball  does  not  turn  down- 
ward the  eyelid  does  not  descend,  at  the 
same  time  the  patient  can  close  his  eyes 
when  told  to  do  so.     (Russel.) 


Fig.    10. — Cerebral    sypliilis.       External 
rectus  palsj',  right  eye. 


direction.  Notes  on  the  direction  of  the  slow  and  rapid  movements 
should  be  mafle.  They  are  of  great  value  in  determining  labyrinthine 
and  cerebellar  nystagmus.  Nystagmus  may  be  present  on  central 
fixation,  or  only  become  apparent  as  the  eyes  are  directed  to  one  side. 
Slight  tremors  of  the  globes  on  extreme  lateral  position  may  be  of  small 
diagnostic  importance.  Nystagmus  should  be  tested  for  in  the  vertical 
and  horizontal  and  also  in  oblique  axes.  Rotatory  nystagmus  may 
be  looked  for. 

In  the  presence  of  a  nystagmus,  certain  supplementary  tests  are 
advisable.  The  most  important  are  the  turning  stool,  and  hot-  and 
cold-water  tests.  In  the  former  the  patient  is  seated  on  a  revolving 
office  chair,  and  turned  to  the  right  or  left  ten  re\olutions,  with  moder- 
ate rapidit}'.     On  arresting  the  ^e^■olutions  careful   note   should   be 


54     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

made  of  the  direction  of  the  nystagmus  in  each  eye,  and  also  the  length 
of  time  it  persists.  The  head  may  be  held  erect,  and  bent  forward  or 
backward  to  test  each  semicircular  canal.  Specially  constructed  chairs 
are  needed  for  careful  work.    (See  Testing  of  Labyrinth,  p.  (39.) 

Hot  or  cold  water  is  syringed  gently  into  the  external  auditory 
meatus  of  each  ear.  A  large  bulb  syringe  is  used.  Cold  causes  a 
definite  rotatory  nystagmus  toward  the  ear  not  irrigated.  The  patient 
also  has  vertigo  and  marked  disturbance  of  equilibrium.  If  hot  water 
be  used  the  quick  nystagmic  movement  will  be  toward  the  ear  irrigated, 
and  the  ataxia  is  different.  The  presence  of  labyrinthine  trouble  or 
cerebellar  disorder  involving  the  vestibular  apparatus  cause  modi- 
fications in  the  character  of  this  nystagmus.     (See  Vestibular  Tests.) 


Left  supr. 
Bectus 


Right  supr. 
Rectus 


Left  infr. 
Rectus 


Right  infr. 
Rectus 


Left  infr. 
oblique 


Right  infr. 
oblique 


Left  supr. 
oblique 


Right  supr. 
oblique 


A  B 

Fig.  11. — Werner's  "artificial  memory"  for  the  double  images  in  ocular  paralyses 
(Ophthalmic  Review,  1886).  A  shows  the  position  of  the  images  in  paralysis  of  the 
recti  muscles;  B,  in  paralysis  of  the  oblique  muscles.  The  clotted  lines  indicate  "false" 
images,  the  thick  black  lines  "true"  images.     (Stewart.) 

Diplopia  is  next  tested  for.  The  patient  should  be  asked  if  he  has 
ever  seen  double  and  a  single  light,  or  one  finger  held  to  the  right  or 
left,  up  or  down,  used  to  test  if  double  vision  exists.  Should  it  be 
present  the  position  of  the  images  in  relation  to  one  another  should  be 
noted;  whether  they  separate  or  approach  as  the  candle  is  farther  or 
nearer,  and  a  red  glass  should  be  placed  before  one  or  the  other  eye  to 
determine  the  location  of  the  images,  and  their  relative  position. 

The  accompanying  schemes  are  of  value  in  memorizing  the  muscles 
involved  (Fig.  11): 

Monocular  diplopia,  seeing  double  with  one  eye,  is  occasionally  met 
with.  It  is  due  to  gross  corneal  or  eye  defects,  occasionally  in  central 
scotomata,  but  usually  it  is  a  product  of  projection  in  hysteria. 

Pupils. — The  size  should  be  compared  and  noted.  Right  equals  left, 
right  larger  than  left  or  vice  versa  and  recorded  thus :  (r=l:r>l,l>r), 
and  a  rough  measure  given,  2,  3,  4  mm.,  as  the  case  may  be;  mydriasis, 
myosis.  The  form  and  the  presence  of  irregularities,  changes  in  out- 
line, oval,  polygonal,  ragged,  and  the  implantation  (ectopia)  should  be 


CRANIAL  NERVES 


55 


Fig.  12. — Pontine  syndrome,  with  eye  palsies  of  central  origin  and  syringomyelic  dis- 
sociation. There  is  here  a  crossed  hemianesthesia  with  alternating  paralysis  of  the 
VI  and  VII  cranial  nerves,  anesthesia  of  the  V  nerve  due  to  hemorrhage  in  the  lateral 
and  lower  portion  of  the  pontine  tegmentum  of  the  left  side.  The  right-hand  figure 
shows  the  hemianesthesia,  dissociated  as  in  syringomyelia  (hemianalgesia  and  hemi- 
thermanesthesia  due  to  lesion  of  the  crossed  sensory  pathways  of  the  lateral  portion  of 
the  reticular  formation).  There  is  preservation  of  the  tactile  and  postural  sensibilities 
and  of  the  stereognostic  sense,  because  of  the  incomplete  extension  of  the  lesion  to  the 
median  lemniscus  (Rm).  The  left-hand  figure  shows  (1)  atrophic  paralysis  of  the  VII 
nerve  with  reaction  of  degeneration,  lagophthalmia,  drooping  of  the  lips,  loss  of  facial 
mimicry,  paralysis  of  the  entire  left  facial  (VII)  indicated  (Fig.  «) ;  (2)  anesthesia  of 
the  face,  following  involvement  of  the  descending  root  of  the  trigeminus  (see  V  on  a') ; 
(3)  paralysis  of  the  external  rectus  with  convergent  straV)ismus  by  reason  of  the  over- 
action  of  the  antagonists.  Furthermore,  there  is  a  paralysis  of  the  lateral  movements 
of  the  eyeballs  toward  the  left  notwithstanding  the  integrity  of  the  posterior  longitu- 
dinal fasciculus  (Flp.),  of  the  nucleus  of  the  VI  and  of  the  adjacent  reticular  formation. 
The  lesion  of  Deiters's  nucleus,  and  of  the  labyrinthine  oculorotary  fibers  which  unite 
Deiters's  nucleus  (ND)  to  the  nuclei  of  the  III  and  VI  causes  this.  By  rea.son  of  the 
overaction  of  the  antagonists  the  patient  looks  to  the  right.  (After  Dejerine.)  For 
abbreviations  on  the  anatomical  sketch  see  section  on  Midbrain  and  Plate  VIII. 


56     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 


carefully  noted.  Particular  attention  should  be  directed  to  the  esti- 
mation of  changes  due  to  drugs,  to  accidents  or  injuries,  violent 
emotions,  to  inflammatory  products,  and  to  changes  due  to  chest  dis- 
orders or  neck  disorders  or  to  other  involvements  of  the  cervical 
sympathetics. 

The  reactions  to  light  should  then  be  tested,  at  first  with  clear 
daylight,  the  patient  looking  at  some  distant  object.  In  bed  cases, 
electric  hand  lamps  are  very  serviceable;  with  these  the  light  is  best 
directed  somewhat  obliquely.  The  reaction  may  be  prompt  and  wide, 
prompt  and  restricted,  slow%  sluggish,  diminished  or  absent.  Each 
eye  is  to  be  tested  separately,  and  then  the  consensual  test  applied 
by  illuminating  one  eye  only  and  noting  the  reaction  in  the  other. 

Ready  fatigability  of  the  pupillary  re- 
actions to  light  should  be  tested  and 
the  results  noted. 

Wernicke's  hemiopic  phenomenon 
should  be  sought  for  in  hemianopsias. 
In  this  the  pupil  does  not  react  if  the 
light  falls  upon  the  blind  segment  of 
the  retina.  It  is  indicative  of  a  lesion 
in  the  optic  neurones  between  the 
chiasm  and  the  corpora  quadrigemina. 
Tests  for  the  accommodation  reflexes 
are  then  made.  The  patient  looks  at 
the  finger  as  it  is  moved  near  to  or 
away  from  the  eye,  and  there  is  corre- 
sponding contraction  or  dilatation  of 
the  pupils.  Here  the  reaction  may  be 
prompt  or  slow,  with  slight  or  marked 
amplitude.  In  blind  people  the  request 
to  look  at  their  own  nose  and  then 
across  the  room  may  bring  out  the 
reaction.  A  lost  or  partially  lost  light 
reflex,  with  unimpaired  accommodation 
reflex,  is  known  as  the  Argyll-Eohertson  imijU.  It  may  be  present  in 
one  or  both  eyes,  and  may  be  partial  or  complete.  It  is  due  to  a 
dissociation  of  the  sensorimotor  reactions,  and  may  be  present  in  a 
variety  of  diseases,  although  it  is  most  frequently  found  in  syphilitic 
disorders  (Fig.  14). 

The  mechanism  is  variously  although  not  entirely  satisfactorily 
explained  because  of  the  complexity  of  the  afferent  and  efferent 
fiber  tracts  and  their  connections  (Lewandowsky  scheme,  see  Fig.  14). 
Marina's  hypothesis  of  its  peripheral  origin  (disease  of  ciliary  ganglion) 
explains  many  of  the  tabetic  and  paretic  cases,  but  does  not  explain 
some  of  the  traumatic  or  mesencephalic  cases.  Cajal's  scheme  is  as 
follows : 

1.  Retinal  neuron  with  its  optic  fibers  ramifying  in  the  anterior 
corpora  quadrigemina. 


Fig.  13. — Inequality  of 
Left  pupil  larger  than  right . 
bile.     Cerebral  tumor. 


pupils. 
Immo- 


CRANIAL  NERVES 


57 


2.  The  mesencephalic  neuron  with  its  axis-cylinder  formation  of  the 
posterior  commissure. 

3.  The  neuron  of  the  interstitial  nucleus  of  the  tegmentum  (calotte) 
with  its  collaterals  destined  to  the  motor  nuclei. 

4.  The  neuron  of  the  bulbar  nuclei  of  the  oculomotor  communis  et 
externus  and  of  the  patheticus  with  axis-cylinders  going  to  the  muscles 
of  the  eve. 


Pyramidal 
Tracts 


Ganglion 
Siipremum 

Si/mpathetic 


Fig.  14. — The  paths  of  the  chief  eye  reflexes:  (1)  Pupillary  reflex:  Retina;  optic;  X; 
corpora  quadrigemina ;  X;  oculomotor  nucleus,  oculomotor  nerve;  ciliary  ganglion; 
sphincter  iridis.  (2)  Closing  eyes  to  light:  Retina;  optic;  X;  corpora  quadrigemina, 
Meynert's  fillers;  X;  facial  nucleus;  facial  nerve;  lid  muscles.  (3)  Winking  on  approach 
of  object:  Retina;  optic;  X;  corpora  quadrigemina  or  pulvinar,  external  geniculate; 
cortex;  pyramidal  tract;  X;  facial  nucleus;  facial  nerve;  lid.  (Same  paths  also  in  2.) 
(4)  Corneal  reflex:  Cornea;  trigeminus;  trigem.  nucleus;  facial  nucleus;  facial  nerve; 
lid.     (Lewandowsky :  Funktionen  d.  Zentralnervensystem,  p.  122,  Fig.  29.) 


The  sympathetic  reflex  is  tested  by  pinching  the  skin  of  the  cheek  or 
some  other  part  of  the  body.  The  pupils  dilate  under  the  influence  of 
painful  stimuli. 

Ophthalmoplegia  externa  is  the  name  given  to  a  paralysis  of  the 
external  eye  muscles;  ophthalmoplegia  interna  to  those  of  the  pupil, 
which  is  widely  dilated  and  immobile  to  light  and  convergence.  Com- 
plete ophthalmoplegia  is  found  when  all  of  the  pupillary  phenomena 
are  absent  and  there  is  loss  of  all  eye  movements  with  y)tosis. 

Ptosis  consists  in  a  drooping  of  the  upper  lid,  paralysis  of  levator 
palpebrse,  which  is  supplied  by  filaments  from  the  third   nerve. 

Henneberg's  reflex,  consisting  in  a  slow  spastic  action  contraction  of 


58     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 


the  orbicularis  oris  when  the  hard  palate  is  stroked,  may  be  mentioned 
with  the  reflexes  of  the  cranial  nerves. 

The  following  conditions  of  pupillary  activity  are  of  great  clinical 
importance  and  will  be  discussed  seriatim:  (1)  Complete  immobility. 
(2)  Keflex  immobility.  (3)  Dilator  or  sympathetic  paralysis.  (4) 
Inequality  and  deformity  of  the  pupil. ^ 

1.  Absolute  or  complete  pupillary  immobility.  All  stimuli  via  the 
vegetative  nerves  to  the  muscles  of  the  iris  are  absent  except  a  minimal 
effect  of  the  s^Tnpathetic.  When  the  sphincter  is  paralyzed  this  in- 
fluence upon  iris  activity  is  next  to  nothing.  The  pupils  are  dilated 
more  than  normally  and  are  somewhat  distorted.  Since  the  ciliary 
ganglion  automatically  maintains  a  certain  amount  of  tone  in  the 
sphincter  muscle,  destruction  of  the  ciliary  ganglion  or  nerves  causes 

an  almost  maximal  dilatation  greater 
than  is  observed  in  disease  of  the  nu- 
cleus or  root  of  the  oculomotor.  Ab- 
solute immobility  is  observed  in 
syncope,  central  lues,  epileptic  at- 
tacks, occasionally  in  hysterical  dis- 
sociation, in  war  shock  and  in  very 
marked  fear  from  other  causes.^ 
Immobility  in  the  mydriatic  state  is 
due  in  these  cases  to  inhibition  of 
cortical  origin,  immobility  in  the 
myotic  state  to  an  increase  of  the 
sphincter  tone  (Bumke). 

2.  Reflex  or  isolated  immobility 
is  more  difficult  to  explain.  This 
is  the  Argyll-Robertson  phenome- 
non. Its  characteristics  are  one  or 
both-sided  failure  to  react  to  light 
thrown  either  on  the  same  or  other 
side,  intact  vision,  narrowness,  in- 
equality and  irregularity  of  the  pupil- 
lary outline,  retained  reaction  to  accommodation  and  to  convergence. 
The  question  as  to  the  genesis  of  this  condition  and  particularly  the 
myosis  which  so  often  accompanied  it  is  of  great  theoretical  as  well 
as  of  practical  importance.  It  has  been  one  of  the  most  frequently 
discussed  questions  in  neurology  for  several  decades.^  It  is  discussed 
more  fully  in  the  chapter  on  Syphilis  of  the  Nervous  System,  q.  v., 
Chapter  V. 

3.  The  picture  of  sympathetic  or  dilator  paralysis  is  known  in 
physiology  as  Budge's  symptom  complex,  in  the  clinic  as  Horner's 

>Higier:  Vegetative  Neurology,  Nervous  and  Mental  Disease  Monograph  Series, 
No.  27,  1919. 

s  Guillain  and  Barre:  Pupils  and  War  Shock,  Annals  de  Med.,  1917,  No.  5,  vol.  v. 

3  Compare  Lutz:  The  Light  Pupillarj-  Reflex,  Arch,  of  Ophthalmology,  1918,  vol. 
xl\-ii,  for  a  very  complete  discussion  of  this  problem  which  cannot  be  entered  into  here 
more  fully. 


Fig.   15. — Tabes  with  bilateral   ptosis. 
Operated  upon  to  holdeyelid  open. 


CRANIAL  NERVES  59 

symptom  complex.  In  addition  to  vasomotor  and  sweat  anomalies, 
it  is  characterized  by  sinking  in  of  the  eyeball,  narrowing  of  the  lid 
slit  (m.  or})italis),  lowering  of  the  npper  lid  and  raising  of  the  lower 
lid  (m.  tarsi),  narrowing  of  the  pupil  (m.  dilator  pupillse)  and  reten- 
tion of  the  psychic  and  optic  nerve  reflexes  of  the  pupil. 

In  experimental  section  of  the  sympathetic  tlie  paralysis  lasts  only 
until  the  preganglionic  part  of  the  sympathetic  chain  again  joins  the 
preganglionic  and  the  postganglionic  the  postganglionic. 

The  contrast  between  the  very  active  sphincter  contraction  and  the 
equally  sluggish  relaxation  is  typical  of  sympathetic  paralysis  (Bumke). 

The  localizing  value  for  a  diagnosis  of  sympathetic  paralysis  or 
irritation  is  self-evident.  Both  conditions  indicate  pressure  upon  the 
sympathetic  chain  due  to  a  timior,  a  traumatic  lesion  of  the  spinal  cord 
(hematomyelia),  or  a  tumor,  degenerative  process  (gliosis)  or  an 
infective  process  in  the  upper  dorsal  segments  of  the  spinal  cord 
involving  Budge's  center.  IMore  rarely  a  lesion  of  the  oblongata 
(thrombosis  of  the  posterior  inferior  cerebellar  artery,  syndrome  of 
Babinski-Xageotte)  or  a  lesion  of  the  spinal  roots  in  the  cervicodorsal 
region  (neuritis  syndrome  of  Dejerine-Klumpke).  The  sjTnpathetic 
lesion  may  be  localized  in  its  cervicodorsal,  bulbar  or  basal  parts 
according  to  the  accompanying  symptoms  (disturbance  of  the  hand 
muscles,  tongue,  deglutition  apparatus,  trigeminus). 

Pharmacological  experiments  with  sympathicotropic  substances 
during  the  last  few  years  have  added  something  to  the  differential 
diagnosis  of  sympathetic  disease.  For  this  cocaine  and  adrenalin 
mydriasis  have  been  used.  These  tests  can  be  easily  performed 
provided  there  is  a  healthy  uninjured,  not  inflamed  conjunctival  sac 
which  is  absorbing  normally. 

Cocaine  in  a  moderately  strong  solution  (under  3  per  cent.)  stimu- 
lates the  dilator.  Absence  of  cocaine  mydriasis  indicates  weakness 
of  the  s\Tnpathetic.  If  a  paralysis  has  been  shown  by  this  method 
in  the  absence  of  other  signs,  it  becomes  necessary  to  discover  the 
location  of  the  lesion  for  prognostic  and  therapeutic  reasons.  Whether 
it  be  preganglionic  or  postganglionic,  above  or  below  the  superior 
cervical  ganglion  and  whether  it  be  in  a  place  accessible  to  the  surgeon. 
The  very  active  endogenous  hormone,  adrenalin,  will  settle  this 
question.  A  1  per  cent,  solution  dropped  into  the  conjunctival  sac 
normally  produces  no  reaction.  (2  drops  are  dropped  in  every  five 
minutes  for  three  times  [Cords].)  If  the  irritability  of  the  dilator  be 
increased  as  is  the  case  in  postganglionic  disease  dilatation  of  the  pupil 
will  result  after  fifteen  minutes. 

As  Iligier  has  shown,  it  is  probable  that  after  the  nerve  to  the  dilator 
is  gone  the  contractile  muscle  tissue  becomes  more  irritable.  (IMunk's 
isolation  phenomenon,  Langendorf's  paradoxical  mydriasis.)  This 
adrenalin  mydriasis  is  analogous  to  the  rapid  and  maximal  dilatation  of 
the  pupil  after  electrical,  sensory  or  pyschic  stimuli  in  animals  in  whom 
postganglionic  section  of  the  sympathetic  has  been  experimentally 
secured. 


60    METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

In  many  instances  of  disease  involving  in  some  way  the  region  of 
the  anterior  or  middle  cranial  fossae  (orbital  disease,  fracture  of  the 
base)  adrenalin  mydriasis  has  been  observed.  The  explanation  of  this 
is  that  the  sympathetic  fibers  going  to  the  eye  come  from  the  carotid 
plexus  and  join  the  first  branch  of  the  trigeminal  nerve  distal  to  the 
Gasserian  ganglion.  A  combination  of  disease  of  the  first  branch  of 
the  trigeminal  with  the  adrenalin  mydriasis  of  postganglionic  sym- 
pathetic paralysis  can  therefore  be  of  great  localizing  value  and  make 
an  exact  localization  of  the  fracture,  fissure  or  tumor  of  the  base  quite 
possible. 

Bilateral  mydriasis  occurs  as  well  as  unilateral  in  diseases  of  internal 
secretion  (pancreas  diabetes,  hyperthyroidism).  This  indicates  an 
increase  in  the  irritability  of  the  entire  sympathetic  system. 

Deformity  of  the  pupils  (asymmetry,  irregularity)  and  inequality 
(anisocoria)  are  sometimes  congenital,  usually,  however,  acquired  and 
of  organic  origin.  The  cause  is  most  frequently  syphilis  in  some  form. 
Sometimes  local  disease  as  pressure  upon  the  cervical  sympathetic 
will  cause  the  deformity.  Transient  inequality  of  the  pupils  is  often 
found  in  severe  unilateral  migraine  or  in  myalgias  of  the  head  and  neck 
muscles  with  painful  points  in  the  neck  (inequality  due  to  pressure  of 
the  contracted  muscle  upon  the  cervical  ganglion). 

Hippus  of  the  pupil  and  rapid  mydriasis  are  of  very  slight  clinical 
value. 

From  the  point  of  view  of  vagotonia,  the  eye  shows  many  signs  worth 
clinical  investigation.  The  vagotonic  shows  lacrimation  and  accom- 
modation spasm  which  is  relieved  by  atropine.  The  reaction  of  I>6wi 
(positive  adrenalin  mydriasis)  in  diabetes  and  Graves's  disease  shows  a 
lowered  sympathetic  tone.  If  there  be  spastic  conditions  in  other 
vegetative  regions  as  gastric  crises,  asthma  nervosum,  acute  myosis  will 
be  found  as  an  accompanying  evidence  of  spasm.  The  opposite  to 
vagotonia,  i.  e.,  stimulating  the  sympathetic  is  shown  in  some  cases 
of  Graves's  disease  by  paralysis  of  convergence  (Moebius's  sign)  and 
exophthalmos  (spasm  of  Miiller's  muscle). 

Atropine  acts  slowly  but  persistently  upon  the  pupil  of  elderly  people, 
on  the  other  hand  rapidly  but  briefly  in  young  people  and  vagotonics. 
V.  Graefe's  sign  shows  an  increased  tone  in  the  autonomic  levator 
palpebrse.  It  usually  follows  pilocarpine  instillation  in  young  vago- 
tonics. 

The  Fifth  ( Trigeminus)  Nerve. — The  motor  functions  of  the  fifth 
nerve  are  most  readily  tested  by  having  the  patient  move  his  jaw  to 
the  right  and  left.  The  examiner's  hand,  exerting  contrary  pressure, 
can  determine  dilTerences  in  innervation  (external  pterygoid,  temporals). 
The  finger  wrapped  in  a  towel  can  be  used  to  bite  upon  to  determine 
variations  in  bite  (masseter,  temporal).  The  state  of  hardness  of  the 
temporals  and  masseter  muscles  can  be  directly  palpated.  The  jaw 
should  be  protruded  (internal  pterygoid). 

The  use  of  a  tuning-fork  of  low  pitch  is  useful  in  determing  loss 
of  function  of  the  tensor  tympani  muscle. 


CRANIAL  NERVES  61 

In  one-sided  paralysis  of  the  motor  fibers  of  the  fifth  nerve,  the 
opened  jaw  deviates  to  the  paralyzed  side  by  the  action  of  the  sound 
external  pterygoid.  The  patient  chews  on  the  sound  side.  It  may 
or  may  not  be  accompanied  by  sensory  changes.  Elevation  of  the 
eyelid  on  strong  biting  is  a  frequent  associated  movement. 

The  jaw  reflex  may  be  tested  at  the  same  time.  With  the  mouth 
partly  open  a  pencil  or  flat  object  is  placed  upon  the  teeth  and  lightly 
tapped  with  the  hammer.  There  is  a  quick  contraction  of  the  mas- 
seters  and  temporals,  and  usually  an  associated  movement  of  closing 
the  eyelids. 

The  sensory  functions  of  the  fifth  nerve  demand  very  careful  testing 
by  reason  of  its  wide  distribution. 

The  supra-orbital,  infra-orbital  and  mental  points  should  first  be 
pressed  upon  to  determine  the  degree  of  sensitiveness.  Then  the 
palpebral,  conjunctival,  and  corneal  reflexes  should  be  tested.  This 
is  best  done  with  a  long  pin  with  a  globular  glass  head.  With  the 
patient  looking  away  from  the  examiner,  the  palpebral  margin  is 
touched  with  the  head  of  the  pin,  then  the  conjunctiva,  and  finally 
the  choroid,  and  running  along  the  globe  over  the  cornea  the  effect 
is  noted.  Both  eyes  should  be  compared  and  the  tear  secretion  noted. 
The  amount  of  tear  secretion  may  be  measured  by  hanging  two 
small  strips  of  litmus  paper  on  each  lower  lid,  by  bending  in  the  paper 
at  the  top  so  as  to  make  a  small  ledge  to  hang.  The  rate  of  moistening  of 
the  two  sides  will  show  quantitative  variations  in  the  amounts  secreted. 
The  ordinary  sensibility  of  the  face  should  be  tested  first  with  a 
camel-hair  brush — the  two  sides  compared.  Then  with  the  point  and 
head  of  a  very  sharp  pin;  then  the  skin  should  be  pinched  on  each  side 
and  differences  noted.  Deep  pressure  over  the  malar,  frontal,  and 
jaw  bones  made  to  determine  deep  pressure  sense,  and  the  use  of  hot  and 
cold  test-tubes  to  learn  if  variations  in  thermal  sense  exist.  Finally 
a  slowly  vibrating  tuning-fork  should  be  applied  to  the  bones  of  the 
head  to  determine  their  bony  sensibility. 

The  interior  of  the  mouth  and  the  surface  of  the  tongue  should  not 
be  neglected  in  these  tests,  and  special  care  should  be  taken  in  outlining 
changes  in  the  ear  areas,  and  within  the  auditory  canal. 

Careful  inspection  of  the  teeth  should  not  be  omitted,  and  anomalies 
of  dentition  carefully  noted.  The  two  sides  of  the  bony  structures 
of  the  face  should  be  compared  for  bon\-  atrophies  (hemiatrophy) 
or  hypertrophies  (acromegaly). 

The  Seventh  (Facial)  Nerre. — The  seventh  (facial)  nerve  is  a  motor 
nerve.  The  state  of  the  musculature  at  rest  should  first  be  recorded. 
The  folds  and  the  angles  of  the  mouth  and  muscle-twitching  carefully 
noted.  The  patient  is  then  asked  to  wrinkle  his  forehead  (frown), 
raise  the  eyebrows,  close  his  eyes  tight,  show  the  teeth,  whistle,  and 
puff  out  the  cheeks.  In  this  way  the  musculature  of  the  two  sides  can 
be  compared.  A  mild  joke  will  bring  out  the  emotional  contractions 
which  are  equally  important  to  note.  Some  individuals  can  move 
their  ears. 


62     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

Not  only  should  the  presence  of  paralysis  or  paresis  be  carefully 
noted,  but  spasms  observed  under  voluntary  and  emotional  reaction. 
Their  degree,  character,  and  intensity  should  be  carefully  recorded. 


Fig.   16. — Facial  palsy.     Smiling.  Fig.   17. — Facial  palsy.     Whistling. 

Sensory,  secretory,  and  motor  changes  are  to  be  tested,  and  many 
differences  are  to  be  noted  in  the  distribution  of  the  palsies  according 
to  the  location  of  the  lesion  of  this  nerve:  (1)  after  its  exit  from  the 


^^  -">.   M 

^^L      <i#y^is9L^^I 

H  "v^jH 

Ik  ^^^1 

Fig.   18. — Facial  palsy.     Closing  the  eyes.       Fig.  19. — Facial  palsy.  Showing  the  teeth. 

stylomastoid  foramen,  (2)  within  the  Fallopian  aqueduct,  (3)  between 
its  emergence  from  the  pons  and  the  geniculate  ganglion,  or  (4)  within 
the  pons.     (See  Fig.  20.) 


CRANIAL  NERVES 


63 


The  Eighth  {Cochlear  and  Vestibular)  Nerves. — Here  two  entirely 
different  nerves  with  absolutely  separate  functions  need  to  be  tested. 
They  are  the  cochlear  nerves  (hearing)  and  the  vestibular  nerves 
(equilibrium). 

Hearing  is  roughly  tested  most  satisfactorily  by  speech.  Having 
first  ascertained  that  the  auditory  canals  are  free  from  wax,  the  exam- 


rears 

Taste  <&  Sativa 


-Sweat 
-  Tears 
-Taste  &  Saliva 


Fig.  20. — Diagram  of  facial  nerve,  showing  course  of  secretory  and  of  taste  fibers  of  the 
accompanying  gicssopalatine  and  vegetative  fibers.     (Stewart.) 


iner,  some  six  to  ten  feet  away,  pronounces  six  numbers,  asking  the 
patient,  who  has  one  ear  closed,  to  repeat  them  after  him.  The  voice 
is  then  raised  or  lowered,  and  the  distance  \aried  to  determine  the 
hearing  caj)acity.  The  opposite  ear  is  tested  in  the  same  manner  and 
comparisons  made.  A  watch-tick  or  tuning-fork  may  also  be  used. 
Careful  tests  with  tuning-forks  and  whistles  are  needed  in  complicated 
cases. 


64     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

Bony  conduction  should  also  be  tested.  This  is  done  by  putting  the 
vibrating  tuning-fork  over  the  mastoid,  and  the  patient  indicates 
when  he  no  longer  hears  it,  the  fork  is  then  brought  to  the  auditory 
meatus  to  test  the  air  conduction.  Rinne's  test  is  positive,  i.  e.,  air 
conduction  present  after  loss  of  bone  conduction  is  the  normal  formula : 
the  negative  Rinne  indicates  middle-ear  disorder.  The  tuning-fork 
on  the  center  of  the  forehead  is  heard  in  both  ears  equally  under 
normal  conditions  (Weber).  In  middle-ear  affections  it  may  be  heard 
unequally  on  the  two  sides.  Positive  Weber  (i.  e.,  louder  on  affected 
side)  with  negative  Rinne  is  largely  indicative  of  middle-ear  disorder. 
Deafness  due  to  central  disorder  is  usually  associated  with  other  local- 
izing signs,  yet  it  may  be  an  isolated  phenomenon  of  beginning  tumor, 
encephalitic  process,  tabes,  multiple  sclerosis,  etc. 

Bony  conduction  tests,  Rinne,  Weber,  Schwabach  and  Gelle  are 
also  of  service  in  differentiating  labyrinthine  deafness,^  and  they  are  of 
value  in  testing  the  general  bony  conductivity  of  the  cranial  walls 
as  well,  they  thus  afford  useful  information  concerning  functional 
states  of  the  trigeminus,  which  might  falsely  be  referred  to  acoustic 
anomalies.  The  two  possibilities,  acoustic  and  deep  bony  sensibility 
should  be  differentiated  in  the  tests.  In  this  differentiation  the  usual 
mastoid  site  is  inadvisable  as  it  registers  the  acoustic  reaction  too 
massively.  Other  parts  of  the  cranial  walls  should  be  chosen.  A  watch 
can  be  utilized  for  these  tests  as  well. 

Forks  of  very  slow  vibration  are  of  value  in  determining  the  func- 
tional capacity  of  the  stapedius  muscles.  Tests  with  continuous 
tone  series  following  Bezold's  methods  are  indicated  in  all  complicated 
cases,  since  defects  in  lower  or  in  higher  tone  perception  usually 
indicate  a  difference  in  the  site  of  the  lesion. 

Certain  patients  show  hyperacusis,  tinnitus.  Ringing  in  the  ears 
is  an  extremely  elusi^'e  sign.  It  is  an  evidence  usually  of  middle-ear 
or  of  cochlear  irritation.  The  sounds  vary  greatly.  They  may  be 
beating,  buzzing,  or  whistling,  and  may  at  times  be  the  point  of 
departure  of  illusions,  or  hallucinations.  The  pulsating  types  of 
tinnitus  are  usually  associated  with  the  heart  beat.  They  are  found 
in  certain  tumors,  in  aneurisms,  or  in  anxiety  states  with  cardiac 
irregularities.  Continuous  tinnitus,  low-pitched  or  high-pitched,  is 
the  more  common.  The  effects  upon  the  tinnitus  by  lying  down  and 
also  the  effects  of  certain  drugs,  amyl  nitrite,  etc.,  are  of  service  in 
differentiating  the  causes  and  probable  site  of  a  tinnitus. 

Malingering  for  deafness  has  become  frequent  since  the  great  war 
and  numerous  communications  have  been  made  upon  the  subject.  The 
following  notes  may  be  of  service  in  testing  malingering.^ 

]\Iost  malingerers  of  deafness  who  are  subject  to  the  military  draft 
know  that  deafness  of  a  certain  grade  in  either  ear  will  exempt  them. 
Complete  bilateral  deafness  is  therefore  for  various  and  obvious  reasons 

'  Bard:    Du  role  de  la  sensibilite  osseuse,  Ann.  de  Med.,  January  and  February,  1918. 
"-  Kerrison:  Transactions  of  Am.  Otol.  Soc,   1918.     Jones:  Value  of  Ear  Examina- 
tions to  Neurologists,  Ibid. 


CRANIAL  NERVES  65 

rarely,  if  ever,  claimed.  Two  types  of  unilateral  deafness  are  therefore 
assumed,  i.  e.,  (1)  deafness  advanced,  but  not  complete;  and  (2) 
absolute  deafness. 

The  tests  upon  the  combined  results  of  which  one  may  be  usually 
able  to  base  a  definite  conclusion  are:  (1)  Weber's  test;  (2)  the  loud 
conversational  voice  test;  (3)  the  binaural  stethoscope  test;  (4)  tests 
eliciting  incongruous  variations  in  response;  (5)  the  noise  apparatus, 
reading  test  (Lombard's  test). 

Kerrison's  method  of  examination  is  about  as  follows:  Only  one 
registrant  at  a  time  is  admitted  to  the  room.  Learn  from  him  which  ear 
is  the  deaf  or  deafer  one,  and  whether  he  regards  this  deafness  as  partial 
or  complete.  If  only  one  ear  is  complained  of  make  a  rapid  test  of  the 
hearing  of  the  better  ear  to  determine  that  it  is  functionally  sound  or 
at  least  only  slightly  subnormal.  This  preliminary  testing  of  the  sound 
ear  is  essential  to  a  proper  interpretation  of  tests  to  be  applied  later. 

Weber's  Test.  Apply  a  vibrating  tuning  fork  of  256  double  vibrations 
to  the  vertex  of  his  skull,  and  if  he  refers  the  sound  to  his  supposedly 
deaf  ear,  one  may  be, favorably  impressed  as  to  his  honesty.  If  he 
refers  it  to  his  sound  ear,  one  is  skeptical,  and  proceeds  to  the  next  test. 

Loud  Voice  r^.s-f.-— The  registrant's  eyes  are  now  blindfolded.  Re- 
questing him  to  close  with  a  finger  his  better  ear  which  has  already 
been  determined  to  be  approximately  sound  functionally,  words  and 
numbers  are  repeated  to  him  at  first  in  low  voice  and  then  in  progres- 
sively lower  and  louder  tones.  If,  when  a  pitch  and  intensity  at  which 
he  should  be  able  to  hear  and  interpret  the  words  with  the  sound  ear 
even  though  tightly  occluded  is  reached  he  still  states  that  he  cannot 
hear  it,  he  is  an  intentional  malingerer.  This  is  a  useful  test  which  will 
expose  many  malingerers.  An  alert  individual,  however,  may  throw 
this  procedure  out  of  court  by  stating  that  he  hears  the  soimd  through 
his  occluded  normal  ear. 

Stethoscope  Test. — For  this  the  ordinary  clinical  stethoscope  with 
funnel-shaped  chest-piece  is  used.  One  ear-piece  is  completely  occluded 
with  wax.  Occlusion  with  cotton  will  not  exclude  the  soimd.  Standing 
behind  the  registrant,  the  stethoscope  is  adjusted  with  the  occluded 
ear-piece  in  his  "deaf"  ear.  Words  in  a  low  whisper  are  spoken  into 
the  funnel-shaped  chest  piece,  which  naturally  he  should  hear  perfectly. 
The  stethoscope  is  removed  for  the  ostensible  purpose  of  trying  some 
other  test,  and  then  replaced,  the  occluded  ear-piece  being  this  time 
placed  in  his  sound  ear.  If  he  is  able  to  hear  now  approximatel\'  as  well 
as  before,  fairly  sound  evidence  is  present  that  his  deafness  is  either 
as.sumed  or  grossly  exaggerated.    This  is  a  fairly  reliable  test. 

Tests  for  FAlcitincj  Contradictor  if  Responses. — ^The  registrant's  eyes 
are  now  uncovered,  the  sound  or  better  ear  is  closed  with  a  finger,  and 
the  "deaf"  ear  is  subjected  rapidly  to  the  commoner  classical  tests,  e.  g., 
hearing  distances  for  watch,  acoumeter,  whisjx'r  or  conversational 
\'oice;  tuning-fork  tests  to  determine  lower  tone  range,  etc.  His  re- 
sponses, so  far  as  he  admits  hearing  in  an\'  of  the.se  tests,  are  carefull\" 
noted.    Following  this,  he  is  again  blindfoiderl  and  the  same  tests  are 


66     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

repeated  many  times,  fairly  rapidly  and  in  varying  order.  If  he  is  a 
malingerer  and  has  not  in  the  first  instance  claimed  almost  total  deaf- 
ness, his  responses  will  almost  surely  demonstrate  incongruous  and 
contradictory  variations. 

LomharcVs  Test. — This  test,  which  requires  a  Barany  noise  apparatus, 
is  one  of  the  mosi  dependable  means  of  determining  absolute  or  very 
advanced  deafness  confined  to  one  ear.  It  depends  upon  the  fact  that  to 
the  normal  man  the  sound  of  his  own  voice  is  necessary  to  the  proper 
regulation  of  its  tone  and  intensity.  It  is  carried  out  as  follows:  the 
noise  apparatus  is  adjusted  in  his  sound  ear  and  its  machinery  started 
in  order  to  accustom  him  to  its  grating  noise.  He  is  given  a  book  and 
told  to  read  aloud  in  his  natural  voice  and  not  to  stop  reading  when  the 
noise  instrument  is  set  in  action.  As  soon  as  the  noise  begins,  a  man 
whose  opposite,  or  open,  ear  is  profoundly  deaf  will  at  once  raise  his 
voice;  and  if  his  deafness  is  absolute,  he  may  literally  shout.  The 
malingerer,  on  the  other  hand,  claiming  a  one-sided  deafness  which  is 
not  real,  will  continue  to  read  in  an  even  tone  or  in  a  tone  only  slightly 
elevated.  This  is  a  test  which  a  malingerer  who  has  been  coached  may 
easily  turn  to  his  advantage.  Otherwise  it  is  one  of  the  most  useful 
at  one's  disposal.  With  a  quiet  room  and  sufficient  time  at  his  disposal, 
there  are  few  malingerers  who  will  be  able  to  deceive  a  careful  and 
experienced  examiner.  If  the  registrant  claims  absolute  unilateral 
deafness  and  his  reactions  to  the  commoner  tests  seems  to  justify  this 
claim,  he  should  raise  his  voice  almost  to  a  shout  when  a  noise  apparatus 
is  operated  in  his  sound  ear.  If  he  claims  only  partial  but  advanced 
deafness,  his  reactions  to  the  various  tests  should  be  approximately 
constant.  The  examiner  is  able  then,  usually  to  detect  the  malingerer; 
but  if  the  malingerer  really  suffer  from  some  degree  of  impairment,  it 
may  be  and  usually  is,  quite  impossible  to  determine  even  approxi- 
mately his  grade  of  deafness.  And  so  it  may  happen  that  a  man 
partially  deaf  may  be  misjudged. 

Bilateral  Deafness. — Naturally,  as  there  are  many  cases  representing 
all  grades  of  bilateral  deafness  malingering  may  take  the  form  merely 
of  an  exaggeration  of  an  existing  defect.  If  such  a  person  were  per- 
sistently to  assume  absolute  or  very  profound  bilateral  deafness,  it  is 
difficult  to  say  what  tests  would  expose  the  deception.  The  time- 
honored  tricks  of  attempting  to  ensnare  him  by  a  sudden  and  startling 
question,  the  use  of  insulting  or  disparaging  remarks,  etc.,  are  men- 
tioned in  many  test-books.  Such  crude  methods  are  usually  uncalled 
for,  however,  for  the  reason  that  the  risks  to  the  malingerer  of  such 
flagrant  lying  are  too  great.  The  pretense  of  partial  though  disabling 
deafness,  on  the  other  hand,  though  less  likely  to  be  exposed  through 
the  testimony  of  acquintances,  is  not  so  difficult  to  detect  for  the  reason 
that  the  malingerer  is  burdened  with  the  necessity  of  maintaining 
uniformity  in  his  responses  to  the  various  tests.  The  surest  means  of 
exposure  in  such  cases  is  the  application  of  the  classical  tests  first  to  one 
ear  and  then  to  the  other,  and  then,  after  blindfolding  him,  repeating 
the  same  tests  and  comparing  the  two  sets  of  responses. 


CRANIAL  NERVES 


67 


Aiiditory^orbicidaris  Reflex. — When  a  loud  sound  is  heard  close  by 
there  is  a  tightening  of  the  orbicularis  palpebrarum  fibers,  chiefly 
those  innervated  by  the  cervical  sympathetic.  This  is  a  useful  test 
for  psychogenic  deafness.  One  ear  being  tightly  closed  with  cotton, 
the  patient  faces  the  physician  who  with  a  magnifying  glass  of  about 
two  inches  focus  minutely  observes  the  orbicularis  muscle  response  to 
the  loud  honk  of  a  bicycle  or  automobile  horn  which  is  blown  just 
behind  the  patient  or  in  a  closely  contiguous  space.  Practically  no 
one  can  consciously  inhibit  this  sympathetic  reflex  response. 


Fig. 


21. — Asynergia  of  Babinski. 
(Schaller.) 


Fig.  22. — Dysmetria  of  Babinski  devel- 
oped on  attempting  to  take  hold  of  a  glass. 
The  fingers  are  held  very  far  open. 
(Thomas.) 


I  'erti()o. — This  belongs  largely  to  the  symptomatology  of  the  vestib- 
ular apparatus.  Equilibration  is  an  extremely  complex  adjustment 
of  eye  muscles,  kinetic  static  sense  and  the  muscle  sense  in  general, 
involving  the  comparative  integrity  of  a  large  number  of  neurones  from 
the  periphery  to  the  coordinating  centers,  which  latter  are  fairly  .satis- 
factorily proved  to  be  in  the  cerebellum.  The  vestibular  apparatus  is 
the  chief  ganglion  of  the  cranial  end  of  this  whole  apparatus  or  system 
termed  by  Sherrington  the  proprioceptive  system.  The  cerebellum 
is  its  chief  center.    A  comprehensive  anatomical  survey  of  the  path- 


68    METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

ways  involved  is  to  be  found  in  the  extremely  valuable  plate  worked 
out  by  Dejerine.    (See  Plate  VII.) 

Tests  for  Equilibration. — The  more  standard  equilibration  tests  are 
the  Romberg  and  the  Babinski  asynergic  tests.  The  Romberg  test 
is  obtained  by  having  the  patient  stand  erect  with  closed  eyes,  with 
heels  and  toes  together.  Under  normal  conditions  there  should  be  only 
a  very  slight  swaying,  but  the  person  with  well-marked  Romberg  sways 
sidewise,  or  backward,  or  forward,  or  may  even  fall  if  the  feet  are  not 
spread  apart.  Slight  degrees  of  Romberg,  or  unilateral  localization  of 
Romberg,  may  be  brought  out  by  having  the  patient  stand  on  one  or 
the  other  foot.  Other  observations  of  the  Romberg  can  be  made  by 
having  the  patient-  bend  forward  or  backward  or  sidewise,  and  in  this 
way  quantitative  suggestions  may  be  received  as  to  the  localization  of 
the  fiber  tracts  involved. 

In  certain  cerebellar  disturbances  there  is  a  special  loss  of  equilibrium 
which  Babinski  has  termed  asynergia.  This  may  be  tested  in  a  number 
of  different  ways.  The  more  valuable  are  by  means  of  walking.  The 
patient  usually  has  a  great  deal  of;  ataxia  and  walks  with  difficulty. 
If  supported  on  either  side,  it  is  noticed  that  in  walking  he  shows  a 
marked  tendency  to  walk  from  underneath  himself,  so  that  his  legs 
finally  are  put  out  far  in  advance  of  his  center  of  gravity.  The  same 
type  of  loss  of  cerebellar  equilibrium  may  be  demonstrated  by  having 
the  patient  stand  erect  and  then  slowly  bend  backward,  making  an 
arc  de  cercle.  In  the  healthy  condition  the  muscular  adaptation 
bends  the  knees  forward  and  the  indvidual  assumes  a  well-balanced 
position;  but  in  the  asynergic,  the  legs  are  held  straight,  the  trunk  is 
bent  backward  and  the  patient  has  a  tendency  to  fall  backward.  A 
patient,  on  grasping  a  glass  opens  the  hand  far  wider  than  needed. 
This  is  dysmetria,  a  sign  of  cerebellar  disturbance. 

For  the  upper  extremities  a  similar  type  of  muscular  incoordination 
shows  itself  in  the  typical  diadokokinesis  tests.  These  tests  are  made 
by  having  the  patient  perform  certain  alternating  or  opposing  move- 
ments very  rapidly,  such  as  quickly  pronating  and  supinating  the  hand 
in  a  semiflexed  position,  or  making  rapid  piano-playing  movements 
with  the  fingers,  or  sudden  flexions  and  extensions  of  the  arm  on  the 
forearm.  In  involvements  of  certain  of  the  cerebellar  tracts  usually 
associated  with  equilibrium  there  is  a  marked  inability  to  perform 
certain  of  these  movements;  they  are  done  clumsily,  irregularly  and 
slowly,  depending  on  the  grade  of  sensory  tract  involvement. 

For  testing  the  equilibrium  of  the  trunk  the  patient  should  be  on  the 
back,  with  the  arms  folded,  and  with  the  legs  drawn  up  in  the  obstetrical 
position.  Here  considerable  variation  will  be  found.  The  healthy 
individual,  if  on  a  fairly  hard  foundation,  can  equilibrate  fairly  well; 
there  is  but  very  little  swaying,  while  the  ataxic  patient  sways  consid- 
erably. Cerebellar  patients  also,  and  certain  type  of  cases  with  fronto- 
cerebellar  pathway  disturbances  show  what  is  known  as  cataleptic 
rigidity  in  this  position.  After  swaying  for  a  certain  length  of  time  the 
legs  and  body  steadih'  become  fixed  with  sufficient  rigidity  to  enable 


CRANIAL  NERVES  69 

them  to  be  photographed.  This  sign  of  cataleptic  rigidity  is  considered 
by  Babinski  as  characteristic  of  cerebellar  defect. 

In  further  testing  equilibrium  the  gait  occupies  an  important  place. 
The  patient  should  be  directed  to  walk  along  a  crack,  or  a  straight 
line,  first  witli  the  eyes  open ;  he  should  be  asked  to  suddenly  reverse, 
inquiry  being  made  if  there  are  symptoms  of  vertigo  in  the  reversion 
either  to  the  right  or  to  the  left,  for  both  sides  should  be  tested,  and  then 
the  patient  should  be  directed  to  return  with  his  eyes  closed  when,  if 
disturbance  of  equilibrium  be  present,  it  will  become  intensified,  and 
staggering  to  the  right  or  to  the  left,  forward  or  backward,  will  be  noted 
(lateropulsion,  propulsion,  or  retropulsion) . 

The  patient  should  also  be  tested  by  walking  on  the  toes,  on  the  heels; 
he  should  also  be  asked  to  walk  side  wise. 

Inspection  of  the  shoes  (heel  and  sole)  is  highly  desirable  if  there 
are  minor  modifications  of  gait,  there  are  characteristic  worn  spots 
for  various  forms  of  motor  weakness  or  stiffness. 

Various  modifications  in  gait  are  known  as  ataxic,  as  reeling  or 
drunken,  as  spastic,  scissors  gait,  high  stepping  (steppage),  shuffling, 
pendulum  gait  of  hemiplegics,  gait  with  little,  short  steps,  titubating, 
choreic,  propulsion.  Stilted  gait  is  a  t^'pe  seen  in  schizoplu-enia. 
Irregular  gaits  are  noted  in  hystericals;  here  walking  sidewise  is  useful 
in  distinguishing  a  hysterical  hemiplegic  from  a  hemorrhagic  one. 
The  hysteric  swings  the  leg  out  in  the  arc  of  a  circle,  the  hemiplegic 
raises  the  pelvis  and  the  toes  stick  to  the  ground  (spastic). 

The  subjective  sense  of  giddiness  is  often  extremely  complicated. 
Certain  patients  complain  of  objects  turning  about  them,  in  which 
case  the  direction  of  the  moving  object,  in  terms  of  the  hands  of  a 
clock,  should  always  be  noted.  Association  of  giddiness  or  vertigo 
with  eye  disturbances  is  very  widespread. 

The  internal  ear  has  such  an  intimate  relation  with  the  central  ner- 
vous system  that  its  study  is  of  especial  interest  to  the  neurologist.^ 
The  Barany  ear  tests  are  of  particular  value  in  making  a  differential 
diagnosis  between  lab\Tinth  and  intracranial  lesions  and  in  furnishing 
additional  data  in  intracranial  localization.  Nystagmus  and  vertigo, 
with  loss  of  equilibrium,  associated  perhaps  with  nausea  and  vomiting, 
may  be  produced  either  by  a  disturbance  of  the  internal  ear  or  by  an 
intracranial  lesion.  In  many  instances  the  symptoms  of  internal  ear 
disturbances  and  of  a  cerebellar  lesion  are  identical.  It  is  in  such  a 
differentiation  that  the  ear  tests  are  often  invaluable.  It  not  rarely 
happens  that  a  careful  neurologic  study  indicates  a  lesion  of  the  cere- 
bellum, whereas  the  ear  examination,  by  giving  additional  data  to  the 
neurologist,  demonstrates  conclusively  that  one  is  dealing  with  a  lesion 
of  the  labyrinth. 

A  differential  diagnosis  between  peripheral  and  central  lesions  by 
means  of  the  ear  tests  depends  on  certain  general  principles. 

A  peripheral  lesion  of  the  labyrinth  or  eighth  nerve  is  suggested 
by  the  following,  as  given  by  Jones: 

>  Jones,  I.  H.:  The  Value  of  Ear  Examination  to  the  Neurologist,  Tr.  Am.  Otolog. 
Soc,  XX,  p.  55.     Equilibrium  and  Vertigo,  J.  B.  Lippincott  Co.,  1918. 


70    METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

1.  An  impairment  of  the  function  of  both  the  cochlear  and  kinetico- 
static  labyrinth.  If,  for  example,  the  hearing  tests  show  cochlear  deaf- 
ness and  the  tests  of  the  semicircular  canals  show  that  their  function  is 
also  impaired,  it  immediately  becomes  probable  that  an  end-organ 
lesion  is  present. 

2.  The  history  or  presence  of  tinnitus;  the  absence  of  tinnitus  does 
not  necessarily  indicate  that  the  end-organ  is  not  involved,  but  its 
presence  is  very  suggestive  of  labyrinth  involvement. 

3.  Proportionate  impairment  of  the  responses  from  the  horizontal 
canal  and  of  the  responses  from  the  vertical  canals.  If,  for  example, 
the  tests  show  that  the  horizontal  canal  retains  only  one-half  of  its 
normal  function  and  that  the  vertical  canals  similarly  retain  only  one- 
half  of  their  normal  function,  a  lesion  of  the  end-organ  itself  is  suspected. 

4.  Proportionate  impairment  of  both  nystagmus  and  vertigo.  If 
the  horizontal  canal  produces  one-third  of  the  normal  nystagmus  and 
one-third  of  the  normal  vertigo,  it  is  suggested  that  the  lesion  is  in  the 
horizontal  canal  itself  or  in  the  fibers  from  the  canal  within  the  eighth 
nerve;  if,  in  addition,  the  vertical  canals  produce  one-third  of  the 
normal  nystagmus  and  one-third  of  the  normal  vertigo,  an  end-organ 
lesion  is  strongly  suggested.  In  a  word,  it  is  the  "proportionate 
impairment"  of  responses  that  speaks  for  a  peripheral  lesion. 

A  central  lesion  is  suggested  by  the  following: 

1 .  A  normal  cochlea  but  impaired  or  non-responsive  semicircular  canals. 

2.  Normal  responses  from  the  horizontal  canal  but  absent  responses 
from  the  vertical  canals. 

3.  Normal  responses  from  the  vertical  canals  but  impaired  responses 
from  the  horizontal  canal. 

4.  Normal  vertigo  but  impaired  nystagmus  from  the  horizontal  canal. 

5.  Normal  nystagmus  but  impaired  vertigo  from  the  horizontal  canal. 

6.  Normal  vertigo  but  impaired  nystagmus  from  the  vertical  canals. 

7.  Normal  nystagmus  but  impaired  vertigo  from  the  vertical  canals. 

8.  Normal  vertigo  and  normal  nystagmus  from  any  semicircular  canal, 
but  impaired  past  pointing  in  any  direction  of  any  one  extremity. 

9.  Normal  vertigo  and  normal  nystagmus  from  any  semicircular  canal 
but  an  impairment  or  absence  of  the  normal  falling. 

10.  Spontaneous  vertical  nystagmus  is  pathognomonic  of  a  central 
lesion  and  is  indicative  of  involvement  of  the  brain  stem  caused  either 
by  their  infiltration  or  pressure.  A  lesion  of  the  labyrinth  may  produce 
many  forms  of  spontaneous  nystagmus — horizontal,  rotary,  oblique, 
or  a  mixed  nystagmus  of  the  various  types;  but  an  ear  lesion  does  not 
produce  a  spontaneous  vertical  nystagmus,  either  upward  or  downward. 

11.  If  there  exists  a  spontaneous  nystagmus  to  the  right  and  non- 
responsive  semicircular  canals  of  the  right  ear,  an  intracranial  lesion  is 
suggested.  The  non-responsive  labyrinth,  if  the  labyrinth  itself  alone 
were  responsible,  would  produce  a  nystagmus  to  the  left. 

12.  A  spontaneous  nystagmus  of  increasing  intensity  or  of  long 
duration  is  indicative  of  a  central  lesion.  A  spontaneous  nystagmus 
due  to  a  lesion  of  the  labyrinth  shows  its  greatest  intensity  at  the  onset 


CRANIAL  NERVES  71 

of  the  disease,  becomes  less  and  less  marked  and  disappears  after  a 
few  days. 

13.  If  a  stimulation  of  any  semicircular  canal  produces  a  "  perverted" 
or  "inverse"  nystagmus,  it  is  pathognomonic  of  a  central  lesion  and  is 
indicative  of  brain-stem  involvement.  Such  phenomena  as  the  following 
are  frequently  seen :  Douching  the  right  ear  with  cold  water  with  the 
head  back  sixty  degrees,  stimulating  the  right  horizontal  canal,  should 
produce  a  pure  horizontal  nystagmus  to  the  left.  If  on  stimulation 
there  occurs  a  vertical  nystagmus  upward  or  downward,  a  rotary, 
oblique,  or  mixed  nystagmus,  it  may  be  spoken  of  as  "perverted."  If, 
instead  of  a  horizontal  nystagmus  to  the  left,  there  is  produced  a  pure 
horizontal  nystagmus  to  the  right,  it  may  be  termed  an  "inverse" 
nystagmus.  Neither  a  perverted  or  an  inverse  nystagmus  can  possibly 
be  produced  by  a  lesion  of  the  labyrinth  or  eighth  nerve ;  a  peripheral 
lesion  produces  a  poor  nystagmus  or  no  nystagmus  at  all,  but  an 
absolutely  false  response  of  necessity  demonstrates  a  central  lesion. 

14.  If  ear  stimulation  produces  a  conjugate  deviation  of  the  eyes 
instead  of  a  nystagmus,  it  is  pathognomonic  of  a  central  lesion. 

This  outline  indicates  how  additional  data  may  be  furnished  to  the 
neurologist  by  the  ear  tests  in  determining  whether  a  lesion  of  the 
internal  ear  or  of  the  brain  stem  or  cerebellum  is  present.  In  the 
broader  field  of  localization,  examination  of  the  ear  and  of  the  vesti- 
bular apparatus  is  also  of  distinct  value.  The  particular  feature  of  the 
ear  examination  is  that  the  aurist  sends  in  a  stimulus  to  the  brain 
centers,  and  then  notes  the  responses  of  the  different  parts  of  the  body 
to  this  stimulus.  For  example,  by  stimulation  of  the  ear  there  results 
a  nystagmus  in  a  given  direction,  a  pointing  of  the  extremities  to  the 
right  or  to  the  left,  as  the  case  may  be,  and  a  falling  to  the  right,  to  the 
left,  forward  or  backward  as  the  case  may  be.  Now,  if  the  ear  and  these 
central  paths  from  the  ear  are  intact,  all  the  normal  responses  will 
appear.  If  there  is  a  failure  of  all  or  any  of  the  responses  it  is  positive 
evidence  of  an  interruption  along  that  particular  path  or  paths  that 
fail  to  bring  about  these  responses. 

In  order  to  utilize  the  knowledge  obtained  from  these  tests  it  is 
essential  to  have  in  mind  the  various  pathways  constituting  the  vestib- 
ular apparatus.  It  has  been  demonstrated  that  the  pathways  from  the 
horizontal  semicircular  canal  are  different  after  entering  the  brain  stem 
from  those  of  the  vertical  canals;  furthermore,  that  each  set  of  tracts 
divides  into  two  separate  pathways;  one  pathway,  the  vestibulo- 
ocular  tract,  is  responsible  for  the  eye  movement,  and  the  other  path- 
way, the  vestibulocerebellocerebral  tract,  conveys  the  impulses  from 
the  ear  to  the  cerebral  cortex,  producing  vertigo.  If  the  horizontal 
canal  fails  to  produce  both  nystagmus  and  vertigo  the  lesion  indicated 
is  at  a  point  before  the  division  of  the  horizontal  canal  fibers  into  their 
two  separate  pathways.  Further,  if  the  horizontal  canal  produces 
normal  vertigo  but  no  nystagmus,  the  lesion  indicated  is  in  the  vestib- 
ulo-ocular  tract  at  a  point  beyond  the  point  of  division  into  the  two 
paths.     If  the  horizontal  canal  produces  normal  nystagmus  but  no 


72     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

vertigo,  the  lesion  indicated  is  at  a  point  along  the  vestibulocerebello- 
cerebral  path  beyond  the  point  of  division  into  the  two  pathways. 
Similai-h-,  if  the  vertical  canals  produce  normal  vertigo  but  no  nystag- 
mus the  lesion  indicated  is  in  the  vestibulo-ocular  tract  at  a  point 
beyond  the  division  into  the  two  paths.  If  the  vertical  canals  produce 
normal  nystagmus  but  no  vertigo,  the  lesion  indicated  is  at  a  point 
along  the  ^•estibnlocerebellocerebral  path  beyond  the  point  of  division 
of  the  two  pathways.  The  ear  tests  have  proved  themselves  sur- 
prisingly helpful  in  locating  lesions  in  the  cerebellopontine  angle, 
medulla  oblongata,  pons,  cerebellar  peduncles,  cerebellum  and  the 
parietal,  temporal  and  occipital  lobes. 


Fig.  23. — Organic^laryngeai  paralyses.  (Barwell.)  J,  left  abductor  paralysis  during 
inspiration;  //,  left  abductor  paralysis  during  phonation;  ///,  left  recurrent  laryngeal 
paralysis,  during  inspiration;  IV,  left  recurrent  laryngeal  paralysis,  during  phonation. 

Examination  of  the  Pharynx. — Careful  note  should  be  made  of  the 
position  of  the  fauces  while  at  rest  and  during  phonation,  and  of  the 
movements  of  the  soft  palate  during  phonation.  The  pharyngeal 
reflexes  are  tested  by  touching  successively  the  right  and  left  pillar, 
and  the  vomiting  reflex  is  best  tested  by  irritating  the  back  of  the 
pharynx  with  a  wooden  spatula. 

While  under  normal  conditions  the  uvula  should  hang  in  the  middle 
line,  a  certain  amount  of  variation  in  position  is  very  frecpiently 
observed. 

While  the  pharynx  is  being  examined,  note  should  be  made  of  the 
pharyngeal  vault,  and  also  of  the  contour  of  the  posterior  pharyngeal 
walls.  Further,  the  integrity  of  the  superior  pharyngeal  muscles 
should  be  tested  by  having  the  patient  swallow,  and  noting  whether 


SPEECH  EXAMINATIOX  73 

the  act  is  coordinated,  or  whether  there  is  regurgitation  through  the 
nose. 

Exann'naiion  of  Taste. — Taste  is  a  complex  function  and  utiUzes  at 
least  three  different  nerves.  It  is  best  tested  by  solutions  which  are 
sweet  (sugar),  bitter  (quinine),  acid  (vinegar),  salty  (salt  solutions). 
The  solutions  should  be  kept  in  wide-mouthed  bottles,  and  are  applied 
in  small  quantities  by  means  of  a  glass  rod  applied  to  different  parts 
of  the  tongue  to  determine  the  functional  capacity  of  these  parts. 
Small  amounts  should  be  used,  and  it  is  best  to  reserve  the  bitter  test 
until  the  last,  commencing  with  the  sweet,  and  following  with  the  sour, 
the  salt,  and  the  bitter.  The  mouth  should  be  washed  out  between 
the  tests  if  careful  records  are  to  be  made. 

Taste  can  also  be  tested  by  means  of  a  minimal  galvanic  current 
when  the  positive  pole  will  produce  an  acid  taste,  the  negative  pole 
an  alkaline  or  salty  one. 

While  testing  for  taste  the  condition  of  the  secretion  of  the  buccal 
mucous  membrane  can  be  tested.  This  is  best  done  by  rubbing  the 
floor  of  the  mouth  with  a  glass  rod  and  noting  the  rapidity  of  excretion 
and  the  amount. 

Examination  of  the  Larynx. — This  naturally  has  to  be  carried  out 
by  means  of  a  laryngological  mirror,  when  malposition  or  changed 
position  of  the  vocal  cords  during  inspiration,  expiration  and  phona- 
tion  are  observed.  Fig.  23  shows  the  position  of  the  vocal  cords  in 
four  characteristic  palsies. 

Examination  of  Speech. — A  complete  analysis  of  the  function  of 
language  will  not  be  entered  into  in  this  place.  The  chief  points  of 
neurological  interest  to  be  observed  are  whether  the  tongue  is  protruded 
in  the  middle  line,  whether  it  is  freely  movable,  up,  down,  right  and 
left,  and  can  be  made  to  push  out  both  cheeks.  Careful  search  should 
be  made  for  scars  on  the  tongue,  and  the  presence  of  a  leukoplakia  on 
the  sides  of  the  tongue  or  of  the  mucous  membrane  of  the  cheeks 
should  not  be  overlooked. 

Tremors  of  the  tongue  may  be  very  fine,  involving  the  whole  organ 
(fibrillary),  or  coarse  and  irregular.  Considerable  attention  should  be 
devoted  to  the  search  for  tongue  tremors. 

In  testing  ordinary  speech,  certain  test  phrases  ar6  advisable.  The 
patient  should  be  directed  to  repeat  the  alphabet,  and  the  numbers 
up  to  twenty-five,  and  should  repeat  something  well  known,  such  as 
the  Lord's  Prayer,  or  some  bit  of  poetry,  and  during  the  repetition 
careful  attention  should  be  directed  to  the  enunciation  of  the  individual 
letters,  to  the  presence  of  stumbling  over  words,  of  running  words 
together,  to  the  omissions  of  words,  or  the  omission  of  syllables,  and 
particularly  to  the  repetition  and  the  displacement  of  syllables.  In 
order  to  bring  out  some  of  these  defects,  certain  test  phrases  are  utilized. 
Among  the  most  valuable  are  the  following:  Truly  Rural;  Third 
Riding  Artillery  Brigade;  Methodist  Episcopal;  National  Intelligencer. 
Naturally  the  type  of  case  will  suggest  certain  defects,  which  can  then 
be  examined  for. 


74     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

In  stating  the  speech  defects  due  to  laryngeal  loss,  special  attention 
should  be  directed  to  the  presence  of  cough,  of  stridor,  and  to  the  pitch 
of  the  breathing. 

The  speech  is  further  tested  by  having  the  patient  repeat  foreign 
words,  read  spontaneously,  read  after  writing,  and  define  spoken, 
written,  and  printed  words. 

Aphasic  Status. — A  brief  aphasic  status  should  include  the  following 
tests : 

First  ascertain  the  usual  habits  of  the  patient  and  of  the  parents  with 
reference  to  the  use  of  the  right  and  left  hand  in  every-day  acts. 

1.  Is  spontaneous  speech  possible  and  is  it  intelligible  or  non-intelli- 
gible? Record  should  be  made  of  the  choice  of  words,  complete  steno- 
grams  of  the  words  uttered  should  be  made.  Attention  should  be  given 
to  variations  in  pronunciation,  cadence,  and  rhythm.  Observe  "word 
salad,"  "jargon  aphasia,"  "jumbled,  precipitate  speech,"  "stumbling 
speech,"  "torrent  speech,"  "hot-potato  speech." 

2.  Doesthepatient  understand  words?  This  is  tested  by  asking  him 
questions,  which  should  not  be  too  simple,  such  as  what  is  his  name, 
etc.  He  should  be  asked  to  touch  his  nose,  his  left  ear,  the  top  of  his 
head,  his  right  knee.  These  should  all  be  asked  without  any  trace  of 
movement  on  the  part  of  the  inquirer  and  preferably  so  that  the  patient 
cannot  see  the  lip  movements. 

3.  The  knowledge  of  written  or  printed  words  should  be  tested.  The 
same  commands  as  previously  mentioned  (2)  should  be  written  out  and 
shown  to  him. 

4.  Can  the  patient  repeat  words  spoken  such  as  Sasaktai,  Constanti- 
nople, hoi-polloi,  or  mouse,  rose,  sunfish,  etc. 

5.  Can  he  write  spontaneously,  left  hand,  or  on  typewriter.  Observe 
misplacing  and  similar  defects,  as  in  speaking  (1).  Is  he  able  to  write 
from  dictation?    Is  he  able  to  copy  what  he  sees? 

6.  Can  he  name  objects  pointed  out;  is  he  able  to  recognize  objects 
after  their  name  is  spoken,  or  their  names  written?  Can  he  pick  out 
objects  named,  written,  or  shown? 

7.  Can  he  obey  commands  calling  for  simple  gestures,  such  as  bow- 
ing, throwing  a  kiss,  clenching  the  hand  in  definace,  either  in  response 
to  spoken  wish  or  to  imitative  gesture? 

8.  Can  he  understand  the  use  of  objects,  such  as  striking  a  match, 
using  a  paper  cutter?     (Apraxic  tests). 

Stuttering  is  a  spasmodic  form  of  speech  disturbance  which  calls  for 
special  mention.  Certain  patients  show  a  very  marked  slowing  of 
speech  (bradylalia),  while  others  show  an  intermittent  enunciation, 
and  still  others  a  peculiar,  monotonous,  semi-singsong  type  of  enuncia- 
tion known  as  "  scanning  speech."  In  complicated  speech  disturbances 
due  to  involvement  of  the  hypoglossal  nerve  one  obtains  the  so-called 
bulbar  thick  speech :  the  patient  speaks  as  though  he  had  a  hot  potato 
in  his  mouth.  Further,  in  extensive  speech  disturbances  due  to 
coexisting  lesions  in  different  parts  of  the  speech  mechanism  one 
has  other  disturbances  known  as  anarthria,  or  more  particularly  as 


HEAD  AND  NECK 


75 


dysarthria,  or  "jumbled  speech."  Special  attention  should  be  given  in 
stuttering  or  stammering  to  the  type,  whether  dental,  lingual,  labial, 
etc.,  and  a  list  made  of  the  characteristic  hitches  of  the  patient.  It  will 
be  found  they  usually  have  some  symbolic  significance,  which  the 
psychoanalytic  technic  may  reveal. 

The  Tenth  Nerve. — The  study  of  the  heart  action  and  tests  is 
referred  to  the  section  on  the  Vegetative  Nervous  System. 

The  Eleventh  or  Spinal  Accessory  Nerve. — This  nerve  supplies  the 
sternomastoid  and  the  trapezius.  Its  functional  capacity  is  tested 
by  the  ability  to  raise  the  shoulders  and  to  turn  the  neck,  pressure 
being  made  on  the  chin  in  resistance.  There  are  a  number  of  striking 
displacements  resulting  from  paralysis  of  this  nerve  which  will  be 
discussed  later  under  the  head  of  Paralysis. 

Ninth,  Tenth,  and  Tivelfth  Nerves. — The  glossopharyngeal,  vagus, 
and  h^-poglossal  nerves  may  be  considered  more  or  less  together  in 
their  testing.  The  glossopharyngeal  is  involved  in  the  function  of 
taste  of  the  posterior  third  of  the  tongue  and  of  the  soft  palate.  It 
is  also  a  nerve  of  common  sensation  for  the  back  of  the  tongue,  part 
of  the  soft  palate,  and  upper  part  of  the  pharynx.  It  supplies  the 
middle  constrictor  of  the  pharynx  and  the  stylopharyngeus  with 
motor  fibers. 

For  further  details  of  the  testing  of  the  other  nerves  see  the 
chapter  on  Cranial  Nerves. 

Head  and  N6ck. — The  muscles  that  move  the  head  and  neck,  their 
function,  their  nerve  supply,  and  the  spinal  segment  in  which  the 
motor  synapses  are  located  are  as  follows  (Figs.  27,  28,  29  and  30) : 


Muscle. 
Platysma  myoides. 


Rectus  capitis  antici. 
Rectus  capitis  lateralis. 

Rectus  capitis  posticus. 

Scalenus  medium. 

Scalenus  posticus. 

Scalenus  anticus. 

Splenius  capitis. 
Trapezius. 

Sternocleidomastoid. 

Levator  anguli  scapuli. 

Complexus. 
Obliquus  superior. 
Obliquus  inferior. 


Function. 
Depresses  the  angle  of  the 

jaw    and    mouth;    draws 

tense    and    wrinkles    the 

skin  of  the  neck. 
Flexion  of  the  head. 
Lateral      movement      and 

slight  rotation. 
Extension  and  rotation  on 

same  side. 
Lateral      movement      and 

slight  exten.sion. 
Lateral      movement      and 

slight  extension. 
Lateral      movement      and 

slight  extension. 
Extension. 
Lateral  and  extensor. 

Lateral,  flexion  and  rota- 
tion to  opposite  side. 

Lateral  and  rotation  on 
same  side.  Rai.se  angle 
of  scapula. 

Extension  and  rotation  on 
same  side. 

Extension  and  rotation  to 
opposite  side. 

Rotation  to  same  side. 


Nerve  Supply  and 
Spinal  Segment. 
Facial,  C2. 


Cervical  branches,  CI,  C4. 
Anterior  cervical,  Cl-6. 

Posterior  cervical,  CI. 

Anterior  cervical,  C2-8. 

Anterior  cervical,  C5-8. 

Anterior  cervical,  C4-7. 

Cervical  nerves,  C2-8. 
Spinal   accessory,    cervical, 

C2-4. 
Spinal   accessory,    cervical, 

C2-4. 
Anterior  cervical,  C3-5. 

Posterior  cervical,  Cl-8. 
Posterior  cervical,  CI. 
Posterior  cervical,  C2. 


7G    METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 


Fig.  24. — Partial  atrophy  of  tongue  in  occlusion  of  inferior  cerebellar  artery. 


Fig.  25. — Pronounced  atrophy  and  hypotonus  in  a  patient  with  amyotonia  congenita. 

(Abrahamson.) 


UPPER  EXTREMITIES  77 

Upper  Extremities. — A  systematic  examination  of  the  upper  extrem- 
ities is  next  in  order,  the  muscular  apparatus  first  claiming  attention. 
The  two  sides  of  the  body  should  be  examined  systematically.  (See 
Figs.  27  to  30.) 

Anomalies  of  structure  should  first  be  noted,  such  as  habit  or 
occupational  positions,  alterations  of  posture,  etc.  Gross  differences 
in  the  size  of  the  bones,  the  wrists,  hands,  etc.,  should  be  measured. 

Atrophy. — This  may  be  determined  by  simple  palpation  and  by 
measurement.  After  natural  differences  in  the  muscular  volume 
are  taken  into  consideration,  striking  variations  should  be  carefully 
measured.  The  circumferences  over  the  biceps  and  just  below  the 
elbows  on  the  two  sides  should  be  compared.  Special  individual 
muscular  atrophies  may  be  picked  out  later  by  means  of  electrical 
reaction  tests. 


Fig.   26. — Marked  hypotonus  in  a  patient  with  amyotonia  con.;enita.     (Neustadter.) 

Hypertrophy. — This  is  best  brought  out  by  palpation  and  by  measure- 
ment. Due  weight  should  be  given  to  natural  variations — right  and 
left  sides — and  to  the  influence  of  certain  occupations — blacksmiths, 
iron-workers,  etc. 

Hypotonus. — This  is  indicated  by  unusual  fiaccidity  of  the  muscu- 
lature and  movements.  Sudden  pronation  or  supination  of  the  arm, 
extension  or  flexion  at  the  shoulder-,  wrist-  or  elbow-joints,  may  show 
sudden  sharp  resistances,  followed  by  marked  flaccidity.  Marked 
overextension,  etc.,  is  a  sign  of  hypotonus. 

The  observer  should  bear  in  mind  that  muscular  function  is  con- 
trolled by  vegetative  as  well  as  by  sensorimotor  nerves. 

Spasm. — This  indicates  hypertonus.  When  permanently  present 
contractures  result.  The  particular  muscles  which  show  hyi)ertonus, 
or  spasticity  or  contractures,  should  be  recorded.  In  certain  spastic 
conditions  the  hypertonus  may  be  relieved  by  passive  movements. 

Mnsculdr  Power. — This  is  first  tested  i)y  having  the  ])atient  execute 
all  the  chief  movements  of  the  shoulders  and  arms.  The  chief  tests 
are  as  follows: 


78     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 


Trapezius  XI Ci,  i 
Sterno  cleido  mastoid 
XI.  C2-S~ 


Subclavius  Co,  I 
Deltoid- 
Sub  scapular  is  CoW)/ 
Coraco  brachialis 
Cff,  7 
Pectoralis  ininqr_ 
Latissimus  dorst 
CG-8~ 


Superficial  muscles 
of  the  face  VII 


Infei  101  hyoid  region. 

XII  pair 

Intel  nal  descending 

blanch  C2,3 

Platysma  myoides 
^^11+02 


Serratus  magnus 


Biceps- 
Transversalis  _ 


Brachialis  anticus 

Co-0 
Internal  oblique - 

Supinator  brevisf 
C5,  6,  7 


Internal 
rectus 
L2-i 
'Adductor  magnus 

I^-^    Anterior  rectu 
{Red.  fern.) 


Pectoralis  major 
Xclavicular) 
Deltoid 

Pectoralis  major 
{sternal) 

Pectoralis  major 
{costal) 


-Biceps 

Brachialis  anticus  C5,  6 
^Pronator  rad. 
teres 
E^tens.  carl), 
^ad.  I.  C(5)e,  7 
Suinnator 
'  longus 
I  Extens.  carp, 
rad.  brev. 
-^€{5)6,  7 
Palmaris 
'longus 
Palmaris 
'  brev  is 
-  Flexor 
sublimis 
\digitorum 
CI,  8  Dl 
^Flexor 
^  longus 
j)olHcis 


Anterior  rectus 
{Beet,  f em.) 


Fig  27  —Radicular  innervation  of  the  muscles  of  the  anterior  regions  of  the  head, 
neck  trunk,  and  upper  extremities.  On  the  left,  the  superficial  muscular  layer;  on  the 
right,  the  deep  muscular  layer.     (After  Dejerine.)     C,  cervical;  D,  dorsal;  L,  lumbar. 


UPPER  EXTREMITIES 


79 


Inferior  maxillary  ^^ 

Internal  descending  \ 

branch  cervical  plexus 

Hypoglossal  •■ 

N.  to angularis  \^  „ 
N.toscaleni       i       •\ 

N.  of  rectus  caintis 

anticus  major 

C.P.  CI  i 


Anterior  thoracic  int. 


Axillary 

b.p: 


p^k 

—-n  Sk, 

V--- 

-f-,/:  » 

-^ 

^ 

? 

-"S^-S 

i>^ 

-^ 

% 

B 

M 

w 

m 

itr^ 

Maxillary  inferior  N. 

Spinal  accessory  XI 
'Sternocleidomastoid  C.P, 

Sjfinal  accessory  XI 
^to  trapezius  C.P. 


Subseapularis 

sup.  et  inf. 

Brachial  plexus 


Latissimus  dorsi 

B.P. 

Posterior  thoracic 

B.P. 

Musculo  cutaneous 
Brachial  plexus 

Musculo  cutaneous 

and  radial 

Brachial  plexus 

Intercostals 
Hypogastric 


'ircumflex 
^Brachial.plexuB 

Anterior 
thoracic  ext. 
Brachial  plexus 

Anterior 
thoracic  irit. 
Brachial  plexus 
-Musculo 

cutaneous  B.P. 


Intercostals 
Hypogastric 


Median  B.P. 


Radial 


Median 
B.P. 


Hadial  B.P. 


Median 


r 


Obturator  • 

Lumbar  idexus 

Fig.  28. — Peripheral  innervation  of  the  anterior  niusfles  of  the  head,  neck,  tnink, 
and  upper  extremities.  To  the  left  the  superficial  muscular  layer;  the  deep  muscular 
layer  to  the  right.  The  same  markings  on  this  and  the  preceding  figure  are  followed 
for  the  facial,  trigeminal,  spinal  accessory,  hypoglossal,  cervical  plexus,  and  inlercostal 
nerves  respectively.  Different  shadings  differentiate  different  innervations  in  the 
brachial  plexus.  (After  Dejerine.)  The  chief  plexu.ses  are  indicated.  C.P.,  cervical 
plexus;  B.P.,  brachial  plexus;  L.P.,  lumbar  plexus;  S.P.,  sacral  plexus;  D.  in  Fig.  27 
indicates  dorsal  roots;  /  5-7,  /  9-12,  etc.,  in  Fig.  28  intercostal  nerves. 


80     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

Shoulder,  Arm,  Hand,  and  Fingers. —  Deltoid. — Request  the  patient 
to  raise  the  arms  laterally  to  a  horizontal  position.  Inability  so  to  do 
indicates  deltoid  weakness  or  paralysis. 

Trapezius. — Ask  the  patient  to  raise  the  shoulders  as  close  to  his 
ears  as  possible  against  the  pressure  of  the  examiner's  hands.  This 
will  demonstrate  the  strength  of  the  upper  part  of  the  trapezius.  The 
middle  and  lower  portions  are  tested  by  desiring  him  to  bring  the 
scapulse  as  close  together  as  possible. 

Latissimvs  Dorsi. — Raise  the  arms  laterally  to  a  level,  then,  while 
keeping  them  fully  extended,  bring  the  arms  downward  and  back- 
ward as  if  to  make  the  hands  meet  beyond  the  sacrum.  The  examiner 
standing  behind  the  patient  resists  the  movement. 

Pectoral  Muscles. — Stretch  out  the  arms  straight  in  front  and  then 
approximate  the  hands  against  resistance  by  the  examiner,  meanwhile 
watching  both  heads  of  the  pectoral  muscle. 

Serratus  Magnus. — Desire  the  patient  to  push  with  his  hands  against 
those  of  the  examiner  or  against  a  solid  object.  If  the  serratus  has 
lost  its  power  the  scapula  will  project  and  the  digitations  of  the  muscle, 
which  ordinarily  should  be  visible,  will  not  be  seen.  Inspiration  is 
weakened  in  paresis  or  paralysis. 

It  is  hardly  possible  to  detect  paralysis  of  the  levator  anguli  scapulae 
and  rhomboids  unless  the  trapezius  is  also  involyed^ 

Biceps. — Let  the  patient  flex  his  extended  arm,  his  elbow  resting' 
in  the  observer's  left  hand,  while  the  latter's  right  hand,  grasping  the 
WTist  of  the  patient,  ofiers  the  necessary  resistance.     Also  supinate 
the  hand  against  resistance. 

Triceps. — The  triceps  may  be  tested  as  are  the  biceps,  excepting 
that  the  previously  flexed  arm  is  to  be  extended  against  resistance. 

Supinator  Longus. — Test  as  for  the  biceps,  except  that  the  hand 
should  be  midway  between  supination  and  pronation.  If  the  muscle 
is  paralyzed  it  will  fail  to  become  conspicuous  on  the  radial  side  of  the 
upper  part  of  the  forearm. 

Flexors  of  the  Wrist. — Grasping  the  patient's  hand,  the  palm  being 
upward,  desire  him  to  bend  the  hand  up  toward  his  forearm  against 
resistance. 

Extensors  of  the  Wrist. — ^The  patient's  hand  being  held  palm  down- 
ward, he  is  required  to  bend  it  backw^ard  against  resistance.  Moderate 
weakness  of  the  extensors  of  the  wrist  may  be  manifested  by  asking 
him  to  squeeze  the  examiner's  hand,  in  which  case  the  wrist  will  become 
involuntarily  flexed,  the  weakened  extensors  being  unable  to  counteract 
the  flexors.  Marked  or  complete  paralysis  of  the  extensors  causes 
wrist-drop. 

Fle.rors  of  the  Fingers. — Because  of  the  usual  difference  in  the  strength 
of  the  two  hands  the  examiner  should  cross  his  forearms  and  place  his 
right  hand  in  the  right  hand  of  the  patient,  and  vice  versa.  Then  let 
the  patient  squeeze  the  hands.  If  the  observer  keeps  his  own  fingers 
extended  and  bunched  loosely  together  he  will  be  able  to  withstand  a 
very  hearty  grasp  without  discomfort. 


UPPER  EXTREMITIES 


81 


Adductor  Pollicis. — ^Ask  the  patient  to  pinch  with  his  thumb  and 
finger  one  of  the  examiner's  fingers. 

Opponens  Pollicis. — Desire  the  patient  to  approximate  the  ends 
of  the  Httle  finger  and  the  thumb — while  thus  approximated  the 
examiner  pulls  his  finger  through.  Ability  to  do  so  easily  shows 
weakness  of  the  opponens. 

The  interossei  and  lumhricales  muscles  of  the  hand  flex  the  proximal 
phalanges,  and  extend  the  middle  and  terminal  phalanges.  The  dorsal 
interossei  abduct,  the  palmar  adduct,  the  fingers  from  and  toward 
a  longitudinal  line  drawn  through  the  center  of  the  middle  finger. 
Test  by  making  the  patient  separate  and  approximate  the  fingers, 
and  flex  the  proximal  phalanges,  keeping  the  middle  and  terminal 
phalanges  extended.    Paralysis  of  these  muscles  causes  "claw-hand." 

The  strength  of  the  hand  grasp  is  well  tested  by  the  dynamometer. 
Readings  of  three  tests  for  each  hand  should  be  recorded.  Dyna- 
mometer records  are  available  for  definite  comparisons.  Further,  the 
dynamometer  is  useful  by  many  repeated  readings  (20)  for  obtaining 
an  idea  of  the  fatigability  of  muscles  (neurasthenia,  myasthenia, 
alterations  in  attention,  etc.).  Weiler  has  constructed  a  useful  dyna- 
mometer with  a  graphic  register.  Tilney  has  also  devised  a  useful 
instrument.  Expressed  in  tabular  form  the  muscles,  their  action, 
nerve  supply,  and  spinal  synapses  are  as  follows: 

Muscles  of  Shoulder  Girdle,  Actions  and  Spinal  Synapse  Segments. 


Muscle. 


Trapezius. 


Latissimus  dorsi. 
Levator  scapulae. 

Rhomboidei. 

Pectoralis  major  and  minor. 

Subclavius. 

Serratus  magnus. 


Action. 
These     muscles     move     the 
shoulder  girdle: 

(a)  Vertical  plane.     Eleoa- 

lion. 
Trapezius — upper 

fibers. 
Le\'ator  scapulse. 


Nerve  Supply  and 
Spinal  Segment, 
Spinal    accessory,    cervical 
plexus,    medulla   N,  am- 
biguus  Cl-5,  C3-4. 


Long  subscapular,  C6-8. 

Cervical    plexus,    posterior 
scapular,  C3-5. 


Rhomboidei. 

Sternohyoid. 

Omohyoid. 
Depression. 
Trapezius  (lower  fibers). 
Subclavius. 
Pectoralis  minor. 
Latissimus  dorsi. 
Pectoralis    major    (lower     Brachial  plexus,  C5-6 

fibers) . 


Posterior  scapular,  C5. 

External  and  internal  ante- 
rior thoracic,  C5-7,  C8,  Dl. 


(6)   Horizontal  plane. 
Forward: 

Serratus  magnus. 
Pectoralis  major 
Pectoralis  minor. 
Backward: 
Trapezius. 
Rhomboidei. 
Lati.ssimus  dorsi. 


Posterior  thoracic,  C5-7. 


82      METHODS  OF   EXAMINATION  OF   THE  NERVOUS  SYSTEM 


TetniJuruU 


Complexus  Cl-8 

Si>lenius  f  "«»f }  Ca^p 
of  neck  i 
Rectus  capitis  anticus- 
major  CI 
Levator  anguli  scap  c:i-5 
Scalenus  medius  C'J-S 
Scalenus  posticus  C5-8~- 
Scalenus  anticus  d-l 
Rhomboideu 
Ci-5  B.F. 


Infraspinatus  Co-G- 
Teres  minor  Co  - 
Teres  major^ 

C(5)6(7) 

Latissimus  dorsi 
Brachialis  anticus 
Triceps 
Biceps 


Brauhio  radialis 
Ext.  carpirad.  brev.— 
Anconeus  _Xt7^:^) 

Ext.  carpirad.  long.  CO (7)8 

Ext.  carpi  ulnaris 
Ext.  comm.  diaitoruv, 


Flex.  long,  poliicis — 

Ext.  brev.  poliicis 

OGJ 
Gluteus  maximus 


Abducens  poliicis 

ca,7 

1  Dorsal  interossei 
C8  (Dl) 
1  Lumbricales 
C7-8(Z)2) 

Biceps 


Stylohyoid  {VII,  IX) 


Masse  ter 

Digastric  (VII,  IX) 
StuloliyoidWlI,  IX) 
Ml/lohtioidW) 
if.  fonxtrictor  of  pharynx 
■^Tli  yroh  uoid  Cl-2         -|  iJX,  X,  XI) 
ohi/oid  Cl-3       I 
Sterimhuoid   Cl-3     [ 
Sternothyroid  Cl-i} 
Lont/uscolli   C2-8 


Pectoi-alis  major 


-Serratus  magnus 
Suj).  Intercostals 
D5,6,7 
Inf.  Intercostals 


-Rectus  abdominis 

Transversalis 
jGluteus  minimus 


Sartorius  Li,  3 
— Obturator  ext  L3,i 

—Rectus  femoris 
Adductor  poliicis 

C8{D1) 
Vastus  externus 


Fig.  29. — Radicular  innervation  of  the  lateral  aspect  of  head,  neck,  trunk,  and  upper 
extremity.  The  platysma  myoides,  sternocleidomastoid,  trapezius,  and  oblique  muscles 
of  the  abdomen,  gluteus  medius,  and  tensor  of  the  fascia  lata  have  been  removed.  The 
shading  and  cross-hatching  follow  the  muscles  and  innervation  as  in  Figs.  27  and  28. 
(After  Dejerine.) 


UPPER  EXTREMITIES 


83 


Injeriur  iiiaxUlary  I 


Poxt.  brunches 
cervical  nerves  C2-8p 
A'.  liectus  an  tic  us 
and  longus  collis 
C.P. 
N.  Scaleni  C.P. 
N.  levat.  anauli  scaj). 
Cervii-al  i)lexus 
N.  ritoiitb.and 
lev.  any.  sea}). 
B.P.  Ci-i 

N.  subscavulari 
Uracil  ial  plejcus 
Circumflex  B.P. 

Circumflex 
Brach  ial  plexus 


Lower  aubscapulaf' 
B.P. 

Long  subscapular 
Braciiial  ptexus 


Musculo  cutaneous 
rail  ial 


Musculo  cutaneous 
Brach  ial  plexus 


Radial 

Inferior 
gluteal 
Sacral  plexus 

Median. 


Inferior  majnllary  V 

Stylohyoid  j  VU 

Posterior  digastric  ( IX 

Inferior  dental  V 
Pharyngeal  IX,  X,  XI 
Longus  colli  C.P. 

Int.  desa.  branch  C.P.  Ci,3 
and  Hyjjoglossus  XII 

Anterior 
thoracic 
\Brach  ial  plexus 


Post. thoracic 
Brach  ial  plexus 


Ulnar 
Ulnar 


Sciatic 
Sacral  plexus 


:lnf.  thoracic  int. 


Intercostal  and 
Hypogastric  J-. 


Intercostal,  genito 
crural  and 
hypogastric. 
Lum  bar  plexus 


Superior 

gluteal 

bacral  plexus 

Crural 
Lumbar  plexus 
^bturator 
Lumbar  plexus 


Crural 
Lumbar  plexxis 


Fig.  30. — Peripheral  innervation  of  the  lateral  aspect  of  the  head,  neck,  trunk,  and 
upper  extremity.  Markings  and  cross-hatchings,  as  in  previous  figure,  to  indicate  the 
various  innervations.  (After  Dejerine.)  Letters  C,  D.,  L.,  C.P.,  B.P.,  L.P.,  and  S.P. 
aa  in  previous  diagrams. 


84     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 


Muscles  of  Shoulder-joint,  Actions  and  Spinal  Synapse  Segments. 


Deltoid. 
Teres  minor. 

Supraspinatus. 
Infraspinatus. 


Teres  major. 
Teres  major. 


Abduction: 

Deltoid. 

Supraspinatus. 
Adduction: 

Teres  major  and  minor. 
Pectoralis  major. 

Latissimus  dorsi. 

Coracobrachialis. 

Biceps. 

Triceps. 
Flexion  (forward): 

Deltoid  (anterior). 

Subcapularis. 

Pectoralis  major. 

Coracobrachialis. 

Biceps. 


Circumflex,  C5-6. 
Suprascapular,  C5-6. 

Lower  subscapular,  C5-6. 
Lower  subscapular,  C5-G. 


Muscles  of  Shoulder-joint,  Actions  and  Spinal  Synapse  Segments  (continued). 

Nerve  Supply  and 
Muscle.  Action.  Spinal  Segment. 


Subscapiilaris. 


Muscle. 
Coracobrachialis. 
Biceps. 
Brachialis  anticus. 

Brachialis  anticus. 
Triceps. 

Outer  head. 

Middle  and  inner 


Extension  (backward) : 

Deltoid  (posterior). 

Teres  major. 

Infraspinatus. 

Latissimus  dorsi. 

Triceps. 
Rotation  (out). 

Deltoid  (posterior). 

Infraspinatus. 

Teres  minor. 
Rotation  (in). 

Deltoid  (anterior). 

Teres  major. 

Pectoralis  major. 

Latissimus  dorsi. 

Action. 


Lower  and  upper  subscapu- 
lar, C5-6. 


Nerve  Supply  and 
Spinal  Synapses. 


Musculocutaneous, 
Musculocutaneous, 
Musculocutaneous, 


head. 


Musculospiral,  C5- 
Museulospiral,  C6- 
Musculospiral,  C7- 


Flexors: 

Biceps. 

Brachioradialis. 

Pronator  radii  teres. 

Flexor  profundus  dig. 

Flexor  longus  pollicis. 

Flexor  sublimus  dig. 

Extensor  of  wrist  in 
pronation. 
Extensors: 

Triceps. 

Anconeus. 

Extensors  of  wrist  and  fingers  (in  supination) 


Arm  Muscles  and  Spinal  Synapse  Segments. 


C7. 

C5-6. 

C5-6. 


Posterior  muscles  of  the  forearm: 
Brachioradialis  (supin.  rad.  long.). 
Extensor  carpi  radialis  longior. 
Extensor  carpi  radialis  brevior. 
Extensor  communis  digitomm. 
Extensor  minimi  digiti. 
Extensor  carpi  ulnaris. 
.\nconeus. 

Supinator  radii  brevis. 
Extensor  ossis  metacarpi  pollicis. 
Extensor  brevis  pollicis. 
Extensor  longus  pollicis. 
Extensor  indicis. 


Musculospiral,  C5-6. 

Posterior  interosseus  C6- 
( Musculospiral.) 

Musculospiral,  C6-8. 
Posterior  interosseus,  C6- 


UPPER  EXTREMITIES 


85 


Median  nerve,  C6. 


}-' 


Ulnar,  C8,  Dl. 

Ulnar  and  anterior  interosseus,  C7-8,  Dl. 

Anterior  interosseus,  C7-8,  Dl. 


Anterior  muscles  of  the  forearm: 

Pronator  radii  teres. 

Flexor  carpi  radialis. 

Palmaris  longus. 

Flexor  sublimis  digitorum. 

Flexor  carpi  ulnaris. 

Flexor  profundus  digitorum. 

Flexor  longus  poUicis. 

Pronator  quadratus. 
Muscles  of  the  hand: 

Abductor  pollicis. 

Opponens  pollicis. 

Flexor  breads  pollicis  (superf.). 

Flexor  brevis  pollicis  (deep). 

Adductor  obliquus  pollicis. 

Adductor  transversus  pollicis. 

Lumbricales,  1  and  2. 

Lumbricales,  3  and  4. 

Interossei. 

Flexor  brevis  minimi  digiti. 

Abductor  minimi  digiti.  J 

The  actions  of  these  groups  are  multiform  and  are  best  considered 
separately.  Single  muscles  are  not  capable  of  being  isolated  from  the 
group  actions,  as  a  rule.  The  elbow-joint  action  has  been  given. 
Actions  of  pronation  and  supination  are  important. 


Median,  C6-7. 


\  Ulnar,  C8,  Dl. 


J 


Median,  CG-7. 


IHnar,  C8,  Dl. 


Pronation: 

Pronator  radii  teres. 
Pronator  quadratus. 
Brachioradialis. 
Flexor  carpi  radialis. 


Flexion: 

Flexor  carpi  radialis. 

Palmaris  longus. 

Long  flexors  of  thumb  and  fingers. 
Adduction: 

Flexor  carpi  ulnaris. 

Extensor  carpi  ulnaris. 


Supination: 

Supinator  radii  bre\ns. 

Biceps. 

Brachioradialis. 

Extensors  of  thumb  and  fingers. 
Action  at  the  Wrist-joint. 
Extension: 

Extensors  of  wrist. 

Extensors  of  thumb. 

Extensors  of  fingers. 
Abduction: 

Flexor  carpi  radialis. 

Extensors  of  wrist. 

Extensors  of  thumb. 


Finger 
Flexion: 

Flexor  sublimis  digitorum. 

Flexor  profundus  digitorum. 

Lumbricales.lOn  metacarpophalangeal 

Interossei.      /     joints. 

Flexor  brevis  minimi  digiti. 
Add^iction: 

Palmar  interossei  (to  the  middle  line  of 
middle  finger). 


Thumb 
Flexion: 

Opponens  pollicis  (carpometacarpal). 

Flexor  brevis  pollicis.]  Carpometacarpal 
Adductor  pollicis.  \  and  metacar- 
Abductor  pollicis.        J  pophalangeal. 

Flexor  longus  pollicis  (all  joints). 
Addxiction: 

Adductors  of  the  thumb. 
Flexor  brevis  pollicis. 
Opponens  pollicis. 
Interossei  (1  dorsal). 


Action. 

Extension: 

Extensor  communis  digitorvim. 
Extensor  indicis. 
Extensor  minimi  digiti. 
Lumbricales. 1  Acting  on  interphalangenl 
Interossei.     /     joints. 
Abduction: 
Lumbricales. 
Flexor  brevis  and  Opponens  minimi  digiti 

(from  inner  side  of  hand). 
Dorsal   interossei   (from   middle  line  of 
middle  finger). 
Action. 
Extension: 

Extensor  ossis  metacarpi  pollicis  (carpo- 
metacarpal). 
Extensor  brevis  pollicis  (carpometacarpal 
and  metacarpophalangeal). 

Extensor  longus  pollicis  (all  joints). 
Abduction: 
Abductor  pollicis. 
Extensors  of  thumb. 


86     METHODS  OF  EXAMINATION   OF   THE  NERVOUS  SYSTEM 


Siiprrioi-  gluteal 


Gliitcjif!  mcdi 


Gluteus  maximus 


Tensor  of 
fascia  lata  ~ 


Vastus  externus. 


Crural  N. 
Lumbar  2>lexw 


Crural  N. 
Lumbar  plex'Ui 


Crural  N. 
Lumbar  iilexus 


I 


Oastroenemiiis  -, 


Peroneus  longus 


Peroneus  brefis- 


Peroneus  tertius 
Ext.  brev.  cHgit.^ 

Abductor  minimi 
digiti 


Fig.  Zl.— Radicular  (R)  (to  left)  and  peripheral  (P)  (to  right)  innervations  of  the 
muscles  of  the  external  side  of  the  lower  extremity.  Letters  and  abbreviations  as  in 
preceding  figures.     (After  Dejerine.) 


THE  LOWER  EXTREMITIES 


87 


Pl/i-aiiii 
(lalis  X.  S.P: 
Obturator 
Internus  S.P. 
i.sc/iiococci/!/eiijS 
Genital  Plexus 

Rectum  and  Anus  G.P.- 
Hemorrhoidal  pi.  G 
Vagina  G.P-. 

Obturator. 


Lumbar  iHexus 


LU)5,S1[2) 

Obturator  int. 
ramidalis 
7-.c?i  lococcuoeus 
Levator  ani  S3-i 

']>hinvtcr  ani 
S3-i 


Ibch  io-cavemosus\^, 
Bulbo-cavernosus  i 


Gluteus  maximus 
Adductor  maanus 
Adductor  lonyus 

i>enii  tendinosus 
~^eini  rrembranosus 
Biceps    LU)3,S1 
Giacilis 


~Sai  tortuu 


astrocnemius 


Sole  us 

Flex.  long.  dig. 

Lo,  Sl-i 

Tibialis  posticus 

L3,  Sir>) 

Flex.  long.  hall. 
LJ.  S1--J 


Fir..  32. — Radicular  (R)  (to  right)  and  peripheral  (P)  (to  left)  innervations  of  tlie 
muscles  of  the  internal  side  of  the  lower  extremities.  (After  Dcjerine.)  G.P.,  genital 
plexus. 


88     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

The  Muscles  of  the  Trunk. — The  erector  muscles  of  the  spine  are 
examined  by  causing  the  patient  to  He  face  downward  and  asking  him 
to  raise  the  head  and  shoulders  without  assistance  from  the  hands. 
Unless  paralyzed  the  erectors  become  clearly  visible  during  the  attempt. 
The  abdominal  muscles  are  tested  in  a  similar  manner,  except  that  the 
patient  lies  on  the  back  while  making  an  effort  to  raise  the  head. 


Muscles  of  the  Thigh  and  Buitock  and  Spinal  Synapse  Segments. 


Pectineus. 

Sartorius. 

Iliacus. 

Psoas. 

Quadriceps  extensor. 

Vastus  externus. 

Rectus  femoris. 

Crureus. 

Vastus  internus. 
Tensor  fascise  femoris. 
Gluteus  minimus. 
Gluteus  medius. 
Gluteus  maximus. 
Biceps  (s.  h.). 
Pyriformis. 
Adductor  longus. 
Gracilis. 

Adductor  brevis. 
Obturator  externus. 
Adductor  magnus. 
Semimembranosus. 
Semitendinosus. 
Biceps  (1.  h.). 

Quadratus  femoris 

Gemellus  inferior. 

Gemellus  superior. 

Obturator  internus. 


L2  3. 
L2  3. 
}  L2-4. 
Anterior  crural, 
L3-4. 


L2-4. 


Sacral  plexus. 


Superior  gluteal,  L4-5  SI. 

Inferior  gluteal,  L5,  SI— 2. 

Peroneal,  L5,  Sl-2. 

Sacral  plexus,  Sl-2. 

L2  3. 

Obturator,  L2-4. 

L3-4. 

L3-4. 

Obturator,  L3-4. 

L4-5,  SI. 

Sciatic,  L4-5,  SI. 

L5,  Sl-2. 

L4  5,  SI. 


Sl-3. 


Most  of  these  muscles  act  upon  the  pelvis  and  on  the  hips  and 
knee-joints. 

Hip-joint  Movements. 


Flexion. 
Sartorius. 
Iliacus. 
Psoas. 

Rectus  femoris. 
Pectineus. 
Adductor  longus. 
Gracilis. 
Obturator  externus. 

Adduction: 
Pectineus. 
Adductor  longus. 
Adductor  brevis. 
Adductor  magnus. 
Gracilis. 

Quadratus  femoris. 
Gluteus  maximus  (lower  fibers). 


Extension: 

Gluteus  maximus. 
Gluteus  medius. 
Gluteus  minimus. 
Biceps. 

Semitendinosis. 
Semimembranosis. 
Adductor  magnus. 

A  bduction: 

Tensor  fascise  femoris 
Gluteus  medius. 
Obturator  externus. 
Pyriformis. 
Obturator  internus 
Gemelli. 
Sartorius. 
Gluteus   maximus 
(upper  fibers). 


During  flexion 


THE  LOWER  EXTREMITIES 


89 


Internal  rotation: 

Tensor  fasciae  femoris. 
Gluteus  medius  (anterior). 
Gluteus  minimus  (anterior). 


yPosterior. 


External  rotation: 
Obturator  externus. 
Gluteus  maximus  (lower). 
Quadratus  femoris. 
Gluteus  medius. 
Gluteus  minimus.] 
PjTiformis. 

Obturator  internus.  \  During  extension. 
Gemelli. 
Sartorius. 
Iliopsoas. 
Pectineus. 
Adductors. 
Biceps  flexor  cruris. 


Knee-joint  Movement?. 
Extension: 

Quadriceps  extensor. 


External  rotation: 
Biceps  flexor  cruris. 


Flexion: 
Sartorius. 
Gracilis. 
Semitendinosus. 
Semimembranosus. 
Biceps. 

Gastrocnemius. 
Plantaris. 
Popliteus. 

Internal  rotation: 
Sartorius. 
Gracilis. 
Semitendinosus . 
Semimembranosus. 
Popliteus. 


The  Lower  Extremities. —  The  vmscles  of  the  lower  extremities  are  for 
the  most  part  best  tested  with  the  patient  hing  down  (see  Figs.  27 
to  32): 

Flexors  of  the  Thigh. — The  patient  lying  upon  his  back,  ask  him 
to  raise  the  leg  from  the  bed,  against  resistance,  the  knee  being  kept 
straight.  This  determines  the  strength  mainly  of  the  iliopsoas,  partly 
of  the  quadriceps. 

Extensors  of  Thigh.— The  leg  being  kept  straight  and  the  patient 
lying  upon  his  back,  raise  the  foot  and  ask  him  to  bring  it  down 
upon  the  bed  against  resistance.  This  determines  the  strength  of  the 
gluteus  maximus  and  partly  of  the  hamstring  muscles. 

Abductors  of  Thigh. — "With  the  leg  across  the  middle  line  ask  the 
patient  to  carry  it  toward  the  outer  side  against  resistance,  thus  testing 
mainly  the  gluteus  medius. 

Inrotators  of  Thigh. — ^Yith  the  patient  on  his  back,  flex  the  knee  to  a 
right  angle,  grasp  the  foot,  and  oppose  resistance  while  he  inrotates 
the  thigh,  testing  mainly  the  gluteus  minimus. 

Oiitrotators  of  the  Thigh. — Similarly  test  the  power  of  outrotation, 
thus  determining  the  condition  of  the  obturators,  pyTiformis,  gemelli, 
and  quadratus  femoris. 

Flexors  of  the  Knee. — The  patient  lying  upon  his  back,  desire  him 
to  bend  the  knee  while  the  examiner  resists  the  movement  by  grasping 
the  ankle,  thus  ascertaining  the  power  of  the  biceps,  semimembranosus, 
and  semitendinosus. 


90     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

Extensors  of  the  Knee. — With  the  patient  on  the  back,  flex  the  knee, 
and  by  pressure  on  the  sole  of  the  foot  resist  his  endeavor  to  extend  the 
knee.    The  quadriceps  femoris  is  the  principal  muscle  concerned. 

Plantar  Flexors  (Extensors)  of  the  Foot. — With  the  leg  straight 
resist,  by  pressure  upon  the  sole  of  the  foot,  the  patient's  endeavor 
to  bring  the  tarsus  in  a  line  with  the  leg,  thus  testing  the  gastroc- 
nemius, soleus,  peroneus  longus  and  brevis.  Have  patient  stand 
on  toes. 

Dorsifledors  of  the  Foot. — With  the  leg  straight,  resist  the  patient's 
attempt  of  dorsal  flexion  of  the  foot,  thus  testing  the  tibialis  anticus 
and  the  peroneus  tertius.  Marked  paralysis  of  these  muscles  causes 
"foot-drop."    Have  patient  stand  on  the  heels. 

Muscles  of  the  Foot. — The  flexors,  extensors,  interossei,  and  lumbri- 
cales  of  the  toes  are  examined  in  a  similar  manner  to  those  of  the 
fingers.    There  is  a  sort  of  claw-foot  analogous  to  the  claw-hand. 


Muscles  of  the  Leg  and  Foot  with  Nerve  Supply  and  Spinal  Synapse 

Segments. 


Muscle. 
Tibialis  anticus. 
Extensor  proprius  pollicis. 
Extensor  longus  digitorum. 
Peroneus  tertius. 
Peroneus  longus. 
Peroneus  brevis. 
Extensor  brevis  digitorum. 
Plantaris. 
Popliteus. 
Gastrocnemius. 
Soleus. 

Flexor  longus  digitorum. 
Tibialis  posticus. 
Flexor  longus  hallucis. 
Abductor  hallucis. 
Flexor  brevis  digitorum. 
Flexor  brevis  hallucis. 
First  lumbricales. 
Second,  third,  fourth  lumbricales. 
Flexor  accessorius. 
Adductors  hallucis. 
Interossei. 

Flexor  brevis  minimi  digiti. 
Abductor  minimi  digiti. 


Nerve  Supply  and  Spinal  Segment. 
Anterior  tibial. 

L4-5,  SI. 


Musculocutaneous. 
Anterior  tibial. 


Tibial. 


Posterior  tibial. 


L4-5,  SI. 

Sl-2. 

L5,  SI. 
L5,  SI. 
L5,  Sl-2. 


Internal  plantar,  L4-5,  SI. 


I-  External  plantar,  Sl-2. 


J 


Movement  of  the  Ankle-joint. 


Flexion: 

Tibialis  anticus. 
Extensor  communis  digitorum. 
Extensor  proprius  pollicis. 
Peroneus  tertius. 


Inversion: 

Tibialis  anticus. 
Tibialis  posticus. 


Extension: 

Gastrocnemius. 

Plantaris. 

Soleus. 

Tibialis  posticus. 

Peroneus  longus. 

Peroneus  brevis. 

Flexor  longus  digitorum. 

Flexor  longus  hallucis. 

Eversion: 

Peroneus  tertius. 
Peroneus  longus. 
Peroneus  brevis. 


REFLEXES  OF   UPPER  EXTREMITIES  91 

Movements  of  the  Toes  at  the  Metatarsophalangeal  Joints. 
Flexion:  Extension: 

Flexor  longus  digitoruni.  Extensor  longus  digitorum. 

Accessorius.  Extensor  brevis  digitorum. 

Lumbricales.  Extensor  proprius  hallucis.   ' 

Flexor  longus  hallucis. 
Flexor  bre\'is  hallucis. 
Flexor  brevis  digitorum. 
Flexcr  brevis  minimi  digiti. 
Interossei. 

Movements  of  the  Toes  at  the  Metatarsophalangeal  Joints  (continued). 
Abduction.  Adduction: 

Abductor  hallucis.  Adductors  hallucis. 

Dorsal  interossei.  Plantar  interossei. 

Abductor  minimi  digiti. 

Movements  of  the  Toes  at  the  Interphalangeal  Joints. 
Flexion:  Extension: 

Flexor  brevis  digitorum.  Extensor  longus  digitorum. 

Flexor  longus  digitorum.  Extensor  bre\ris  digitorum. 

Flexor  longus  hallucis.  Interossei. 

Lumbricales. 

Extensor  proprius  hallucis. 

Reflexes  of  Upper  Extremities.— Superficial  and  Deep. — These  are  then 
taken  up.  Those  of  the  cranial  nerves  have  been  considered.  The 
important  reflexes  of  the  upper  extremities  are: 

Elbow  or  Triceps  Jerk. — This  is  best  tested  by  supporting  the 
patient's  arm  at  the  elbow  by  allowing  the  forearm  to  hang  flaccidly 
over  the  edge  of  a  chair.  The  stroke  is  made  just  above  the  olec- 
ranon, and  the  reaction  consists  in  an  extension  of  the  forearm  due 
to  the  contraction  of  the  triceps  muscle. 

Radius  Periosteal  Reflex. — The  radius  periosteal  reflex  consists  in  a 
slight  flexion  of  the  arm  on  the  forearm  when  the  radius  is  tapped  just 
three  or  four  inches  above  the  external  condyle. 

Supinator  Jerk. — The  supinator  jerk  is  obtained  by  striking  the 
muscle  about  midway  between  the  elbow  and  wrist,  the  arm  being 
supported  at  the  wrist.  It  consists  in  a  slight  extension  of  the  pendant 
wrist. 


Reflex. 

Method  of  eliciting. 

Response. 

Segment. 

Biceps. 

Tap  biceps  tendon. 

Biceps  contracts. 

C5  and  C6. 

Triceps. 

Tap  triceps  tendon. 

Triceps  contracts. 

C5,  6  and  7, 

Supinator  longus. 

Tap  radial  styloid. 

Supinator  longus  con- 
tracts. 

C5  and  C6. 

Wrist. 

Tap  flexor  tendons  at  wTist. 

Fingers  are  flexed. 

C6  to  C8. 

Carpometacarpal.        Tap  back  of  wrist.  Fingers  are  extended.     C6  to  Dl. 

Jacobsohn's  radius  reflex  consists  in  a  slight  flexion  of  the  fingers, 
particularly  of  the  terminal  phalanges  when  the  radius  of  the  extended 
outstretched  hand  supported  by  the  observer's  hand  is  suddenly 
tapped  with  a  hammer. 

The  superficial  reflexes  of  the  trunk  should  next  be  tested.  Both 
sides  should  always  be  tested  and  recorded  O  if  absent,  +  if  present; 
R  =  L  or  R  >  L,  R  >  L,  or  R,  o,  L+,  or  vice  versa,  as  the  case  may  be. 


92       METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

Epigastric.  Stroke      downward      from     Epigastrium  dimples.     D7  to  D9. 

nipple. 
Abdominal.  Stroke    down    from    costal     Abdominal       muscles     Dll  to  L2. 

margin.  contract. 

Cremasteric.  Stroke  inner  side  of  thigh.     Testicle  is  pulled  up.     LI  to  L2. 

Gluteal.  Stroke  skin  over  buttocks.     Gluteal   muscles   con-     L4  to  L5. 

tract. 
Bulbocavernosus.         Pinch  dorsum  of  glans  penis     Compressor     urethra;     S3  to  S4. 

contract. 
Superficial  anal.  Prick   skin  of   perineum.  External        sphincter     SSandconus. 

contracts. 

Reflexes  of  the  Lower  Extremities. — The  knee-jerk  (Erb-Westphal's 
sign)  is  one  of  the  most  familiar.  The  knee-jerk  may  be  tested  in  a 
variety  of  ways.  One  of  the  best  is  to  have  the  patient  sit  upon  a 
table,  which  permits  the  limbs  to  hang  freely,  then  tell  him  to  look 
at  the  ceiling,  or  divert  his  attention,  the  tendon  just  below  the  patellar 
is  tapped,  or  the  patient  is  directed  to  cross  one  leg  over  the  knee  of 
the  other  leg  and  the  tendon  is  struck  in  a  similar  manner.  Exagger- 
ated, active,  normal,  sluggish,  or  absent  responses  should  be  recorded. 
Another  method  is  to  have  the  patient  sitting,  and  the  .heels  upon  the 
floor  but  the  legs  comfortably  extended.  The  tendon  is  then  tapped. 
Here  a  simultaneous  tapping  of  both  tendons  may  be  tried. 

In  certain  patients  the  attention  must  be  diverted,  otherwise  the 
leg  is  held  rigidly  which  destroys  the  reflex.  The  patient  may  be 
directed  to  repeat  the  Lord's  Prayer,  or  compute  small  sums  in  arith- 
metic, or  converse  with  an  assistant,  thus  diverting  the  attention 
from  the  testing.  Jendrassik  thought  of  the  expedient  of  utilizing  a 
forced  muscular  act  in  the  upper  extremity  to  reinforce  the  knee-jerk. 
This  may  be  carried  out  by  having  the  patient  make  hard  fists  at  a 
given  signal,  when  the  tendon  is  tapped,  or  by  having  him  grasp  his 
hands  and  pull  at  the  given  signal.  By  reinforcement  a  very  weak 
knee-jerk  may  be  made  very  evident. 

Achilles  Jerk. — This  is  best  tested  by  having  the  patient  kneel  upon 
a  chair,  the  foot  being  just  free  of  the  edge.  The  Achilles  tendon  is 
then  tapped,  and  there  results  pulling  up  of  the  heel. 

For  patients  in  bed,  the  leg  should  be  everted,  slightly  flexed,  and 
the  foot  extended  to  put  the  tendon  on  slight  tension.  One  person 
in  a  hundred  has  lost  the  Achilles  or  knee-jerk. 

Ankle-clonvs . — To  elicit  ankle-clonus  requires  some  care.  It  is  best 
obtained  by  supporting  the  patient's  leg  along  the  under  side,  the 
patient  cooperating  by  thorough  relaxation,  then  the  free  hand  grasps 
the  foot,  and  makes  a  sudden  upward,  dorsal  flexion,  holding  the 
foot  fairly  firmly  flexed  at  the  end  of  the  movement  when  a  series 
of  clonic  extensions  and  flexions  take  place.  The  leg  should  be  slightly 
everted,  and  the  knee  somewhat  flexed.  A  false  clonus  consists  in  half 
a  dozen  flexions  and  extensions;  true  clonus  continues  for  some  time. 

Plantar  Reflex. — By  stroking  the  sole  of  the  foot,  either  at  its  external 
or  internal  border,  a  quick  plantar  flexion  of  all  the  toes  including  the 
great  toe  takes  place.  This  is  normal  plantar  flexion.  As  many 
individuals  are  ticklish,  there  is  frequently  a  sudden  jerking  of  the 


REFLEXES  OF   THE  LOWER  EXTREMITIES  93 

whole  foot,  or  such  a  protective  movement  is  manifested  only  in  the 
tendons  of  the  great  toe.  This  should  be  distinguished  from  true  dorsal 
extension  or  the  Babinski  reflex}  This  consists  in  the  comparatively 
slow  dorsal  extension  of  the  great  toe  when  the  plantar  reflex  is  tested 
and  at  the  same  time  there  is  a  slight  spreading  apart  of  the  other  toes. 


Fig.  33. — Extension  of  the  great  toe  on  irritating  the  sole.    The  Babinski  plantar 
extension  phenomenon.     (Chaddock.) 

If  the  patient  is  in  bed — as  he  should  be  for  testing — a  fully  developed 
Babinski  sign  consists  in  the  slow  dorsal  raising  of  the  great  toe,  the 
spreading  of  the  other  toes,  a  slight  rotation  of  the  thigh  on  the  hip 


Fig.   34. — The  Chaddock  modification  of  the  Babinski,  causing  great  toe  extension  on 
stroking  l)eneath  the  external  malleolus.     (Chaddock.) 

and  a  contraction  of  the  fascia  lata  of  the  thigh.     In  order  to  develop 
the  whole  test  the  feet  should  be  warm,  the  thigh  slightly  rotated 

'  By  the  term  dorsal  extension  is  here  meant  rising  of  the  toes.  By  Babinski  the 
term  plantar  extension  was  used.  We  are  using  the  words  in  the  ordinary  sense,  dis- 
regarding the  fact  that  by  some  plantar  extension  is  made  synonymous  with  dorsal 
flexion,  and  plantar  flexion  with  dorsal  extension. 


94     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

externally,  the  knee  slightly  bent,  and  the  stroke  made  either  on  the 
outer  or  inner  border  of  the  sole  by  either  a  fairly  sharp  instrument, 
the  finger-nail,  or  a  blunt-pointed  instrument.  As  there  is  great  varia- 
tion in  the  thickness  of  the  skin  of  the  soles  of  the  feet,  the  various  ways 
of  bringing  out  a  Babinski  phenomenon  should  be  tried  in  each  case. 
Particular  attention  should  be  directed  to  the  dangers  of  confusing 
the  protective,  pulling-away  motion  on  tickling,  which  causes  a  marked 
quick  dorsal  raising  of  the  great  toe,  with  a  true  Babinski  phenomenon. 

Careful  record  should  be  mad'e  of  the  irregularities  in  plantar 
response.  In  some  patients  there  will  be  planter  flexion  of  the  small 
toes  but  no  reaction  of  the  big  toe.  At  times  this  may  be  as  significant 
as  a  true  dorsal  extension.  It  is  to  be  borne  in  mind  that  dorsal  raising 
of  the  great  toe  is  normal  in  infants  and  children  up  to  the  age  of 
successful  walking.  A  number  of  closely  related  reflexes  have  been 
described,  having  much  the  same  significance  as  the  Babinski  sign,  but 
being  less  constant,  and  at  times  contradictory.    These  are: 

Strilmpell  Reflex. — This  follows  forceful  pressure  over  the  anterior 
tibial  region  with  a  resultant  dorsal  extension  of  the  great  toe.  It  is 
found  in  a  number  of  conditions  other  than  those  of  functional  dis- 
turbance af  the  pyramidal  tracts. 

Oppenheim  Reflex. — Here  the  inner  surface  of  the  leg  is  sharply  and 
deeply  stroked  by  the  thumb  from  the  middle  to  the  ankle  behind  the 
malleolus.    It  brings  out  a  great  toe  dorsal  extension. 

The  Paradoxical  Reflex. — Called  by  a  variety  of  names,  as  described 
by  Gordon,  it  consists  in  a  dorsal  exension  of  the  great  toe  following 
the  grasping  of  the  deep  muscles  of  the  calf  and  making  a  forceful 
indention  along  their  external  border. 

Mendel-  Bechterew. — This  reflex  consists  of  the  dorsal  extension  of 
the  toes,  especially  the  second  and  fifth,  when  the  dorsum  of  the  foot 
is  tapped  about  at  the  base  of  the  middle  toes.  In  reflex  irritability 
this  reflex  is  augmented.  If  plantar  flexion  takes  place  the  authors 
regard  it  as  a  sign  of  organic  aft'ection.^ 

Chaddock. — This  produces  a  toe  extension  by  stroking  the  side  of  the 
ankle  (Fig.  34). 


Reflex. 

Method  of  obtaining. 

Result. 

Location. 

Knee. 

Tap  patellar  tendon. 

Leg  extended. 

L3  and  L4. 

Achilles. 

Tap  tendo  Achillis. 

Plantar  flexion  foot. 

S,  S4. 

Ankle-clonus. 

Sudden  dorsiflexion  foot. 

Quick    up-and-down 

Pyramidal  tracts 

movements. 

(L3,  S2). 

Plantar. 

Stroke  soles  of  feet. 

Plantar  flexion  all  toes. 

L3,  S2. 

Babinski. 

Stroke  soles  of  feet. 

Dorsal  extension  great 

PjTamidal  tracts 

toe. 

(L3,  S2). 

Oppenheim. 

Stroke  inner  side  of  calf. 

Dorsal  extension  great 

Pyramidal  tracts 

toe. 

(L3,  S2). 

Paradoxical. 

Deep  pressure  in  calf. 

.  Dorsal  extension  great 

Pyramidal  tracts 

toe. 

(L3,  S2).2 

'  Krug:  Leipzig  Dissertation,  1911. 

*  For  a  complete  discussion  of  reflex  action  see  Sherrington:  .Jour.  Physiol.,  1910,  vol. 
iv.  The  complicated  problem  of  medullary  reflexes  is  amplified  in  a  thesis  of  A.  Stahl, 
Paris,  1913. 


TYPES  OF   VEGETAriVE  REFLEXES  95 

Types  of  Vegetative  Reflexes. — The  physiological  relation  between 
vegetative  reflexes  and  sensations  of  pain  and  emotions  deserves  a 
closer  examination. 

In  man  the  viscera  not  only  have  a  sensory  reflex,  but  also  a  motor 
and  a  visceral  reflex  (Mackenzie). 

I.  The  sensory  reflex  which  serves  for  protection  is  composed  of  a 
sensory  stimulus  in  the  zone  of  the  organ  concerned.  This  is  either 
internal  or  splanchnic  and  is  pain,  though  all  authors  are  not  agreed 
upon,  this,  or  external  or  somatic,  where  pain  is  readily  felt  in  the 
hyperesthetic  zones.  The  following  are  the  characteristics  of  these 
sensations  of  pain: 

(a)  Splanchnic  pain  usually  is  in  the  midline,  even  if  the  organ  by 
which  it  is  caused  lies  on  one  side  or  partly  so  (esophagus,  stomach, 
liver,  intestines,  kidneys). 

(6)  Contrary  to  the  pain  of  hyperesthetic  zones,  splanchnic  pain  is 
not  relieved  by  moderate  chloroform  narcosis. 

(c)  The  radiating  external  pains  are  never  precisely  defined. 

{d)  The  radiating  external  pains  are  felt  in  deeper  structures  (muscles, 
breast)  not  in  the  superficial  layers  of  the  skin. 

(e)  Artificial  stimuli  as  alcohol,  mustard  plasters,  hot  applications, 
cantharides  and  the  galvanic  or  faradic  currents  prevent  viscerofugal 
stimuli  from  passing  through  the  spinal  roots  to  the  hyperalgesic  zones. 
They  act  as  quieting,  pain-relieving  agents  (derivantia  et  revulsiva). 

(/)  Both  t^pes  of  pain,  visceral  and  radiating,  are  increased  by  intense 
emotions,  as  fear  and  anger. 

II.  The  motor  reflex  usually  consists  in  some  stimulation  in  that^part 
of  the  external  musculature  corresponding  to  the  organ  of  stimulation. 
Examples  are  contraction  of  the  chest  muscles,  particularly  the  large 
spinal  muscles  ''  signe  des  spiriaux,"  which  occurs  in  pleuriti©',  and  con- 
traction of  the  abdominal  muscles  in  diseases  of  the  stomach,  intestines 
and  liver.  Sherrington  was  one  of  the  first  to  demonstrate  the  role  of 
the  efferent  sympathetic  nerves  in  these  reflexes.  He  cut  the  abdominal 
visceral  nerves  and  stimulated  their  central  ends.  There  resulted  a 
distinct  contraction  of  the  abdominal  musculature  corresponding  to 
the  nerve  which  was  stimulated.  This  visceromotor  reflex  stimulation 
gradually  disappeared  on  cutting  the  posterior  roots  which  carried  the 
stimuli  from  the  visceral  nerves. 

III.  The  organ  reflex  is  usually  a  secretory,  peristaltic  or  anti- 
peristaltic stimulation  in  the  organ  which  is  stimulated.  Examples 
are  salivation,  gastrosuccorrhea,  vomiting,  accumulation  of  mucus 
in  the  bronchial  tree  and  hiccough.  In  all  these  reflexes  the  primary 
sensory  irritation  plays  the  main  role  (Head,  ^Mackenzie,  Forster). 

Tabetics  often  do  not  have  these  reflexes  due  to  visceral  anesthesia. 
Such  failures  to  respond  normally  occur  in  instances  like  the  following: 
cremaster  reflex  after  stimulation  of  the  testicle,  pain  and  abdominal 
contractions  during  parturition,  abdominal  pain,  vomiting  and  muscle 
spasm  in  appendicitis. 

Toothache  often  gives  a  viscerosensory  hyperesthesia  of  the  cheek. 


96     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

The  pain  in  the  shoulder  of  the  same  side  as  a  pneumonia  is  due  to 
the  fact  that  the  sensory  innervation  of  the  shoulder  comes  from  the 
same  spinal  segments  (C4  C5)  as  the  phrenic  nerve  supplying  the 
pleura. 

The  severe  pain  of  pleuritis  caused  by  deep  breathing  is  not  due  to 
pain  in  the  pleura,  but  to  a  visceromotor  reflex  involving  the  inter- 
costal and  spinal  muscles. 

In  heart  disease  pain  lies  in  the  arm  and  thoracic  region  whose 
sensory  supply  comes  from  the  same  spinal  segments  (D3  D4)  as  the 
S}Tii pathetic  nerves  to  the  heart.  The  rare  occurrence  of  pain  in  the 
skin  of  the  neck  and  the  left  neck  muscles  is  due  to  the  afferent  vagus 
fibers  which  carry  the  stimuli  over  into  the  second  and  third  cervical 
segments.  A  more  significant  visceromotor  heart  reflex  is  the  feeling 
of  oppression  which  results  from  a  radiating  spasm  of  the  intercostal 
muscles.  A  cardiac  organ  reflex  of  less  significance  is  the  salivation 
and  profuse  urination  which  occurs  in  angina  pectoris,  both  symptoms 
due  to  reflex  stimulation  of  the  centers  lying  near  the  vagus  center  in 
the  floor  of  the  fourth  ventricle. 

The  feeling  of  cold  in  the  stomach  after  drinking  cold  water  is  due 
to  contraction  of  the  skin  bloodvessels  and  is  accompanied  by  a  cir- 
cumscribed area  of  goose-flesh.  Thus  there  are  two  visceral  reflexes, 
one  pilomotor,  the  other  vasomotor.  The  visceromotor  reflex  contrac- 
tion of  the  upper  left  rectus  abdominis  in  gastric  ulcer  is  explained  by 
the  origin  of  the  sympathetic  gastric  fibers,  D6,  D7.  The  heartburn 
which  results  from  regurgitation  into  the  esophagus  localizes  itself  one 
segment  higher.  Pain  of  gastric  origin  rarely  is  localized  according  to 
the  position  of  the  ulcer  or  carcinoma.  It  is  usually  in  the  median  line, 
and  in  cardiac,  fundal  and  p^doric  disease  is  found  in  the  upper,  middle 
and  lower  part  of  the  epigastrium. 

A  very  interesting  organ  reflex  in  appendicitis  is  difficulty  of  urin- 
ation which  is  not  infrequently  associated  with  the  hyperesthesia  over 
McBurney's  point. 

Pain  in  the  upper  arm  in  liver  disease  is  explained  b}^  the  origin  in 
the  same  spinal  cord  segment,  C4,  of  the  nerves  to  the  arm  and  the 
phrenic  nerve  to  the  liver. 

In  disease  of  the  kidney  pelvis  and  ureters  the  viscerosensory  reflex 
passes  to  the  testicles,  the  visceromotor  to  the  cremaster  and  trans- 
verse muscles  since  the  nerve  supply  is  derived  from  the  eleventh  and 
twelfth  dorsal  and  first  and  second  lumbar  segments.  For  this  reason 
the  testicles  and  the  scrotum  are  sensitive  in  cases  of  nephrolithiasis. 

Since  the  upper  portion  of  the  bladder  is  developed  from  the  allantois 
(L2)  and  the  lower  from  the  cloaca  (S2,)  the  reflex  pain  depends  upon 
the  site  of  the  disease.  Sometimes  it  is  above  the  pubic  bone,  sometimes 
in  the  penis  and  perineum.  The  visceromotor  reflex  causes  spasm  of  the 
sphincter  ani. 

Reflex  pain  from  the  uterus  and  ovaries  lies  in  the  lower  abdomen. 

Pain  in  the  knee  in  hip-joint  disease  is  due  to  the  identity  of  the 
spinal  segment  supplying  fibers  to  these  two  joints,  L4.     The  synovia 


TREMORS  97 

are  supposed  to  be  supplied  by  the  vegetative  nervous  system  just  as 
the  peritoneum. 

Tremors. — The  tremors  of  the  upper  extremities  alone  claim  atten- 
tion. Those  of  the  head  may  be  fine  and  oscillatory,  nodding,  rhyth- 
mical, or  jerky.    The  rapidity  of  the  tremors  should  be  noted. 

Tremors  of  the  hand  and  wrist  should  first  be  tested  with  the  pendant 
hand  supported  at  the  wrist.  Then  with  hands  extended  and  fingers 
wide  apart  static  tremors  come  out.  Or  with  the  hands  in  motion 
locomotor  tremors  become  evident. 

Static  tremors  are  either  fine  and  rapid  (eight  to  twelve  per  second), 
or  coarse  and  slow  (four  to  six  per  second).  They  may  be  irregular. 
When  involving  the  whole  arm  one  speaks  of  movement  rather  than 
tremors. 

liocomotor  tremors  are  in  reality  ataxias.  These  ataxias  are  brought 
out  best  by  having  the  patient  bring  his  index  fingers  from  any  position 
slowly  to  the  end  of  the  nose,  first,  with  eyes  open,  then  with  eyes 
closed,  test  both  sides  (finger- nose  test — F.  X.  T.),  or  the  index  fingers 
should  be  brought  to  touch  each  other  (finger- finger  test — F.  F.  T.). 
Here  coarse  irregular  movements  (ataxias)  may  be  brought  out.  The 
patient's  finger  may  overshoot  the  nose  (dysmetria).  The  patient's 
ataxia  increases  markedly  as  the  nose  is  reached  (intention  tremor), 
or  increases  only  just  as  the  object  sought  is  arrived  at. 

Ataxia  in  the  lower  extremities  is  tested  by  the  knee-heel  test 
(K.  H.  T.),  the  patient,  on  his  back,  is  directed  to  touch  the  left  knee 
with  the  right  heel,  and  vice  versa. 

Athetoid  mo\'ements  are  coarse,  slow,  sinuous,  progressive,  rhyth- 
mical movements  in  the  fingers,  arm,  or  trunk. 

Choreic  movements  are  irregular,  coarse,  or  fine  movements,  non- 
rhythmical  and  non-coordinated — they  are  jerky  movements. 

Associated  movements  are  involuntary  movements  of  the  opposite 
side,  induced  by  a  voluntary  act.  Not  infrequently  they  are  quite 
non-homologous  movements  (abduction  of  left  leg  when  making  effort 
with  right  arm). 

Localized  convulsive  movements  and  Jacksonian  epileptic  move- 
ments consist  of  sudden  convulsive  involuntary  extensions  and  flexion 
without  loss  of  consciousness. 

Tics. — These  are  involuntary,  coordinated  movements  of  psychic 
origin. 

Diadokokinesis. — This  signifies  the  ability  to  perform  alternate  rapid 
coordinated  movements,  of  antagonistic  muscles.  Certain  patients 
show  a  loss  of  this  ability  (adiadokokinesis).  The  tests  most  fre- 
quently applied  are  (juick  pronation  and  supination  of  the  semiflexed 
hand;  piano-playing  movements  or  quick  flexion  and  extension  of  the 
forearm  on  the  arm.  The  term  is  applicable  only  in  the  absence  of 
motor  paresis  or  gross  anesthesia.' 

Apra.via. — This,  speaking  generall\',  consists  in  the  loss  of  ability 
to  perform  purposeful  nio\ements.     The  tests  are  to  have  the  patient 

'  Plastic  tonus  and  proprioceptive  reflex,  Sherrington:  Q\iart.  .Jour.  Physiol.,  1909,  ii. 

7 


98       METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

throw  a  kiss,  make  a  salute,  a  beckoning  gesture,  a  threatening  gesture, 
or  to  go  through  an  imaginary  act;  such  as  taking  a  match  out  of  a 
box  and  Hghting  it  or  blowing  it  out.  It  is  also  elicited  by  testing  the 
necessary  movements  in  using  objects  correctly. 


Fi(i.   3.5. — lllustniting  the  segmental  spinal  sensory  areas.     Front  view. 

EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM. 

The  most  important  of  the  tests  of  the  sensory  nervous  system  are 
for:  (1)  light  touch,  (2)  pain,  (3)  thermal  sensations,  and    (4)  deep 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM 


99 


sensibility.  Head  has  suggested  the  terms  epicritic,  protopathic,  and 
deep  sensibility  for  the  three  types  of  sensibihty  which  he  maintains 
exist,  ^^^lile  there  are  good  reasons  to  refer  protopathic  sensibility 
to  the  vegetative  (affective)  systems  the  original  point  of   view  of 


D3- 


Fio.  36. — Illustrating  the  segmental  spinal  sensory  areas.    Rear  view.    These  or  similar 
sen.sory  charts  are  useful  in  plotting  sensory  changes. 

Head  is  here  outlined  with  reservations,  mitil  this   much  discussed 
l)robleni  of  sensibility  becomes  more  definitely  formulated.^ 

'  Hyrncs,  J.:     Jour.  Ncrv.  and  Ment.  Dis.,  1919. 


100     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

1.  Epicritic  sensibility  is  tliat  which  recognizes  light  touch,  dis- 
tinguishes small  differences  between  the  points  of  a  compass,  and 
recognizes  small  variations  in  the  temperature  of  objects. 

2.  Protopathic  sensibility  recognizes  pain  and  extremes  of  heat  and 
cold.  It  and  the  following  are  probably  functions  of  the  vegetative 
system. 

3.  Deep  sensibility  recognizes  deep  pain  and  muscle  and  joint  sense. 
Bony  sensibility  is  included  here. 

Epicritic  Sensibility. — To  test  epicritic  sensibility  one  first  tries  the 
method  of  light  touch.  Touching  the  skin  with  the  end  of  the  finger  is 
not  a  light  touch  test.  Such  a  test  is  too  coarse.  It  is  a  test  for  deep 
sensibility  because  it  deforms  the  surface.  Either  a  wisp  of  cotton- 
wool should  be  used  or  a  fine  camel-hair  brush.  For  most  purposes 
the  cotton- wool  is  to  be  preferred.  In  accurate  testing,  special  esthesi- 
ometers  are  indispensable.  Those  mostly  in  use  are  von  Frey's  hairs 
or  Franz's  simple  esthesiometer. 


Fig.  37. — Holmes's  compass  for  testing  touch  discrimination. 

The  patient's  body  should  be  explored  systematically.  He  is  asked  to 
say  "Yes"  every  time  he  is  touched,  and  then  asked  to  localize  the 
spot  touched.  The  testing  should  be  made  with  the  patient  first  lying 
down,  and  due  attention  should  be  given  to  the  character  (thickness, 
etc.),  of  the  individual  patient's  skin  in  drawing  conclusions  from  the 
tests.  In  going  over  the  extremities  care  should  be  taken  to  circle 
the  limb  with  the  touches  as  well  as  going  up  and  down.  It  is  specially 
desirable  to  avoid  suggestive  qiiestions,  such  as,  Do  you  feel  this? 
What  do  you  feel?  etc.  In  certain  cases,  usually  hysterical,  one  will 
get  the  steady  response  "No"  to  each  touch  over  the  so-called  anes- 
thetic area.  This  is  a  highly  suggestive  reaction.  Modifipations  of 
light  touch  should  be  charted  and  marked  on  the  skin  with  an  anilin 
pencil.  Such  indications  are  very  useful  as  landmarks  for  localization. 
There  may  be  anesthesia  to  light  touch  or  hyperesthesia,  the  patient 
feeling  very  acutely.  In  all  hairy  parts  the  skin  should  be  shaved  for 
accurate  testing,  otherwise  cotton-wool  drawn  across  the  skin  by  bend- 
ing the  hairs  will  deform  the  surface  and  so  give  a  deep  sensibility 
resi)onse  to  test  for  light  touch.  (See  Plates  IX  and  X  for  the 
pathways  involved.) 

A  Weber  compass  is  useful  for  testing  the  individual  capacity  for 
recognizing  one  or  two  points.    That  devised  by  Gordon  Holmes'  is 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM  101 

the  most  practical.  There  is  a  great  variability  in  individuals  and 
in  different  regions.  Some  of  the  available  figures  for  the  minimum 
separation  distance  recognized  as  two  points  are  as  follows: 

Tongue 1.5  mm. 

Finger-tips 2.3  mm. 

Lips 3.4  mm. 

Dorsum  of  fingers      .      .      .      .• 5.6  mm. 

Forearm 9.1  mm. 

Forehead 10  to  15  mm. 

Neck 23  to  30  mm. 

Leg:  back  of  foot 30  to  40  mm. 

Back 50  to  60  mm. 

Arms  and  thigh 70  to  80  mm. 

Further  tests  may  be  made  of  epicritic  light  touch  by  placing 
variously  shaped  objects  on  the  skin.  Such  tests  are  very  valuable 
in  special  cases. 

Epicritic  thermal  sensibility.  This  is  most  easily  tested  by  use  of 
the  back  of  the  finger  for  warm,  and  the  metal  head  of  a  percussion 
hammer  for  cool.  Epicritic  thermal  sensibility  recognizes  differences 
as  small  as  two  to  five  degrees  of  temperature,  while  protopathic 
sensibility  is  unable  to  recognize  differences  between  40°  and  20°  C. 
Loss  of  epicritic  sensibility  for  heat,  with  preservation  of  protopathic 
thermal  sensibility,  is  not  uncommon.  The  reverse,  while  rare,  is 
occasionally  found. 

In  making  careful  thermal  tests  an  electrical  thermometer,  as  con- 
trived by  Mills  is  useful.  In  ordinary  routine  work  test-tubes  with 
ice-water  and  hot  water  may  be  used,  or  metal  tubes  which  have  been 
plunged  in  cold  or  hot  water.  It  is  not  sufficient  to  test  patients  for 
extremes  of  heat  and  cold  alone;  minute  differences  should  be  tested 
for  as  well, 

Protopathic  Sensibility.- — Pain. — This  is  quickest  tested  by  pinching 
the  skin  between  the  nail  and  the  finger.  A  sharp-pointed  pin  with  a 
round  glass  head  is  also  useful.  The  patient  is  asked  to  distinguish 
between  head  and  point.  Absence  of  pain  (analgesia)  should  be  care- 
fully charted  as  well  as  increased  pain  sensibility  (hyperalgesia).  The 
limbs  should  always  be  tested  in  their  circumference  as  well  as  in 
their  length,  care  being  taken  not  to  overlook  thin  strips  of  analgesia 
from  root  lesions.  Hair  sensibility  should  also  be  tested  by  pulling 
the  hair.  Painful  faradic  stimulation  is  at  times  of  value  in  deter- 
mining the  value  of  an  existing  analgesia. 

Deep  Sensibility. — Here  deep  pressure  pain,  muscle  and  joint  sense 
and  bony  sensibility  are  to  be  tested.  Deep  pressure  with  the  thumb 
and  fingers,  or  a  special  instrument  (baresthesiometer),  is  used.  The 
pressure  should  be  sufficient  to  cause  pain. 

Muscle  and  joint  sense  are  tested  by  first  showing  the  patient  that 
one  moves  the  thumb  and  big  toe  up  or  down — and  then  repeating 
movements  up  or  down  while  the  eyes  of  the  patient  are  closed. 
Further,  weights  may  be  used  on  the  supported  and  unsupported  hand, 
and  the  ability  to  estimate  differences  observed;  or  the  patient  is 
requested  to  imitate  with  one  hand  a  definite  position  of  the  other  hand. 


102     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 


Bony  sensibility  is  tested  by  a  tuning-fork  of  low  vibrating  capacity. 
This  is  placed  still  vibrating,  upon  bony  surfaces,  and  sensation  is 
intact  when  the  patient  feels  the  thrill.     It  is  a   highly  important 

test,  particularly  in  lesions  of  the  peripheral 
nerves,  spinal  cord  and  thalamus. 
/^I  Sensibility  of  the   nerve  trunks  to  direct 

pressure  should  then  be  tested.  In  the  upper 
arm  the  brachial  plexus  branches  in  the  neck 
and  under  the  arm  are  palpable  and  along  the 
inner  arm  and  elbow-joint  one  may  reach  the 
median,  radial,  and  ulnar.  Anesthesia  of  the 
ulnar  (Biernacki)  is  frequently  a  tabetic 
symptom. 


:> 


DlOi 


Is  2 


^Sl 


Fig.  38. — Illustrating  the 
segmental  spinal  sensory 
areas.     Side  view. 


Fig.  39. 


-Illustrating    the    segmental    spinal    sensory 
areas  of  the  lower  extremities. 


The  radicular  and  peripheral  sensory  distributions  are  shown  in 
Figs.  27,  28,  29,  30,  31  and  32. 

In  the  lower  limb  the  sciatic,  anterior  crural,  cutaneous  femoris, 
tibialis,  and  superficial  peroneus  are  palpable. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM 


103 


Lasegws  Test. — This  consists  in  flexing 
the  extended  leg,  keeping  it  extended  by 
pressure  on  the  knee,  on  the  abdomen, 
when  in  neuritic  processes  a  sharp  pain 
(popHteal  space)  is  brought  out.  It  is 
an  indispensable  test  in  the  presence  of 
suspected  alcoholic  neuritis. 

The  distribution  of  pain  in  neuralgic 
or  neuritic  affections  should  be  carefully 
charted.  With  the  sensory  examination, 
gnostic  and  praxic  tests  should  be  carried 
out. 

Stereognosis  signifies  the  ability  to 
recognize  objects  by  touch.  Astereo- 
gnosis,  first  described  by  Puchelt  in 
1844,  is  its  absence.  In  a  wider  sense 
the  tests  indicate  the  perception  of 
spatial  and  quality  relations  through  the 
sense  of  touch.  Objects  should  not  only 
be  named  but  their  qualities  described — 
shape,  margins,  density,  etc.  A  lump  of 
sugar,  thimble,  match-box,  marble,  knife, 
pencil,  scissors,  etc.,  are  useful  test  ob- 
jects. 

Apraxia  consists  in^  the  loss  of  ability 
to  carry  out  a  purposeful  movement,  not 
dependent  on  a  palsy.  The  most  useful 
tests  have  been  referred  to.  The  student 
is  specially  referred  to  Plates  IX  and  X 
for  the  interpretation  of  his  findings  and 
their  anatomical  foundations. 

Vasomotor  and  Trophic  Disturbances. — 
The  presence  of  dermographia,  of  blush- 
ing, of  redness,  or  blanching  of  the  skin 
should  be  looked  for.  Ulcers,  thickness 
of  skin,  dryness,  or  other  trophic  dis- 
orders should  be  charted.  Reflex  hyper- 
algesias (referred  pains)  should  always  be 
inquired  for.  Patients  refer  to  them 
chiefly  as  "sore  spots."  (See  Figs.  40 
and  41.) 

Scheme  for  Testing  Sensibility. — The  fol- 
lowing scheme  for  testing  sensibilityMs  si-^ 
advised : 


^//l 


G 


0^ 


y. 


\ 


-Si 


■Si 


Ll- 


^^i 


U 


>  Gans:  Zeit.  f.  d.  g.  N.  u.  P.,  1916,  xxxi. 

Fig.  40. — Cutaneous  reflex  zones  of  hyperalgesia,  showing  their  relations  with  the 
spinal  root  segments  and  their  vegetative  nervous-system  connections.  The  dotted 
areas  are  to  be  referred  to  the  internal  surfaces.     (After  Dejerine.) 


104     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

Vertical  (DS) 


Tcinpordl  (D7) 


Orbital  (./>-', 

Nasofrontal  (C3,4) 
Temporofrontal  (DOS) 

Maxillary 


Nasolabial 


trivial  (D3) 


Occipital  (DIO) 


Fig.  41. — Cutaneous  reflex  zones  of  hyperalgesia  of  the  head,  neck,  and  shoulders  in 
their  relations  to  vegetative  nerve  (somatic)  disturbances.     (After  Dejerine.) 

A.  Spontaneous  Sensations:     Pain,  numbness,  tingling,  position  of 

the  limb,  idea  of  the  limb,  hallucinations  or  illusions. 

B.  Loss  of  Sensation: 

1.  Touch. 

(a)  Light  touch,  cotton-wool  on  hairless  and  shaved 

hair-clad  parts;  threshold  with  von  Frey's  hairs. 

(b)  Pressure  touch,  threshold  with  pressure  esthesiometer. 

2.  Localization:     Naming    the    part    touched.     Henrj^'s    or 

Head's  method,  target,  etc. 

3.  Roughness:    Threshold    with    Graham-Brown's    esthesi- 

ometer.    Sand-paper  tests,   discrimination   of  relative 
roughness. 

4.  Tickling   and   scraping:    Tickling   on    soles   and    palms. 

Cotton-wool  rubbed  o^'er  hair-clad  parts.     Light  scrap- 
ing with  finger-nails. 

5.  Vibration,  tuning-fork:    Loss  or  diminution  of  sensibility. 

Alteration  in  the  character  of  the  sensation  evoked. 

6.  Compass  points:     Points  simultaneously  applied.     Points 

successively  applied. 


EXAMINATION  OF  SENSORY  NERVOUS  SYSTEM  105 

7.  Pain: 

(a)  Superficial  pain:  pin-prick;  threshold  with  algesim- 
eter;  reaction  to  measured  painful  stimuli. 

(h)  Pressure  pain:  threshold  with  the  algometer;  reac- 
tion to  painful  pressure. 

8.  Temperature:    Thresholds  for  heat  and  cold.     P^tt'ect  of 

adaptation  on  threshold.  Discrimination  of  different 
degrees  of  heat  and  cold.  Affective  reactions:  (a)  to 
extreme  degrees,  (b)  to  warmth. 

9.  Position:     By  imitating  w4th  the  sound  limb  the  position 

of  the  affected  limb;  by  pointing  with  the  sound  limb; 
measurement  of  defect  by  Horsley's  method. 

10.  Passive  movement:     Appreciation  of  movement.     Recog- 

nition of  the  directions  of  movement.  Measurement  of 
the  angle  of  the  smallest  movement  which  can  be 
appreciated;  falling  away  of  the  unsupported  limb  when 
the  eyes  are  closed. 

11.  Active  movement:     Imitation  of  movement  by  the  sound 

limb;  ability  to  touch  a  known  spot;  measurement  of 
the  defect  by  Horsley's  method. 

12.  Weight: 

(a)  With  hand  supported :  Recognition  of  differences  in 
weights  applied  successively  to  one  hand.  Appre- 
ciation of  increase  or  decrease  of  weight.  Com- 
parison of  two  weights  placed  one  in  each  hand. 

(6)  With  hand  unsupported :  Comparison  of  two  weights 
placed  one  in  each  hand.  Recognition  of  differ- 
ences in  weights  applied  successively  to  one  hand. 

13.  Size:  Difference;  threshold.     Distinction  of  the  head  from 

the  point  of  a  pin. 

14.  Shape  (two  dimensional). 

15.  Form  (three  dimensional) :  Recognition  of  common  objects 

by  their  form. 

16.  Textures. 

17.  Dominoes:    Ability  to  count  points  by  touch. 

18.  Consistence. 

19.  Testicular  sensibility: 

(a)  Light  pressure.   - 
(6)  Painful  pressure. 

20.  Sensibility  of  glans  penis  to  measured  prick. 

Status  Corporis. — A  systematic  physical  examination  is  a  sine  qua 
non.  The  main  facts  to  be  noted  in  the  questionnaire  are  the  con- 
dition of  the  heart,  the  presence  of  murmurs,  the  character  of  the 
arteries  (hard,  tortuous),  blood-pressure,  the  lungs,  presence  of  tumor 
in  abdomen,  enlargement  of  liver,  and  the  condition  of  the  urine,  the 
blood  with  special  reference  to  leukocytosis  as  an  index  for  hidden 
sources  of  infection,  and  the  cerebrospinal  fluid. 


lOG     MET  HOD  fi  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 
MENTAL-STATUS  QUESTIONNAIRE. 


General  Demeanor : 

Uniform : 

Hair: 

Hands : 

Reaction :               Free : 

Reserved : 

Respectful : 

Hostile: 

Friendly : 

Puerile : 

Evidences  of  struggle: 

Bruises: 

Accessibility : 

Natural : 

Free: 

Alert: 

Happy: 

Elated: 

Exuberant : 

Dull: 

Apathetic: 

Somnolent : 

Depressed : 

Gloomy: 

Worried : 

Fearful : 

Suspicious: 

Perplexed : 

Disdain : 

Scornful : 

Sarcastic : 

Motor  Activity: 

Average : 

Excited : 

Diminished : 

Slow : 

Resistive: 

Frigid: 

Queer : 

Bizarre: 

Speech  Activity: 

Average : 

Talkative : 

Noisy: 

Quiet: 

Slow: 

Mute: 

Disordered : 

MENTAL-STATUS  QUESTIOXXAIRE  .  107 

MENTAL-STATUS  QUESTIONNAIRE— C'ow?ww^(i. 


Stream  of  Thought: 

Description : 

Clear:                 Logical: 

Relevant : 

Jumping:            Coherent 

Incoherent : 

Retarded : 

Irrelevant : 

Disconnected : 

Queer : 

Fragmentary: 

Jumbled : 

Examples : 

Content  of  Thought : 

Worry : 

Insight : 

Apprehensive : 

Anxiety: 

Delusional : 

Phobias: 

Hallucinatory : 

Character : 

Peculiar  Experience : 

Comparison,  former  states: 

Attitude  of  Friends : 

Suicidal  Ideas: 

Dreams : 

General  Orientation: 

Full  name: 

Age: 

When  Born: 

Present  Year 

^lonth            Day 

City: 

Place: 

When  here : 

How  Long: 

What  here: 

Patients : 

Are  you  sick : 

Why  here : 

1 

108       METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

MENTAL-STATUS  QUESTIONNAIRE— Cow/i/me(^. 


Subjective  Idea  of  Memory : 

Attention : 

Thinking : 

Capacity: 
Memory — Combination : 

7  X  S]  r  9  X  8] 

972864]  45319G28] 

516724]  |_  97125684] 

.^  +  19  =  3(3]  .T  -  27  =  15] 

a;  X     7  =  84]  a;  --    8  =  7] 

Change : 

2  eggs  cost  10 — how  much  3] 
School  Knowledge: 

History : 

Geography : 

Countries : 

Rivers : 

Capitals: 
Natural  Objects: 

Birds:  Flowers: 

Fish :  Postage : 

Animals:  Local: 

Masselon :     Hunter — Forest — Gun — Dog — Rabbit] 


Description 


MENTAL-STATUS  QUEST lOXN AIRE  109 

MENTAL-STATUS  QUESTIONNAIRE— C'on^mwed. 


Finckh — People  who  live] 
Ebbinghaus : 
Differences: 

Ox 

Horse 

Ice 

Water 

Steps 

Ladders 

Lie 

Mistake 
Continuous  Sentence: 

If  it  rains] 
Because] 
Nevertheless] 

If  the  Soup] 
Because], 
Nevertheless] 
Backward  Associations : 

Name] 

Months] 
Story: 

Reproduction  Picture: 
Name — Writing:     Eyes  open 


Description : 


Box 

Basket 

Bird 

Butterfly 

Dwarf 
Child 
Shrub 
Tree 


Week  Days] 
75820] 


and  closed : 


no     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

MENTAL-EXAMINATION  METHODS. 

In  no  department  of  medicine  is  a  complete  examination  of  the 
patient  more  important  than  in  that  of  psychiatry.  This  examina- 
tion must  not  only  include  the  symptoms  that  the  patient  may  present 
when  seen,  but  must  also  include  the  most  detailed  obtainable  anam- 
nesis. It  must  be  borne  in  mind  that  a  psychosis  is  a  new  condition  in 
an  individual  who  was  previously  well.  Above  all  it  is  not  a  something 
that  comes  from  without,  which  attacks  and  seizes  on  the  patient  like, 
for  example,  a  pathogenic  microorganism,  but  is  rather  to  be  con- 
sidered as  a  tj^pe  of  reaction  of  the  individual  to  certain  inimical 
conditions.  In  order,  therefore,  to  understand  a  particular  case  it  is 
of  the  highest  importance  to  have,  as  fully  as  possible,  a  conception 
of  the  individual  before  he  became  afflicted,  so  that  the  symptoms 
which  are  the  expressions  of  this  reaction  may  be  understood. 

The  scheme  of  examination  which  follows  is  directed  primarily  to 
elucidating  the  mental  state.  It  is  taken  for  granted  that  the  student 
is  familiar  with  the  various  methods  of  physical  examination.  The 
omission  of  specific  directions  as  to  the  physical  examination  is  not, 
however,  to  be  taken  as  an  indication  that  it  is  considered  unimpor- 
tant. On  the  contrary,  a  physical  examination  in  minute  detail  is. 
of  the  utmost  importance  and  unless  it  is  made  the  risk  is  bound  to  be 
run  that  the  key  to  the  whole  situation  will  be  overlooked. 

The  principal  value  that  a  scheme  of  examination  may  have,  how- 
ever, is  in  formulating  tests  that  call  for  an  actual  record  of  the  patient's 
reaction  and  not  the  conclusions  of  the  examiner.  Hospital  records 
are  filled  with  such  remarks  as  "the  patient  shows  lack  of  judgment" 
or  is  "disoriented"  or  has  "failure  of  memory."  All  of  these  are 
conclusions  and  are  not — records  of  facts.  Such  histories  are  useless 
to  anyone  except  perhaps  the  persons  who  wrote  them.  The  reader 
of  a  history  is  entitled  to  a  statement  of  the  facts  on  which  the  con- 
clusions are  based  and  then  he  is  at  liberty  to  form  his  own  conclusions 
from  the  identical  premises.  How  much  better  and  more  accurate 
than  the  statement  "defective  memory"  would  be  this  test:  The 
patient  in  the  course  of  the  examination  is  given  the  address  375 
Oxford  St.  After  five  minutes  he  is  asked  to  recall  it.  He  gives  the 
number  176,  but  cannot  give  the  name  of  the  street.  Here  is  a  definite 
fact.  A  multiplicity  of  such  facts  gives  one  a  basis  for  conclusions 
about  the  patient.  Of  such  statements  should  the  record  of  an  exam- 
ination be  composed. 

Mental  Examination. — Orieniation:    time;  place;  persons. 

General  memory:  family;  school;  occupation;  marriage;  children; 
diseases. 

Emotional  status:    insight;  sleep;  dreams. 

Halhwinations:    auditory;  visual;  other  senses. 

Speech:  voluntary;  writing  (name,  date,  the  United  States  of 
America,  the  Commonwealth  of  Massachusetts);  auditory;  visual;  test- 
phrases.     (Statistical,  perturbation,  Third  Riding  Artillery  Brigade.) 


MENTAL-EXAMINATION  METHODS  111 

Stories  ("Cowbov,"  "Gilded  Bov,"  "Polar  Bear,"  "Shark,"  "Good 
Girl"). 

Special  memory:  Civil  War;  names  of  two  generals;  three  European 
countries;  capital  of  native  State;  President;  45319628;  35984271; 
487631;  955217;  7368;  487;  352;  375  Oxford  Street  (after  three  to  five 
minutes). 

Masselon  (hunter,  dog,  gun,  forest,  rabbit;  man,  wood,  coal,  stove, 
dinner;  needle,  thread,  button,  vest;  pipe,  match,  smoke;  pen,  ink, 
letter). 

Ziehen  (horse  and  ox;  dwarf  and  child;  lie  and  mistake;  water  and 
ice).  7  X  6;  56  -  18;  23  -  14;  81  -  9;  X  -  5  =  17;  X  -  8  =  13; 
have  50c;  buy  cherries  12c,  butter  7c,  bread  10c;  how  much  change? 

Forward  and  Backward  associations  (month;  days  of  week;  752186, 
25729,  6418,  265,  497). 

General  information:  cost  of  postage;  color  of  stamps;  holidays  and 
meaning  (Christmas,  Easter,  Fourth  of  July). 

Finckh  ("The  early  bird  catches  the  worm;"  "Lies  have  short  legs;" 
"Set  a  thief  to  catch  a  thief;"  "Burn  a  candle  at  both  ends"). 

Ethical  questions: 

Drawing  diagram  (after  five  seconds'  exposure). 

Note. — Here  especial  caution  is  needed  to  avoid  recording  conclu- 
sions. For  example:  Under  orientation  the  patient's  actual  answers 
to  such  questions  as.  When  were  you  born?  How  old  are  you?  What 
day  is  this?  etc.,  should  be  put  down. 

The  stories  which  are  named  are  as  follows : 

"Cowboy  Story." — A  cowboy  from  Arizona  went  to  San  Francisco 
with  his  dog,  which  he  left  at  a  dealer's  while  he  purchased  a  new 
suit  of  clothes.  Dressed  finely,  he  went  to  the  dog,  whistled  to  him, 
called  him  by  name  and  patted  him.  But  the  dog  would  have  nothing 
to  do  with  him  in  his  new  hat  and  coat  but  gave  a  mournful  howl. 
Coaxing  was  of  no  effect,  so  the  cowboy  went  away  and  donned  his  old 
garments,  whereupon  the  dog  immediately  showed  his  wild  joy  on 
seeing  his  master  as  he  thought  he  ought  to  be. 

"Gilded  Boy  Story." — It  is  related  that  at  the  coronation  of  one 
of  the  popes,  about  three  hundred  years  ago,  a  little  boy  was  chosen  to 
act  the  part  of  an  angel;  and  in  order  that  his  appearance  might  be 
as  gorgeous  as  possible  he  was  covered  from  head  to  foot  with  a  coating 
of  gold  foil.  He  was  soon  taken  sick,  and  although  every  known 
means  was  employed  for  his  recovery,  except  the  removal  of  his  fatal 
golden  covering,  he  died  within  a  few  hours. 

"  Polar  Bear  Story." — A  female  polar  bear  with  two  cubs  was  pursued 
by  sailors  over  an  ice  field.  She  urged  her  cubs  forward  by  running 
before  them,  and,  as  it  were,  begging  them  to  come  on.  At  last  in 
dread  of  their  capture  she  pushed,  then  carried  and  pitched  each 
before  her,  until  they  actually  escaped.  The  polar  bear  is  a  wonderful 
swimmer  and  diver.  In  the  capture  of  seals  l\ing  on  the  ice,  it  dives 
some  distance  ott*  and  swinmiing  underneath  the  water,  suddenly 
comes  up  close  to  the  seals,  shutting  oil"  their  retreat  to  the  sea. 


112     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

"Shark  Story." — ^The  son  of  a  governor  of  Indiana  was  first  officer 
on  an  oriental  steamer.  When  in  the  Indian  Ocean  the  boat  was 
overtaken  by  a  typhoon  and  was  violently  tossed  about.  The  officer 
was  suddenly  thrown  overboard.  A  life-preserver  was  thrown  to 
him,  but,  on  account  of  the  heavy  sea,  difficulty  was  encountered  in 
launching  the  boat.  The  crew,  however,  rushed  to  the  side  of  the 
vessel  to  keep  him  in  sight,  but  before  their  shuddering  eyes  the 
unlucky  young  man  was  grasped  by  one  of  the  sharks  encircling  the 
steamer  and  was  drawn  under  the  water,  leaving  only  a  dark  streak 
of  blood.     (Adapted  from  Ziehen.) 

"Good  Girl  Story." — Once  upon  a  time  there  was  a  girl  whose 
father  and  mother  were  dead,  and  who  was  so  poor  that  finally  she 
had  nothing  but  the  clothes  on  her  back  and  a  little  piece  of  bread  in 
her  hand.  She  was  deserted  by  everybody,  but  since  she  was  good 
and  honest  she  went  into  the  world  with  confidence  in  God.  As  she 
went  along  she  was  met  by  a  poor  old  man  who  said,  "  Give  me  some- 
thing to  eat,  I  am  hungry."  The  girl  gave  him  the  piece  of  bread  and 
went  on  farther.  Soon  afterward  she  encountered  a  little  girl  freezing 
and  almost  naked,  who  begged  for  her  clothes.  The  good  girl  gave 
the  poor  child  the  warmest  of  her  garments.  Night  came  on,  the 
good  girl  was  tired,  cold  and  hungry.  She  traveled  into  the  woods, 
and,  wandering  off  the  road,  she  knelt  and  prayed  to  God.  As  she 
knelt  she  saw  the  stars  falling  all  about  her,  and  when  she  looked  she 
found  they  were  many  bright  gold  dollars.     (Adapted  from  Ziehen.) 

These  stories  which  are  used  have  been  selected  with  great  care. 
They  are  especially  valuable.  It  is  remarkable  the  amount  of  infor- 
mation that  one  can  obtain  from  getting  a  patient  to  repeat  one  or 
two.  Defects  of  memory  and  attention  show  immediately,  while  the 
manic  tendency  to  elaborate  is  characteristic.  They  should  never  be 
omitted.  The  cowboy  story  is  usually  the  easiest,  while  the  good 
girl  story  is  hard,  because  of  the  great  amount  of  detail.  The  emo- 
tional feature  of  the  "streak  of  blood"  in  the  shark  story  is  particularly 
impressive  and  may  be  about  the  only  feature  of  the  story  reproduced. 

In  the  special  memory  test,  of  course,  different  people  will  have  to 
be  treated  differently.  A  Polish  immigrant  just  landed  would  hardly 
know  about  the  Civil  War.  The  importarrt  thing,  however,  is  to 
record  actual  question  and  answer.  ' 

In  the  Masselon  tests  the  patient  is  asked  to  incorporate  such 
words  as  pen,  ink,  letter,  into  a  sentence. 

In  the  Ziehen  test  the  patient  is  asked  to  tell  the  difference  between 
horse  and  ox,  dwarf  and  child,  etc. 

The  problem  of  calculating  the  change  left  from  50c  after  making 
certain  purchases  is  an  excellent  example  of  the  usefulness  of  standard 
questions.  Everyone  on  the  hospital  staff  knows  that  the  answer 
is  21c;  and  although  this  is  a  little  thing,  when  multiplied  many  times 
it  makes  a  great  deal  of  difference  in  the  ease  with  which  one  can  go 
over  a  history  or  appreciate  it  when  read. 

The  forward  and  backward  associations  are  valuable  as  roughly 


MENTAL-EXAMINATION  METHODS  113 

quantitative.  The  average  person  should  be  able  to  give  six  numbers 
forward  and  five  numbers  backward.  This  test  will  disclose  just  how 
many  the  patient  can  give  and  is  one  of  the  valuable  tests  for  repeating 
from  time  to  time  during  the  course  of  the  psychosis.  It  is  also  very 
valuable  in  detecting  the  malingerer.  A  definite  intention  to  blunder 
is  usually  readily  distinguishable  from  a  natural  blunder. 

In  the  Finckli  test  the  patient  is  asked  the  meaning  of  sayings  or 
proverbs  such  as,  ''The  early  bird  catches  the  worm." 

Such  ethical  questions  can  be  asked  as,  What  would  you  do  if  you 
saw  a  man  drop  a  SIO  bill? 

In  addition  to  the  tests  given  in  the  table  frequent  use  is  made  of 
the  Ebbinghaus  test  which  consists  of  having  the  patient  complete  a 
sentence  in  which  certain  words  have  been  left  out,  such  as:  I  got  up 
in  the  .  .  .  and  after  washing  my  .  .  .  went  to  ...  Or 
better  often  is  Ziehen's  modification  of  this  test.  The  patient  is  asked 
to  complete  such  a  sentence  as  this:  If  it  rains  .  .  .  because 
.  .  .  .  in  spite  of  .  .  .  The  Bourdon  test  is  very  valuable  as 
a  measure  of  attention.  It  consists  of  getting  the  patient  to  strike 
out  certain  recurring  letters  or  numbers  in  a  standard  page  and  timing 
the  result.  A  similar  test  is  the  tapping  test — timing  the  number  of 
taps  that  can  be  made  in  a  given  time,  say  thirty  seconds. 

The  cases  will  be  numerous  in  which  it  will  be  found  desirable  or 
necessary  to  pursue  the  examination  further  in  some  direction.  Xo 
scheme  can  cover  all  possibilities  and  would  be  useless  if  it  did,  because 
impossible  to  carry  out.  ]Much  must  of  necessity  be  left  to  the  judg- 
ment of  the  examiner.  By  following  this  plan,  however,  it  is  believed 
that  the  general  and  important  features  necessary  for  a  case  record 
will  be  covered  in  the  large  majority  of  cases. 

It  is  useful,  after  completing  the  examination,  to  accent  the  signifi- 
cant features  in  a  short  summary,  which  might  include  a  provisional 
diagnosis  if  the  facts  warranted. 

The  tests  here  described  are  for  the  most  part  intelligence  tests. 
Even  such  questions  as  might  be  propounded  under  the  head  of 
"ethical  questions"  may  very  easih'  have  only  the  value  of  intelligence 
tests  because  the  patient  will  quite  likely  answer  in  accordance  with 
the  conventional  ideas  with  which  he  is  perfectly  familiar  rather  than 
answer  in  accordance  with  the  way  in  which  he  feels.  While  the  intel- 
ligence tests  are  important  and  while  by  taking  them  in  a  routine 
manner  one  can  get  a  good  deal  of  information  from  the  patient,  often 
information  of  matters  that  lie  deeper  than  mere  questions  of  intel- 
ligence, still  they  are  b>'  no  means  all-sufficient.  The  intellect  is  after 
all  only  superficial  as  a  guide  to  conduct.  The  deeper  moti\es  that 
move  men  to  action  come  from  the  realm  of  feeling,  and  if  the  symp- 
toms are  to  be  explained  or  understood  the  emotional  springs  of  conduct 
must  be  fathomed. 

The  same  criticism  may  be  made  of  the  Binet-Simon  tests  which 
were  devised  for  determining  the  degree  of  mental  defect  expressed 
in  terms  of  the  psychological  (7.  v.)  age.     These  tests  are   valuable 


114     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

in  skilled  hands  for  the  more  pronounced  degrees  of  defect,  but  as 
development  proceeds  they  become  progressively  more  unreliable  in 
proportion  to  the  increased  psychic  mass,  the  impossibility  of  standard- 
ization because  of  the  wide  individual  differences,  and  the  greater 
probability  of  distortion  from  unknown  emotional  sources. 

INIore  recently  a  definite  attempt  has  been  made  by  Hoch  and 
Amsden^  to  formulate  a  scheme  of  examination,  directed  more  espe- 
cially to  the  affective  aspect  of  the  psyche.  To  use  such  a  scheme  as 
this,  however,  and  in  general  to  gain  any  real  insight  beneath  the 
surface  of  the  psychic  life,  the  technic  of  psychoanalysis  needs  to  be 
used. 

PSYCHOANALYSIS. 

Psychoanalysis  is  the  method  by  which  the  human  mind  is,  so  to 
speak,  dissected,  and  by  means  of  which  the  hidden  motives  of  con- 
duct are  sought.  If  a  ay  thing  like  a  complete  understanding  of  patients 
is  to  be  had  the  methods  of  psychoanalysis  must  be  used.  This  is 
hardly  the  place  to  discuss  these  methods  at  length.  It  would  require 
more  space  than  a  text-book  of  this  character  could  properly  give  it. 
The  student  is  referred  to  special  works.  Here  only  will  be  given  the 
briefest  suggestions.^  (See  also  in  this  connection  the  Introduction 
to  Part  III  and  the  Treatment  of  the  Neuroses.) 

The  Complex. — The  mind  cannot  be  conceived  of  as  consisting  of 
or  containing  ideas  which  are  deposited  here  and  there,  helter-skelter, 
without  order,  as  the  scraps  of  paper  that  are  thrown  carelessly  into 
a  waste  basket.  Quite  the  contrary.  Ideas  are  grouped  about  central 
experiences,  constellated  one  may  say,  built  into  coherent  and  harmo- 
nious structures  not  unlike  the  way  in  which  bricks  and  stones  are 
brought  together  to  form  buildings  and  these  buildings  are  again 
grouped  to  form  the  larger  whole — the  city.  The  significant  fact  in 
this  connection  is  that  the  cement  that  holds  the  bricks  and  stones 
together,  the  binding  substance,  is  feeling. 

This  orderly  arrangement  of  ideas  upon  a  background  of  feeling 
which  serves  to  unite  them  is  what  gives  character,  individuality  to 
the  personality.  The  creating  of  the  proper  feeling-tone  about  things 
and  events  is  one  of  the  main  functions  of  education. 

Now  it  so  happens  that  in  certain  individuals  a  constellation  of 
ideas,  grouped  about  a  central  event  that  conditions  a  highly  painful 
emotional  state,  is  crowded  out  of  clear  consciousness — repressed — into 
the  unconscious  and  so  tends  to  lead  an  existence  which  is  relatively 
independent  and  in  so  doing  gives  origin  to  various  symptoms.  Such 
a  constellation  is  termed  a  "complex"  in  psychoanalysis. 

'  Guide  to  the  Descriptive  Study  of  the  Personality  with  Special  Reference  to  the 
Taking  of  Anamneses  of  Cases  of  Psychoses,  Rev.  of  Neurol,  and  Psych.,  1913,  xi,  577. 

2  Hitschmann:  Freud's  Theories  of  the  Neuroses,  New  York.  Jung:  Theory  of  Psycho- 
analysis, New  York.  Jelliffe:  Technic  of  Psychoanalysis,  Nervous  and  Mental 
Disease  Monograph  Series,  No.  26.  White:  Foundations  of  Character  Formation,- 
Macmillan  &  Co.     Lay:  Man's  Unconscious  Conflict,  Dodd,  Mead  &  Co.,  1917. 


PSYCHOANALYSIS  115 

The  complex,  crowded  out  of  relation  with  the  personal  conscious- 
ness, seeks  for  expression  notwithstanding ;  and  because  it  is  not  sjTithe- 
tized  with  the  rest  of  consciousness,  because  the  individual  is  not  aware 
of  its  existence,  its  expression  cannot  be  controlled  and  guided  into  the 
usual  channels,  and  thus  it  creates  symptoms. 

The  extreme  difficulty  in  locating  and  uncovering  the  complex  is 
due  to  the  symbolic  forms  in  which  it  usually  manifests  itself.  The 
painful  m.emories  of  disagreeable  experiences,  unethical,  unconven- 
tional, and  otherwise  impossible  and  hateful  wishes  while  crowded  out 
of  mind  by  what  Freud  has  so  aptly  termed  the  "  censor  of  conscious- 
ness" nevertheless  struggle  to  find  expression.  The  complex  cries  for 
recognition,  the  censor  will  have  none  of  it — the  fight  is  on,  the  conflict 
wages,  until  finally  a  sort  of  compromise  is  reached  by  permitting  the 
complex  to  come  into  clear  consciousness  but  only  on  pain  of  not  dis- 
closing its  true  self,  that  is,  it  is  permitted  to  appear  under  the  cloak  of 
a  complete  disguise. 

For  example,  Freud's  case  of  Elizabeth.  She  was  engaged  in  nurs- 
ing her  sick  father  who  afterward  died.  One  evening,  spent  away 
from  home  at  the  solicitation  of  the  family,  she  met  a  young  man  of 
whom  she  was  very  fond  and  he  accompanied  her  back  home.  On  the 
walk  home  she  quite  gave  herself  up  to  the  happiness  of  the  occasion 
and  walked  along  oblivious  of  her  duties.  On  reaching  home  she 
found  her  father  much  worse  and  bitterly  reproached  herself  for  for- 
getting him  in  her  own  pleasure.  She  immediately  repressed  this 
disagreeable  thought  from  her  consciousness.  Now  she  had,  each 
morning,  to  change  the  dressings  on  her  father  swollen  leg.  To  do 
this  she  took  his  leg  upon  her  right  thigh.  The  suppressed  complex 
seized  upon  the  feeling  of  weight  and  pain  of  her  father's  leg  upon  her 
thigh  as  a  handy  and  efficient  means  of  expression  and  so  the  repressed 
wish  comes  into  consciousness  under  the  disguise  of  a  painful  area  of 
the  right  thigh  corresponding  in  extent  and  location  to  the  place  upon 
which  she  rested  her  father's  leg. 

This  is  the  sort  of  mechanism  that  accounts  for  many  unusual  and 
strange  experiences  that  otherwise  appear  to  be  without  reason. 
Unexplained  forgetting,  slips  of  the  tongue,  certain  mental  attitudes, 
moods,  and  even  the  dominant  traits  of  character  are  due  to  the 
activity  of  submerged  complexes  while  the  phenomena  of  dreams  are 
explained  in  the  same  way. 

The  unconscious  methods  are  very  logical.  The  complex  often 
expresses  itself  symbolically  (symbolism),  often  by  the  transfer  of 
an  emotion  from  a  painful  event  to  a  less  painful  or  indifferent  event 
(displacement),  often,  as  in  hysteria,  by  the  conversion  of  the  conflict 
into  a  physical  symptom  {conversion).  In  the  phobias,  obsessions 
and  com})ulsive  type  of  disturbance,  the  repressed  aft'ect  undergoes  a 
substitution .  In  this  way  the  symbol  carrier  of  the  repressed  wishes, 
the  symptom,  is  a  most  ingenious  disguise. 

Dreams. — The  analysis  of  dreams  is  for  the  purpose  of  determining 
the  presence  and  nature  of  c()mi)lexes  which  are  exercising  a  controlling 


116     METHODS  OF  EXAMINATION  OF   THE  NERVOUS  SYSTEM 

effect  upon  the  patient's  conduct  and  feelings.  The  dream  appears  as 
a  quite  senseless  experience  to  the  patient  and  upon  the  face  of  it  it 
would  appear  also  to  be  senseless.  A  very  little  effort,  however,  will 
show  that  there  is  a  certain  rough  meaning  to  the  dream .  For  example, 
the  scenes  or  tlie  dream  will  be  representations,  usually  more  or  less 
fragmentary,  of  things  which  have  happened  in  the  life  of  the  individ- 
ual diu-ing  the  previous  twenty-four  hours  and  may  easily  be  associated 
in  his  mind  with  events  of  some  moment  to  him.  As  soon  as  this  is 
pointed  out  the  patient  will  acknowledge  it,  if  he  does  not  know  it 
first  himself,  and  will  think  that  the  dream  has  been  explained.  This 
however,  is  only  the  manifest  content  of  the  dream.  Behind  this 
manifest  content  there  lies  a  deeper,  a  more  profound,  a  more  important 
meaning  or  series  of  meanings.  The  superficial  experiences  only  serve 
to  hide  the  latejit  cmitent,  which  contains  the  real  material  of  importance 
in  the  dream. 

The  latent  content  will  at  most  invariably  show  that  the  dream  in 
its  deeper  meanings  is  dealing  with  repressed,  unconscious  material, 
with  material  that  the  patient  is  not  willing  to  acknowledge  to  himself. 
One  object  of  the  dream  is  to  conserve  sleep,  and  to  that  end  it  does 
not  permit  the  real  dream  thoughts  to  become  kno^^^l,  and  so  builds 
up  a  structure  that  upon  its  surface  appears  utterly  without  meaning 
and  nonsensical,  and  therefore  is  hardly  deserving  of  attention.  There- 
fore the  dreamer  usually  passes  the  dream  by  without  any  particular 
notice,  and  in  the  course  of  the  day  it  is  generally  forgotten. 

The  mechanisms  by  which  this  disguise  is  effected  have  already  been 
spoken  of  in  discussing  the  complex.  The  mechanisms  are  those  of 
distortion.  These  mechanisms  of  distortion  are  first  the  mechanism  of 
displacement,  which  effectuates  the  transfer  of  the  principal  affect  from 
the  situation  where  it  belongs  to  some  other  situation.  The  feeling 
of  anxiety,  or  fear,  or  disgust,  if  it  were  retained  attached  to  that 
portion  of  the  dream  to  which  it  is  normally  attached,  would  disclose 
to  the  patient  the  real  thing  about  which  he  was  anxious,  fearful,  or 
disgusted,  but  if  displaced  upon  some  indifferent  part  of  the  dream 
serves  the  purpose  of  distortion.  Overdetermination  is  a  mechanism 
which  secures  the  appearance  in  the  dream  of  a  particular  figure,  for 
example  because  of  its  likeness  not  to  any  one  experience  in  the  patient's 
life,  but  to  many.  All  of  these  experiences,  as  it  were,  focus  in  one 
direction  and  produce  the  result ;  for  example  the  face  of  a  dreamed-of 
person  may  appear  to  be  entirely  unknown  to  the  dreamer,  but  when 
it  is,  so  to  speak,  dissected  it  is  discovered  that  each  portion  of  the 
face,  the  nose,  the  lips,  the  eyes,  the  hair,  each  of  them,  have  well- 
defined  associations  in  the  mind  of  the  dreamer  and  that  they  have 
all  condensed  into  this  composite  to  serve  the  purpose  of  the  dream 
formation.  Symbolism  is  another  mechanism  which  aids  distortion 
so  that  instead  of  things  being  dreamed  of  as  they  are  in  reality,  they 
are  dreamed  of  as  being  represented  symbolically,  and  so  the  dream 
becomes  increasingly  difficult  to  read,  and  to  the  dreamer  quite  unin- 
telligible.    Further,   the  mechanism   of  secondary  elaboration   is  the 


PSYCHOANALYSIS  117 

mechanism  by  which  the  dreamer,  after  he  is  awake,  goes  on,  as  it  were, 
with  the  dream  material  and  explains  to  himself  by  a  process  of  ration- 
alization what  certain  features  of  the  dream  mean,  and  still  further 
distorts  it  and  places  it  beyond  the  pale  of  his  power  to  recognize  it. 

Here  in  the  dream  is  seen  one  of  the  most  exquisite  of  defense  mech- 
anisms of  the  human  being  at  work.  One  is  accustomed  to  think  of  the 
human  animal  biologically  as  defending  itself  from  all  sorts  of  attacks 
upon  its  integrity.  The  various  infections  by  microorganisms  are  met 
by  vital  processes  of  defense  which  are  frequently  fully  successful,  and 
the  whole  theory  of  immunity  is  the  theory  of  a  defense  mechanism  of 
the  human  animal.  The  dream  is  just  such  a  mechanism,  and  if  what 
is  going  on  in  the  mind  of  the  patient  would  be  kno\Mi,  what  the  patient 
is  defending  himself  from,  what  are  the  disintegrating  factors  at  work 
at  the  psychological  level,  the  easiest  access  to  the  knowledge  of  these 
factors  can  be  found  if  the  meanings  of  the  dream  can  be  learned. 
Dream  analysis  is  a  most  important  tool  for  the  unravelling  and 
treatment  of  all  of  the  neuroses  and  psychoneuroses  and  for  the 
understanding  of  the  psychoses. 

Technic. — The  techiiic  of  psychoanalysis  is  an  art  more  successful 
in  some  hands  than  in  others.  However,  the  general  methods  of 
procedure  may  be  briefly  outlined.^ 

In  the  first  instance  the  physician  must  be  fully  imbued  with  a 
profound  belief  that  mental  symptoms  have,  each  and  every  one  of 
them,  a  meaning  and  a  meaning  which  can  be  brought  to  light  and  will 
show  them  to  be  logical  and  understandable  in  each  instance.  He 
must  then  have  patience  to  listen  to  the  story  of  his  patient,  and  not 
only  listen  to  it,  but  listen  to  it  attentively  for  the  purpose  of  trying 
to  find  the  meaning  in  it,  for  the  purpose  of  trying  to  find  out  where 
the  ^'ital  points  are  which  can  be  attacked  to  best  advantage. 

It  is  true  that  the  dream  analysis  is  the  main  avenue  to  the  under- 
standing of  the  unconscious  motives  of  action,  but  all  sorts  of  hints 
may  come  from  other  sources.  For  example  one  of  the  authors  was 
recently  listening  to  the  story  of  a  patient.  In  the  course  of  that  story 
the  patient  misspoke  and  said  quinine  when  he  intended  to  say  calo- 
mel. Realizing  that  a  "slip  of  the  tongue"  of  this  sort  must  have  its 
meaning  and  is  not  an  accidental  occurrence,  because  nothing  mental 
is  accidental;  the  slip  of  the  tongue  was  analyzed  and  led  directly  back 
to  one  of  the  most  important  emotional  events  in  the  life  of  the  patient, 
an  event  which  threw  a  flood  of  light  upon  his  psychoneurosis. 

Nothing  is  too  trivial  to  be  worthy  of  analysis,  nothing  but  may 
throw  light  upon  the  situation.  All  the  little  slips  of  the  tongue, 
forgotten  incidents,  points  at  which  two  recitals  of  an  occurrence  do 
not  agree,  even  witticisms,  are  necessary  to  trace  out  besides  the 
analysis  of  the  dream  life,  and  offer  an  abundance  of  material  in  the 
course  of  the  analysis. 

1  .lelliffe :  Technic  of  Psychoanalysis  (complete  Bibliography) ,  Nervous  and  Mental 
Disease  Monograph  Series  No.  26,  New  York.  Brill:  Psychoanalj'sis :  New  York.  1918: 
Psychoanalytic  Review,  a  quarterly  magazine,  New  York  and  Washington. 


118     METHODS  OF  EXAMINATION  OF  THE  NERVOUS  SYSTEM 

The  method  of  procedure  is  the  method  of  free  association.  Whether 
it  be  the  analysis  of  some  component  of  a  dream  or  of  a  slip  of  the 
tongue,  or  what  not,  the  method  of  free  association  is  the  one  employed. 
The  patient  should  be  alone  with  the  physician.  It  is  practically 
impossible  to  conduct  an  analysis,  at  least  beyond  the  surface,  in  any 
other  wa}\  Under  circumstances  of  quiet  and  freedom  from  interrup- 
tion, as  far  as  possible,  the  different  points  which  are  to  be  analyzed 
are  taken  up.  The  patient  is  instructed  to  take  a  certain  element 
of  the  dream  which  he  has  just  recounted,  for  example,  and  hold  it 
in  his  mind,  and  then  tell  freely  all  of  the  ideas  that  come  to  him. 
He  is  told  to  tell  all  of  the  ideas  without  any  effort  on  his  part  of 
selection,  no  matter  whether  the  ideas  appear  to  him  to  have  any 
relationship  with  the  portion  of  the  dream  that  he  has  been  told  to 
keep  in  mind  or  not,  and  no  matter  whether  they  appear  ridiculous 
or  have  other  qualities  that  incline  him  to  lay  them  aside.  He  must 
tell  them  all  just  as  a  man  might  sit  at  the  window  of  a  railroad  train 
and  jot  dowai,  as  far  as  possible,  everything  that  he  sees  pass  the 
window  as  the  train  speeds  on. 

The  theory  of  this  procedure  is  that  if  the  patient  does  not  direct 
the  thought  in  any  way  every  idea  that  comes  must  of  necessity  have 
some  relation  to  the  event  held  before  the  mind  about  which  enlighten- 
ment is  sought.  This  is  the  method  of  unravelling  the  tangled  net- 
work of  the  mental  life  and  while  it  may  be  supplemented  by  word 
association  or  other  means,  still  it  would  seem  as  experience  increases, 
that  no  other  method  is  needed,  that  this  answers  all  the  purposes. 
It  takes  a  long  time,  as  a  rule,  however,  to  effect  an  analysis — weeks, 
months,  perhaps  longer.  It  must  be  remembered  that  what  has  taken 
years  to  form  cannot  be  unravelled  in  an  hour.^ 

It  will  probably  occur  to  many  to  wonder  how  it  is  that  one  can 
expect  to  find  memories  reaching  back  for  years  sufficiently  well 
preserved  to  be  helpful.  As  a  matter  of  fact  the  memories  of  all 
repressed  experiences  are  perfectly  clear  no  matter  how  old.  The 
explanation  for  this  is  that  being  repressed  they  are  dissociated  from 
the  every-day  events  of  life,  they  are  kept  in  their  original  form,  they 
have  not  been  subjected  to  the  attrition  and  amalgamation  with  the 
intricacies  of  associational  life.  They  do  not  fade  out  by  this  process 
of  absorption  as  do  the  memories  of  indifferent  events,  but  remain 
where  ever  after  they  may  be  brought  to  light  by  analysis  and  used  as 
helps  for  cure. 

It  will  be  seen  from  this  short  description  what  a  far-reaching 
method  this  is.  A  method  of  analysis  from  which  no  event  of  life, 
no  matter  how  apparently  trivial,  is  free.  A  method  that  in  its 
results  lays  bare  not  only  the  immediate  antecedents  and  causes  of  the 
symptoms,  but  the  whole  innermost  life  of  the  patient,  reaching  back 
even  to  the  period  of  early  childhood.     This  of  course  takes  time. 

'Freud:  Interpretation  of  Dreams.  Nichol:  Dreams.  Havelock  Ellis:  The  Meaning 
of  Dreams. 


PSYCHOAXALYSIS  119 

A  case  of  any  complexity  and  difficulty  quite  generally  takes  several 
months,  of  at  least  three  seances  each  week,  to  reach  a  final  result. 

The  object  of  psychoanalysis  is  not  merely  a  dissection  of  the  psyche 
and  the  discovery  of  the  roots  of  the  psychosis  or  neurosis,  as  the  case 
may  be,  but  is  distinctly  therapeutic.  The  physician  tries  to  show  the 
patient  to  himself  as  he  really  is.  The  patient  is  thus  enabled  to  see 
how  his  symptoms  are  the  results  of  hanguig  on  to  infantile  ways  of 
pleasure-seeking,  self-indulgences,  which  are  repulsive  to  his  better  self. 
\Mien  he  has  ^een  this  the  path  is  pointed  along  which  he  must  go 
toward  the  effective  sublimation,  socialization,  of  his  infantile  ten- 
dencies in  activities  that  are  useful  and  which  meet  with  conscious 
approval.  The  object  of  psychoanalysis  then  is  to  liberate  the  psychic 
energy  which  is  bound  up  in  infantile  ways  of  pleasure-seeking  and  set 
it  free  for  sociallv  useful  ends. 


PART  I. 

THE  PHYSICOCHEMICAL  SYSTEMS. 

THE  NEUROLOGY  OF  METABOLISM. 


CHAPTER  11. 

VEGETATR'E  OR  VISCERAL  NEUROLOGY. 

THE  AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS— 
THE  ENDOCRINE  SYSTEM. 

Two  characteristic  types  of  nervous  system  structures  are  found 
in  all  higher  animals,  namely,  a  vegetative  and  a  sensorimotor  system. 
Anatomically  they  are  often  combined  in  close  and  complex  inter- 
relationships; functionally  they  serve  quite  different  individual  ends 
though  integrated  in  the  conduct  of  the  animal  as  a  whole.  The  vege- 
tative nervous  system  relates  the  various  organs  of  the  body  for  the 
purpose  of  the  upkeep  of  the  machine.  The  activities  of  the  vegetative 
nervous  system  render  the  satisfaction  of  organic  bodily  needs  possible 
and  thus  may  be  said  to  build  up  the  affective  or  emotional  attitudes 
of  the  various  types  of  well-being  in  separate  segments  of  the  body. 

The  sensorimotor  system  has  developed  as  an  effector  mechanism 
to  satisfy  the  needs  registered,  conscious  as  well  as  unconscious,  by  the 
vegetative  system.  Its  general  functions  and  their  special  distiu-b- 
ances  are  taken  up  in  later  sections  of  this  work. 

This  section  would  treat  of  the  structure  and  the  functions  of  the 
vegetative  nervous  system.  A  number  of  differing  terms  have  been 
used  for  this  system.  The  oldest  was  the  SA,Tnpathetic  Nervous  System. 
It  is  called  the  Autonomic  Nervous  System,  also  the  Neurovisceral 
System.  The  name  is  more  or  less  immaterial  if  the  work  done  by  it 
be  kept  in  mind. 

A  thoroughly  consistent  plotting  of  this  enormous  chapter  in 
neurology  is  not  yet  possible.  All  of  the  disorders  here  brought 
together  may  not  be  found  ultimately  to  be  best  grouped  here.  The 
general  assumption  followed,  however,  is  that  they  all  belong  to  dis- 
orders of  a  particular  group  of  organs,  partly  nervous,  partly  glandular, 
the  proper  functionings  of  which  in  their  complex  regulation  and 
coordination  of  metabolic  activities  are  governed  by  a  homogeneous 
series  of  structures,  the  vegetative  nervous  system. 

(121) 


122  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

Certain  of  these  organs  are  closely  related  to  ner\()us  structures, 
hypophysis  (anterior,  and  pituitary,  posterior  lobes),  epiphysis, 
thyroids,  parathyroids,  suprarenals,  and  blood  glands,  and  have  been 
^'ariously  brought  together  as  the  endocrinous  glands  or  glands  of 
internal  secretions — while  others  are  distinctly  non-nervous,  liver, 
pancreas,  testicles,  interstitial  bodies,  th^^nius,  ovaries,  uterus,  lungs 
stomach,  heart,  etc.,  but  their  functions,  like  those  of  the  endocrinous 
glands,  are  automatically  controlled  and  interrelated  by  one  or  other 
of  the  two  portions  of  the  vegetative  system,  the  sympathetic  proper, 
and  the  parasympathetic  or  autonomic} 

The  vegetative  nervous  system  consists  of  those  nervous  structures 
which  supply,  by  afferent  and  efferent  pathways,  impulses  to  the  special 
sense  organs,  smooth  muscle  fibers,  and  all  those  automatically  working 
organs  such  as  the  heart,  lungs,  intestines,  genital  apparatus,  blood- 
vessels, excretory  glands,  skin  and  organs  of  external  and  internal 
secretion,  such  as  the  liver,  stomach,  pancreas,  intestinal  gland,  and 
the  thyroid,  thymus,  adrenals,  parathjToid,  h}"pophysis  and  epiphysis, 
etc.,  respectively. 

All  of  these  structures  are  constantly  in  function,  and  their  dis- 
turbances are  manifold.  They  result  from  (1)  affect  stimuli  such  as 
fear,  rage,  jealousy,  pain,  as  seen  in  many  neuroses  and  psychoneuroses 
represented  as  palpitation  of  the  heart,  anorexia,  fainting,  crying, 
diarrhea,  mydriasis,  eosinophilia,  etc.,  or  (2)  from  infections  or  toxe- 
mias, causing  reactions  such  as  inflammation,  swelling,  gooseflesh,  tachy- 
cardia, dryness  of  the  mouth,  stenocardia,  gastric  or  visceral  crises, 
Argyll-Robertson  pupil,  etc.,  or  (3)  from  constitutional  defect  states 
showing  such  syndromes  as  mjTfedema,  acromegaly,  scleroderma, 
dwarfism,  mongolism,  eunuchoidism,  myopathies,  disordered  blood 
states,  etc. 

It  has  required  a  long  time  for  orthodox  medicine  to  accept  w^hat 
has  been  known  empirically  for  centuries  that  emotional  factors  are 
capable  of  producing  acute  as  well  as  chronic  structural  alterations 
(so-called  organic  disease)  as  w^ell  as  being  constantly  operative  in 
causing  so-called,  and  badly  so-called,  functional  disease.  The  study 
of  the  vegetative  system  has  enabled  this  gap  in  knowledge  to  be 
bridged  by  showing  the  exact  mechanisms  by  which  these  structures, 
cooperating  with  certain  of  the  endocrinous  glands,  may  produce 
pathological  conditions. 

The  role  of  this  system  in  its  reactions  to  mental  stimuli  has  bee  a 
studied  recently  and  for  a  period  of  years  by  Pawlow,  Cannon  and 
others  by  experimental  methods  and  has  helped  to  give  an  interpretative 
status  for  empirically  held  beliefs.     Starting  with  the  well-known  fact 

'  See  Higier, Vegetative  Neurology,  Tr.  by  Kraus,  Nei'vous  and  Mental  Disease  Mono- 
graph Series,  1919,  No.  27.  Eppinger  and  Hessf  Vagotonia,  Nervous  and  Mental 
Disease  Monograph,  No.  20,  New  York,  1915.  Gaskell:  Involuntary  Nervous  System, 
1916.  Noel  Paton:  Nervous  Regulators  of  Metabolism,  1913.  Giovanni:  Patologia 
del  Simpatico,  1876.  Castellino  e  Pende:  Patologia  del  Simpatico,  1915.  Pottenger: 
Symptoms  of  Visceral  Disease  and  Their  Relation  to  the  Vegetative  Nervous  System, 
1919. 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     123 

that  the  vegetative  nervous  system  takes  its  origin  from  the  cranial- 
cervical,  thoracic-lumbar  and  sacral  regions  of  the  cerebrospinal  axis, 
these  studies  attempt  to  correlate  the  activity  of  these  portions  of 
the  system  with  differing  types  of  emotions.  As  a  result  of  physio- 
logical experiment  whenever  the  innervation  from  the  thoracic-limibar 
(sympathetic)  portion  of  the  system  meets,  in  a  viscus,  the  innerva- 
tion from  either  the  cranial-cervical  or  the  sacral  portion,  there  is 
always  an  opposition  in  the  effects  produced.  In  the  eye,  the  contrac- 
tion of  the  pupil  is  due  to  impulses  received  from  the  cranial  auto- 
nomic, while  the  dilatation  of  the  pupil  is  effected  by  nerve  fibers 
w^hich  come  from  the  thoracic  portion  of  the  SATnpathetic.  Again,  the 
secretory  and  tonic  motor  innervation  of  the  stomach  come  through 
the  vagus  nerve  from  sympathetic  neurons  which  have  their  origin  in 
the  cervical  division  of  that  system.  Stimulation  of  the  splanchnic 
nerves  causes  an  inhibition  of  both  the  secretory  and  motor  functions 
of  the  stomach.  The  viscera  of  the  pelvis  are  likewise  doubly  inner- 
vated. The  s^Tnpathetic  neurons  from  the  thoracic-lumbar  segment 
cause  relaxation  of  the  lower  end  of  the  intestine.  The  sacral  autonomic 
fibers  for  the  same  organ  cause  contraction.  The  bladder  and  reproduc- 
tive organs  are  similarly  innervated.  Corresponding  to  the  three  parts 
of  the  vegetative  system  and  the  organs  associated  with  them,  accord- 
ing to  Cannon,  there  are  three  t^'pes  of  response  to  emotions.  The 
cranial  portion  of  the  system,  by  regulating  the  secretion  and  motion 
of  the  digestive  organs,  is  concerned  with  building  up  the  reserves  of 
bodily  strength.  The  emotions  correlated  with  these  physiological 
activities  are  bodily  satisfaction  and  well-being.  The  sympathetic 
system  is  concerned  especially  with  the  functions  of  the  suprarenal 
gland.  The  physiological  effects  of  the  secretion  of  this  gland  are  iden- 
tical with  the  effects  of  stimulating  the  thoracic-lumbar  sjTnpathetic 
system;  the  pupil  is  dilated,  the  heart  is  accelerated,  the  functions  of 
the  stomach  are  inhibited,  and  the  glands  of  the  skin  and  the  erector 
muscles  of  the  hair  are  excited.  Glycogen  in  the  liver  is  liberated  in 
the  form  of  dextrose,  with  the  result  that  sugar  is  available  for  muscles. 
The  emotions  which  call  for  a  sudden  summoning  of  muscular  energy 
are  rage  and  fear,  since  correlated  with  them  are  the  instincts  of 
fighting  and  flight.  The  functions  of  the  pelvic  viscera  are  mainly  in 
the  nature  of  emptying  accumulated  secretions.  The  emotions  depend- 
ing on  these  functions  are  those  of  satiety  and  repletion.  Cannon's 
very  crude  classification  of  the  emotional  reactions  is  hardly  to  be 
accepted,  but  the  underlying  facts  concerning  the  interrelationship 
between  psychogenic  (symbolic),  i.  e.,  emotional  factors  and  neuronic 
mechanistic  alterations  of  physical  structures  are  incontrovertible.^ 

The  vegetative  nervous  system  is  essentially  the  primitive,  archaic 
])ortion  of  the  ganglionic  or  metamefic  system  of  the  lower  verte- 

•  Cannon:  Fear,  Rage,  Hunger  and  Pain  and  the  Nervous  System,  Appleton  &  Co., 
New  York.  For  a  luller  and  more  penetrating  study  of  this  problem  consult  Kempf. 
The  Autonomic  Functions  and  the  Personality.  Nervous  and  Mental  Disease  Mono- 
graph Series,  1918,  No.  28.    Higier:  Vegetative  Neurology,  Ibid.,  1919,  No.  27. 


124  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

brates.  Its  chief  central  switchboard  is  in  the  midbrain.  In  the 
evohition  of  liigher  animals  its  prominence  has  been  submerged,  as  it 
were,  by  the  relatively  more  important  {i.  e.,  for  purposes  of  civilization 
and  culture)  neopallium  or  cortex,  with  its  rich  cortical-association 
system,  but  with  which  it  has  remained  in  close  relationship,  since 
these  structures  underlying  consciousness  and  intelligence  have  grown 
out  of  the  phylogenetically  older  systems.  Thus  it  comes  about  that 
the  ganglionic  system  which  in  man  serves  the  vegetative  functions  of 
the  body  is  represented  in  the  primary  metameres,  the  spinal  cord, 
again  in  the  brain-stem,  central  gray  matter  and  midbrain,  lenticular 
nucleus  and  optic  thalamus  (hypothalamus),  and  finally  in  the  cortex 
where  the  different  organs  under  vegetative  control  have  localization 
as  surely  as  those  of  the  bodily  musculature.  As  yet  the  cortical 
areas  for  the  viscera  are  but  vaguely  guessed  at  but  the  brutal  vivi- 
sections of  the  great  war,  when  more  carefully  analyzed  have,  already 
done  much  to  make  the  cortical  stations  of  the  visceral  reflex  arcs 
more  accurately  localized.  There  are  cortical  representatives  for  every 
structure  in  the  body  probably. 

The  conceptions  of  anatomists  concerning  the  vegetative  system  are 
rapidly  developing.  Their  detailed  consideration  cannot  advanta- 
geously be  followed  here.^ 

Vegetative  Receptors. — These  may  be  distinct  from  those  utilized  by, 
the  sensorimotor  system-  or  the  same  receptor  structure — probably  a 
compound  receptor — may  be  utilized  for  different  types  of  stimuli. 

Langley  has  stated  that  the  vegetative  system  is  not  a  reflex  arc 
system,  in  the  sense  that  has  been  shown  for  the  sensorimotor  reflex 
arc  pathways.  This  is  largely  based  on  the  conception  that  the  incom- 
ing pathways  are  identical,  that  is  the  sensory  neuron  of  the  myelinated 
tj^De  serves  for  both  arcs.  This  we  believe  to  be  a  misconception. 
The  conception  here  advanced  is  that  many  receptors  are  compound. 
They  function  for  differing  qualities  of  stimuli.  The  vegetative  affer- 
ent path  is  certainly  partly,  if  not  chiefly  unmy  elinated,  and  has  been 
neglected  in  almost  all  physiological  and  pathological  interpretations. 
Langley 's  principle  if  applied  too  rigidly,  would  eliminate  the  sensori- 
motor reflex  arc  system,  since  the  vegetative  arc  is  probably  more 
primary,  but  this  is  probably  quite  absurd.  Hence  a  double  system 
is  functionally  active,  one  chiefly  utilized  in  the  upkeep  of  the  machinery 
itself,  the  other  serving  for  an  effector  mechanism  to  keep  the  organs 
and  the  individual  as  a  whole  going.  Many  vegetative  receptors,  lie 
within  the  organs  themselves,  many  of  which  are  being  discovered  by 
newer  technical  microscopical  methods,  and  unmyelinated  and  mye- 
linated fibers  carry  the  primary  cosmic  energy  stimuli,  chemical  or 
physical,  from  the  recipient  structure  to  be  transformed  and  recom- 
bined  for  the  effector  purposes  of  the  body,  growth,  or  conduct  as  the 
case  may  be. 

'  See  Hanson's  many  papers,  particularly  Jour.  Comp.  Neurol.,  August  15,  1919,  for 
a  new  systematization. 

2  Carpenter:  Nerve  Endings  of  Sensory  Type  in  Muscular  Coat  of  the  Stomach, 
Jour.  Comp.  Neurol.,  October  15,  1918. 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     125 

Spinal  Synapses. — These  are  chiefly  located  in  the  lateral  gray  of  the 
spinal  cord  (Jacobsohn)  (Figs.  43,  44,  45).  Jacobsohn^  has  gone  over 
the  old  work  by  examining  a  complete  set  of  serial  sections  from  a 
human  spinal  cord  stained  by  the  Xissl  method.    INIalone^  has  carried 


Spinal  Gan:;iion 


Skin  Sensibility 

Vessels 
Sweat  Gland 

Pilvmotor      


Fig.  42. — ^Diagram  of  the  central  connections  and  peripheral  distribution  of  the  vegeta- 
tive system, ;  the  motor, ,  and  the  sensory, ,  fibers.     (Higier.) 

these  studies  up  into  the  midbrain.    According  to  Jacobsohn,  there  are 
two  columns  of  vegetative  cells  in  the  spinal  cord  (Fig.  43). 


vi-,-,,   f/ucleus   Sympath 
>a^_^«^  lateralis    Super 


Nuclu/i  Synpaffr 
lateralis  inler 


XZHL 


S.  IZ. 


Fig.   4.3. — .Sympathetic  nuclei  at  the  seventh  dorsal  and  fourth  sacral  levels  of  the  spinal 
cord.     (Timnic,  .Jour.  Nerv.  and  Mciit.  Dis.  frrm  .Jacobsohn.) 


I.  The  lateral  cell-column  is  c'omi)o.se(i  of  two  i)arts:  (a)  An  up])er 
column  corresponding  t(^  Langley's  "Sympathetic  System."  This 
lies  in  the  lateral  horn  of  the  dorso-lumbar  cord  {C^ — L3)  and  is  desig- 


'  .Jacobsohn:     Ueber   die    Kerne   des  inen.schlicheii    Riickcnmarks,    1908. 
Kerne  des  nieiischlichen  Hirnstamnies,  1919. 

2  Malone:     I'cbcr  die  Kerne  des  nienschlichen  Diencephalon,  1910. 


I'elicr   die 


126 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


nated  the  Nucleus  s^inpatheticus  lateralis  superior  s.  cornu  laterale. 
(b)  A  lower  column  lying  in  the  sacral  cord,  from  S2  caudalward. 
It  is  placed  between  the  anterior  and  posterior  horns  and  is  designated 
the  Nucleus  s^nnpatheticus  lateralis  inferior  s.  sacralis. 

The  dorsolumbar  column  is  thickest  at  the  upper  dorsal  segments 
and  at  the  upper  lumbar  segments,  that  is  near  the  cervical  and 
lumbar  enlargements  where  there  are  collections  of  ganglion  cells 
for  the  extremities. 


j>£e. 


m.G. 


Fig.  44. — The  reflex  path^  in  the  cord.  (Gaskell.)  A,  of  the  sensorimotor  volun- 
tary system.  The  receptor  neurons  run  in  the  posterior  root,  their  cells  lying  in  the 
posterior  root  ganglia,  P.E.G.  The  connector  synapse  neurons  lie  within  the  dorsal 
horn,  D.H.,  and  make  with  the  effector  neurons  lying  in  the  ventral  horns,  V.H.,  a 
synapse  with  the  motor  neuron,  which  emerges  from  the  cord  as  the  motor  spinal  nerve. 
B,  of  the  vegetative  nervous  system.  The  receptor  neurons  run  in  the  posterior  root 
as  a  sensory  neuron,  making  its  first  synapse  in  the  cells  of  the  lateral  horn,  L.H.  (Jacob- 
sohn's  sympathetic  nuclei.  Fig.  43).  The  connector  neuron  runs  out  with  the  motor 
nerve  as  the  white  ramus  communicans,  making  its  synapse  with  the  effector  neuron 
in  the  sympathetic  ganglia,  Sy.G.  The  effector  neuron  runs  in  gray  ramus  communicans 
to  the  viscus  directly  or  through  intercalated  connector  neurons,  making  a  final  synapse 
within  the  viscus  it.self  (Fig.  46). 


II.  The  medial,  cell  column  lies  in  the  medio- ventral  marginal 
zone  of  the  anterior  horn  of  the  lumbosacral  cord,  from  L4  distal- 
ward  and  is  designated  the  Nucleus  sympatheticus  .medialis  s.  lumbo- 
sacralis.  Low  down  in  conjunction  with  the  Nucleus  radialis,  it 
forms  an  area  of  groups  of  cells  which  takes  up  almost  the  entire 
anterior  horn  and  the  space  between. 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     127 

All  the  cells  of  these  three  columns  have  the  following  three  char- 
acteristics: (1)  They  are  always  in  groups  and  closely  packed  together. 
(2)  They  are  long,  round,  club-shaped  or  vesicular,  rather  small, 
round  cells.  (3)  They  have  a  homogeneous  appearance  and  are  usually 
stained  more  darkly  than  the  larger,  less  closely  packed  motor  and 
sensory  cells. 

A  cross-section  of  the  medulla,  at  a  level  in  which  the  most  important 
cranial  nerves  are  placed,  shows,  in  addition  to  the  large  multipolar 
motor  cells,  small  circumscribed  groups  of  oval  or  pear-shaped  unipolar 
cells  (paracentral  cells).  These  are  the  nuclei  from  which  the  pre- 
ganglionic rami  communicantes  spring.  The  nuclei  are  as  follows 
(see  Plate  III) : 

1.  Nucleus  pupillaris  (Bernheimer) — median  to  the  oculomotor 
nucleus. 

2.  Nucleus  lacrlmalis — median  to  the  facial  nucleus. 

3.  Nucleus  salivatorius  superior  (Kohnstamm) — ^dorsal  to  the  facial 
nucleus. 

4.  Nucleus  salivatorius  inferior  (Kohnstamm) — near  the  glosso- 
phar\Tigeal  nucleus. 

5.  Nucleus  dorsalis  vagi — between  the  motor  and  sensory  vagus 
nuclei,  i.  e.,  between  the  nucleus  ambiguus  and  the  nucleus  solitarius 
vagi. 

A  closer  analysis  of  the  anatomical  position  of  the  various  vege- 
tative nuclei  shows  that  their  relation  to  sensory  and  motor  nerves 
is  the  same  as  in  the  spinal  cord. 

As  a  paradigm  Higier  takes  the  most  orally  placed  ganglion — 
the  ciliary.  It  is  of  great  clinical. significance.  For  years  well  known 
authors  have  spoken  of  this  ganglion  as  a  spinal  ganglion  or  a  mixed 
ganglion  (Schwalbe,  Budge,  Remak,  His,  Gehuchten,  Kolliker,  Bach). 
A  cross-section  of  the  brain-stem  shows  the  following:  the  oculomotor 
nerve  is  the  anterior  motor  root,  the  trigeminal  nerve  is  the  posterior 
sensory  nerve,  the  Gasserian  ganglion  corresponding  to  a  spinal  gang- 
lion while  the  ciliary  ganglion  is  the  vegetative  ganglion. 

The  white  rami  go  from  the  visceral  nuclei  by  way  of  their  corre- 
sponding motor  nerves.  L.  Miiller  and  Dahl  have  tried  to  establish 
this  on  a  firm  basis. 

Afferent  and  efferent  may  be  differentiated  in  the  cranial  ganglia 
as  well  as  in  those  of  the  sympathetic  chain.  The  white  rami  com- 
municantes pass  by  way  of  the  anterior  motor  roots  in  the  cranial  as 
well  as  in  the  spinal  region.  Many  rami  albi  spring  from  cranial  nerves 
and  haxe  been  anatomically  described  though  the  part  they  play  was 
not  even  thought  of. 

1.  Radix  motorica,  or  R.  albus  ganglii  ciliaris — from  the  oculomotor. 

2.  Nervus  petrosus  superficialis  major,  or  R.  albus  ganglii  spheno- 
palatini — from  the  facial. 

3.  Nervus  t\inpanicus  and  its  process  going  to  the  otic  ganglion — 
nervus  petrosus  superficialis  minor,  or  R.  albus  ganglii  otici — from 
the  motor  part  of  the  glossopharyngeal  nerve. . 


128  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

4.  The  chorda  t;s'Tnpani,  which  sends  fibers  as  the  R.  albus  to  the 
submaxillary  ganghon — from  the  motor  nervus  intermedins. 

The  post-cellular  tracts  of  the  cranial  ganglia  are  unmyelinated  like 
those  of  the  s\inpathetic  chain.  They  supply  smooth  muscle  and 
glands  exclusively.  When  they  have  a  long  path  to  follow  to  reach 
the  organs  which  they  innervate,  they  join  spinal  sensory  nerves. 

After  making  the  first  synaptic  junctions,  where  distributions  to 
different  levels  may  take  place,  the  stimuli,  which  are  to  serve  the 
vegetative  functions  travel  chiefly  by  way  of  the  white  ramus — 
ramus  communicantes  albus  to  a  second  synaptic  junction.  The 
collection  of  nerve  structures  making  up  this  synaptic  junction  are 
termed,  for  the  most  part,  the  sympathetic  ganglia  (see  Figs.  44,  45, 
46,  47).  A  further  redistribution  of  the  stimuli  takes  place  at  this  level 
from  which  the  stimuli  travel  by  means  of  the  ramus  communicantes 
griseus  (mixed  with  motor  fibers  of  the  sensorimotor  system  from  the 
anterior  horn  synapse),  and  find  their  next  synapse  (typically)  in  the 
viscus  of  final  distribution.  Here  the  analogue  of  the  motor  horn  cell 
is  located  and  the  final  eft'ector  vegetative  function,  physical  (contrac- 
tion or  relaxation)  or  chemical  (glandular — anabolic  or  catabolic)  takes 
place. 

This  reflex  arc  (various  types  of  which  are  recognized)  is  shown  in 
the  work  of  Gaskell  to  differ  materially  from  the  ordinary  reflex  arc 
of  the  voluntary  system  as  is  seen  in  the  accompanying  illustrations 
(Figs.  42, 44, 45, 46, 47) .  This  is  the  original  type  for  each  metamere, 
the  symmetry  of  which,  however,  has  been  much  distorted.  This 
distortion  is  shown  as  an  irregular  or  inconstant  localization  of  the 
ganglia,  or  synapses,  or  as  an  inconstancy  of  the  communicating 
branches,  irregular  distribution  of  the  synapses  in  the  cerebrospinal 
axis  or  as  an  incongruity  of  the  embryonal  metameres  with  the  spinal 
and  cranial  segments  (Figs.  45-47). 

The  chief  anatomical  features  concerning  this  process  of  distortion 
in  the  head  region  may  be  recapitulated  as  follows:  In  the  skull  the 
distortion  is  greatest  as  many  metameres  are  anatomical  conglomerates. 
Many  of  the  ganglia  are  modified  intervertebral  ganglia,  such  as  the 
geniculate  and  the  Gasserian;  others  are  compound  ganglia  due  to 
the  amalgamation  of  a  spinal  with  a  sympathetic  ganglion  as  the 
jugular  and  vagus  ganglia;  other  pure  intervertebral  ganglia  are  the 
ciliary,  otic,  sphenopalatine,  submaxillary  and  sublingual  which  supply 
the  smooth  muscles  of  the  eye,  the  vessels,  the  tear,  salivary  and 
mucous  glands  respectively.  Some  of  the  chief  anatomical  features 
for  the  head  ganglia  may  be  seen  in  the  superb  charts  from  Miiller.^ 

The  upper  ganglion,  ganglion  cervicale  supremum,  obtains  its 
precellular  fibers  from  the  last  cervical  (C8)  and  upper  dorsal  (Dl-8) 
segments  and  innervates  the  vessels,  hair  muscles  and  skin  glands  of 
the  head,  the  dilator  pupillse  and  JMiiller's  orbital  muscle.  The  inferior 
cervical  ganglion  with  its  closely  related  stellate  ganglion  derives  its 

'  Higier,  loc.  cit. 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS      129 

pregangliar  fibers  from  the  Dl-5,  and  gives  rise  to  the  accelerator 
nerve  of  the  heart  and  probably  the  vasoconstrictor  fibers  of  the 
pulmonary  vessels. 

The  largest  ganglion  of  the  abdomen,  the  celiac,  has  its  chief  root  in 
the  celiac  plexus  in  the  major  and  minor  splanchnic  nerves,  the  former 
of  which  comes  from  D4-9,  the  latter  from  D 10-12.  As  the  mesenteric 
nerves  they  innervate  the  stomach  glands,  liver,  pancreas,  spleen, 
kidneys,  adrenals,  and  intestinal  glands  as  far  as  the  ascending  colon. 


Fig.  45. — Reflex  paths  in  the  bulbar  region.  (Gaskell.)  A,  the  sensorimotor  sys- 
tem. The  sensory  neuron  runs  in  the  fifth  nerve,  V.,  its  cell  bodies  forming  the  Gasserian 
ganglion,  G.G.  The  connector  neuron  synapse  occurs  in  the  descending  root  of  the 
fifth,  D.S.V.,  and  an  effector  synapse  in  the  twelfth  nerve  nucleus,  N.XIL;  B,  the 
sympathetic  system.  The  receptor  neuron  runs  in  the  tenth  nerve,  X,  the  cell  bodies 
making  up  the  vagus  ganglion,  V.G.,  making  its  connector  sjTiapse  in  the  dorsal  vagus 
root,  D.N.X.  The  connector  fiber  makes  its  synapse  vfith  the  effector  in  the  nucleus 
ambiguus,  N.A.;  C,  the  parasympathetic  or  autonomic  system.  The  receptor  neuron 
runs  in  the  tenth  nerve,  X.  The  first  connector  synapse  forming  the  nucleus  intercalatus 
of  Staderini,  A'./.,  pari,  of  the  dorsal  vagus  synapse  group,  D.N.X.  The  connector 
neuron  runs  out  with  the  vagus,  X.  finally  making  an  effector  synapse  in  some  viscus, 
or  plexus.     Auerbach's  plexus  for  the  intestines,  etc. 

The  inferior  mesenteric  ganglion  receives  its  precellular  fibers  from 
Ll-3  and  sends  its  postganglionic  fibers  to  the  colon  and  as  the  h>"po- 
gastric,  in  part,  to  the  anus,  bladder,  sphincter  of  bladder  and  genitals. 
In  these  abdominal  ganglia,  and  the  plexus  of  Auerbach  and  ^Nleissner, 
intercalated  vegetative  neurons  are  found.  Higier,  loo.  cit.,  to  whose 
valuable  monograph  the  student  is  referred  for  more  detailed  study  of 
the  vegetative  system,  follows  a  group  of  investigators  and  includes 
in  the  s.Mnpathetic  system  various  glands  which  contain  chromaffin 
cells,  that  is  to  say  cells  which  have  a  great  affinity  to  chromium,  and 
on  that  account  take  u])  an  intense  brown  coloration  in  Miiller  potas- 
sium bichromate  solution.  Those  cells  all  develoj)  from  the  sympa- 
thetic aulage,  and  are  therefore  in  very  close  relationship  to  the 
ganglion  cells.  They  are  found  partly  separate,  ])artly  in  small  groups 
in  the  sNTnpathetic  system,  in  the  symi)athetic  ganglia,  or  in  large 
9 


130 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


ne^^'e  networks  about  bloodvessels.    Where  they  are  found  as  indi- 
vidual bodies  they  are  designated  paraganglia. 


Fig.  46. — ^The  vagus  nerve,  V.,  contains  connector  and  effector  neurons  as  far  as 
the  ileocolic  sphincter.  Further  effector  (sympathetic?  and  autonomic)  neurons  lie 
within  the  viscera  themselves.  The  pelvic  nerve,  P.,  contains  connector  (and  effector) 
neurons  for  the  sacral  outflow,  terminal  effector  neurons  lying  within  the  walls  of  the 
large  intestine  and  bladder.  The  vagus  thus  carries  connector  neurons  to  the  motor 
effector  cells  of  the  heart,  H.,  which,  Gaskell  states,  have  to  do  with  the  slow  wave- 
like contractions  only  (?)  found  in  certain  tortoises.  The  vagus  also  carries  connector 
fibers  to  the  effectors  in  the  bronchi,  La.,  and  also  connector  fibers  to  the  effectors  within 
the  walls  of  the  gall-bladder  and  bile  ducts,  L.I.  (vagotonic  icterus),  to  the  walls  of 
the  esophagus,  CE,  the  stomach,  St.,  and  small  intestine,  S.I.  The  pelvic  nerve,  synapses 
in  the  sacral  root,  »S',  S',  S',  carries  connector  fibers  to  the  effectors  in  the  large  intestine 
L.I.,  and  bladder,  B.     (Gaskell.) 


They  are  for  the  most  part  spherical  with  a  connective-tissue  capsule 
and  are  broken  up  by  large  nerves  and  bloodvessels,  between  which 
the  chromaffin  cells  lie  in  unequal  masses. 

Of  the  larger  chromaffin  bodies  the  following  four  may  be  named : 
(1)  the  carotid  paraganglion  incorrectly  spoken  of  as  a  gland  (carotid 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     131 

gland  or  epithelial  organ) .  (2)  The  coccygeal  paraganglion  incorrectly 
spoken  of  as  the  sacral  gland  (coccygeal  gland) .  (3)  Aortic  paraganglion 
at  the  bifucation  of  the  aorta.  (4)  The  best  studied  and  largest 
chromaffin  body,  the  suprarenal  body,  the  medulla  of  the  adrenals, 
from  which  the  active  blood-pressure  raising  adrenalin  is  produced, 
a  substance  which  stimulates  the  s\inpathetic  system,  and  plays  an 
enormously  important  role  in  the  body. 

According  to  Aschoff,  chromaffin  bodies  are  also  to  be  found  in  the 
vicinity  of  or  in  the  paroophoron  and  epididj^mis  which  are  also  organs 
of  internal  secretion. 

The  chrojnaffin,  or  more  properly  speaking  phaochrom  cells  all 
develop  from  the  sympathetic  anlage,  and  are  at  least  closely  related 
to  the  ganglion  cells.  The  assumed  transitions  between  the  two  have 
not  received  general  confirmation,  and,  in  spite  of  the  hypothesis  of 
Diarnera  that  the  chromaffin  cells  are  secretory  epithelial  cells,  H. 
Kohn,  one  of  the  first  describers  of  this  picture,  justly  maintains  the 
propriety  of  not  putting  these  cells  in  any  definite  histological  group 
but  in  a  group  of  their  own. 

These  cells,  which  may  resemble  alike  epithelial  cells,  muscle  cells 
and  nerve  cells,  also  take  their  place,  both  embryologically  and  physio- 
logically as  a  distinct  typ^  very  closely  related  to  the  sympathetic 
cord. 

Midbrain  Synapses  of  the  Vegetative  Pathways. — These  are  still  not 
definitely  charted  but  they  undoubtedly  exist.  Jacobsohn,  jNIalone 
and  others  have  given  the  histological  evidence  pointing  to  wide  dis- 
tributions of  vegetative  cells  in  the  corpora  striata,  the  hypothalamus, 
corpus  Luysii,  the  thalamus  and  other  midbrain  structures.  Experi- 
mentally obtained  facts  gathered  in  recent  years  making  it  probable 
that  the  hypothalamus,  i.  e.,  the  region  lying  between  the  epiphysis 
and  the  hypophysis,  contains  synapses  for  the  vegetative  pathways. 

Destruction  of  the  corpora  mamillaria  causes  polyuria,  of  the  thala- 
mus, hyperthermia,  of  the  lateral  area  of  the  gray  matter  on  the  floor 
of  the  midbrain,  dilatation  of  the  pupil  and  widening  of  the  lid  slit. 
Stimulation  of  the  ventricular  floor  in  the  region  of  the  hv'pophysis 
brings  about  contraction  of  the  bladder,  the  intestines  and  uterus, 
while  stimulation  of  the  tuber  cinereum  produces  dyspnea  and  arrest 
of  the  heart's  action,  followed  by  bradycardia.  Lesions  of  the  floor 
of  the  midbrain  induce  marked  metabolic  and  trophic  changes  in  young 
and  adult  animals  (genital  dysplasia  of  the  ovaries  and  testes,  retarda- 
tion of  growth)  and  puncture  of  the  floor  of  the  third  ventricle  causes 
intense  glycosuria  similar  to  the  Claude  Bernard  puncture  of  the  floor 
of  the  fourth  ventricle.  Simultaneous  section  of  the  distal  ends  of  the 
sympathetic  (NN  splanchnii)  prevents  the  occurrence  of  this  glyco- 
suria of  hj-pothalamic  origin  as  well  as  any  glycosuria  which  may 
be  caused  by  stimulation  proximal  to  the  place  of  section.  Careful 
study  of  the  vegetative  disturbances  in  paralysis  agitans  point  in  the 
same  direction,  while  we  are  disposed  to  see  in  the  extensive  changes 
in.the  lenticular  nucleus,  so  largely  in  evidence  hi  Wilson's  disease,  and 


132  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

related  syndromes  direct  evidence  of  impairment  of  vegetative  path- 
ways from  the  Hver.  What  relationship,  other  than  purely  suggestive 
by  analogy,  the  chromaffin-like  substances  of  the  substantia  nigra 
have  with  other  chromaffin  structures  is  not  known. 

Cortical  Stations  of  the  Vegetative  Pathways. — There  are  many  dis- 
cordant observations  concerning  cortical  stations  in  the  vegetative 
pathways.  Our  conclusion  from  the  many  studies  is  that  there  are 
such,  and  we  are  inclined  to  maintain  that  every  organ  of  the  body  has 
cortical  representation.  The  evidence  cannot  be  discussed  here  very 
fully  as  it  transcends  the  limits  of  a  text-book.  ]\Iuch  light  on  this 
question  will  undoubtedly  be  thrown  upon  the  problem  when  the 
cortical  scleroses  so  abundantly  demonstrated  by  Southard  in  schizo- 
phrenics, for  example,  are  histologically^  correlated  with  the  vegetative 
syndromy,  inclusive  of  the  endocrinopathies,  equally  manifest  in  this 
heterogeneous  group.  Higier  in  treating  this  question  raises  the  follow- 
ing problems:  (I)  the  location  of  the  sympathetic  centers,  i.  e.,  the 
psycho-vegetative  centers  in  the  cortex  analogous  to  psychomotor 
centers,  (II)  the  relationship  of  mental  states  upon  the  tone  of  the 
vegetative  nervous  system,  (III)  emotional  states  and  vegetative 
activities.  Kempf  (loc.  cit.)  has  discussed  this  latter  aspect  very 
exhaustively. 

Have  internal  and  external  visceral  organs  cortical  representation 
like  voluntary  muscles  and  the  sensory  functions  of  the  skin?  Higier 
calls  attention  to  the  relations  of  the  vagus  in  this  connection,  since 
its  anatomical  relations,  its  viscero-bulbo-thalamo-cortical  parts,  have 
been  very  carefully  studied.  The  vagus  is  a  mixed  nerve  having  at 
least  three  nuclei  whose  embryological  development  is  different.  The 
motor  nucleus  ambiguus  corresponds  to  the  anterior  horn,  the  sensory 
nucleus  solitarius  to  the  posterior  horn  and  the  visceral  nucleus  dorsalis 
vagi  to  the  vegetative  nerve  cell  group  which  lies  in  the  lateral  gray 
matter  of  the  spinal  cord.  The  sensory  fibers  supply  the  mucous  mem- 
branes of  several  cervical  and  thoracic  organs — and  they  continue 
upward  through  the  thalamus  to  the  cerebral  cortex.  It  is  also  known 
that  there  are  cortical  synapses  for  the  fibers  to  the  voluntary  muscles  of 
the  pharynx  and  larynx.  These  exist  whether  such  came  at  a  period 
when  these  muscles  became  striated  or  whether  they  existed  when  they 
were  still  involuntary,  unstriated  muscles.  The  phylogenetic  rela- 
tions do  not  alter  the  facts.  A  cortical  representation  of  the  visceral 
part  of  the  vagus,  of  the  cardiac,  bronchial,  pulmonary  and  gastric 
fibers  of  the  vagus  is  not  clearly  demonstrated.  The  same  question  of 
course  arises  in  connection  with  the  other  autonomic  and  sympathetic 
fibers  which  have  to  do  with  the  excretory  and  secretory  organs  of  the 
skin  and  body  cavities.  There  are  now  two  quite  opposite  views  upon 
this  subject.  One  school  is  headed  bv  Bechterew,  the  other  by  Pavlov 
and  L.  Miiller. 

Bechterew 's  school  tried  to  arrive  at  a  conclusion  by  investigating 
the  association  or  conditioned  reflexes.  Association  reflexes  according 
to  this  school  consist  in  a  reaction  which  is  not  determined  by  either 


AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     133 

the  strength  or  the  quahty  of  the  external  stimuhis,  but  by  the  relation 
of  this  stimulus  to  other  reflexes  in  the  past,  i.  e.,  a  relation  that  has 
been  often  repeated  and  whose  repetition  occurs  in  the  same  manner 
as  the  reactions  produced  by  habit.  The  hissing  of  a  snake  produces 
an  associative  reflex  which  manifests  itself  in  an  intense  motor  reaction 
not  because  of  the  loudness  or  softness  of  the  noise,  not  on  account  of 
the  hissing  itself,  but  because  the  hissing  is  associated  with  danger. 
The  presence 'of  a  cat  causes  a  dog  to  run  because  of  past  experiences 
with  the  cat's  claws.  The  smell  and  sight  of  appetizing  food  starts 
gastric  secretion.  Shice  vegetative  functions  are  concerned  with  the 
most  vital  of  body  processes,  processes  which  are  activated  by  simple 
reflexes,  extirpation  of  the  cortex  does  not  interfere  with  their  activity, 
does  not  cause  any  paralysis  as  extirpation  of  the  motor  cortex  supply- 
ing the  extremities.  But  there  does  result  a  great  defect  of  nerve 
activity'  in  so  far  as  associative  reflexes  go. 

If  one  removes  in  the  dog  what  Bechterew  regards  as  the  cortex 
representing  respiratory  activities  and  remove  both  centers,  respira- 
tion proceeds  normally  due  to  the  presence  of  the  spinal,  the  co- 
ordinating medullary  and  the  subcortical  midbrain  centers.  The 
respiratory  reflex  to  artificial  as  well  as  normal  sensory  stimuli  is 
the  same  as  before.  But  the  psychoreflexes,  the  associative  reflexes, 
are  modified.  The  presence  of  a  cat  does  not  cause  the  intense  motor 
effect  upon  respiration  which  occurred  in  the  unoperated  dog  under 
like  circumstances. 

If  sjTiaptic  junctions  of  sex-organ  pathways,  which  Bechterew 
locates  in  the  upper  third  of  the  g}Tis  postcruciatus  be  removed  in  a 
dog,  the  erection  of  the  penis  to  mechanical  stimulation  obtains,  but 
the  psychoreflexes  related  to  the  presence  of  a  bitch  in  heat  are  absent. 
If  the  sjTiapses  of  iris  pathways  in  the  frontal  lobes  be  removed,  certain 
sjTnbols,  as  threatening  with  a  stick  or  whip  are  not  responded  to  by 
dilatation  of  the  pupil.  Thus  Bechterew  has  shown  that  many  sjin- 
bolic  conditioned  reflexes  are  absent  following  cortical  injury,  and 
concluded  that  visceral  tracts,  motor  and  secretory,  have  cortical 
representation.  Pavlov  on  the  contrary  reached  conclusions  which 
are  diametrically  opposed  to  those  of  Bechterew. 

The  enormous  material  now  being  accumulated  from  the  war  is 
tending  to  show  more  and  more  the  validity  of  Bechterew's  conten- 
tions and  the  results  of  histological  examination  of  the  brains  in  severe 
aft'ect  disturbances  in  the  psychoses,  notably  dementia  precox,  paresis, 
and  senile  arteriosclerotic  changes  are,  also  tending  in  the  same  direc- 
tion. The  evidence  derived  from  emotional  disturbances  can  best  be 
obtained  from  Kempf's  monograph. 

In  fact  a  large  number  of  bodily  manifestations  of  psychic  occurrences 
are  so  constant  in  the  vegetative  domain  that  they  have  been  used  as 
precise  measures  "in  psychophysiological  studies.  Thus  the  following 
reactions  have  been  used:  (a)  Measurement  of  the  pupil  (Bumke), 
(6)  measurement  of  the  activity  of  the  salivary  glands  (Pavlov), 
(c)  measurement  of  the  in\oluntar3'  activity  of  the  bladder  (Mosso),  (r/) 


134       ■  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

measurement  of  the  skin  resistance  during  various  degrees  of  sweating 
(Veraguth's  psychogalvanic  reflex  phenomenon),  (e)  measurement 
of  the  activity  of  the  A'asomotors  and  the  heart  (Mosso-Lehmarin 
plethj'smographic  test  of  blood  distribution).  A  large  number  are 
being  added  to  these  with  the  more  intense  study  of  the  endocrino- 
pathies  associated  with  emotional  disturbances.^ 

Sympathetic  and  Autonomic  Divisions. — ^Anatomically  as  well  as 
pharmacologically  it  appears  that  two  types  of  physiolf)gical  activity 
are  present  in  the  vegetative  nervous  system.  These  have  been  termed 
the  sympathetic  and  the  parasympathetic  or  autonomic.  All  those 
non-voluntarily  influenced  organs,  smooth  muscle  structures,  heart 
muscle,  glands,  whose  nerve  fibers  are  related  to  the  spinal  cord 
from  the  first  dorsal  above  to  the  fourth  lumbar  segment  below, 
belong  in  the  sympathetic  system  in  the  narrower  sense.  All  others 
are  controlled  through  the  parasympathetic  or  autonomic.  The  upper- 
most come  from  the  midbrain,  enter  the  ciliary  ganglion,  and  are 
distributed  to  the  smooth  internal  muscles  of  the  eye.  A  second  or 
bulbar  autonomic  system  passes  through  the  facial  and  goes  as  the 
chorda  timpani  to  the  salivary  glands.  The  glossopharyngeus  and 
vagus  belong  to  this  bulbar  autonomic  system.  A  sacral  autonomic 
system  supplies  the  organs  of  the  pelvis  and  genitalia.     (See  Fig.  47.) 

How  this  division  will  prove  out  in  clinical  work  is  yet  to  be  tested, 
but  it  has  become  a  practical  working  hypothesis  to  harmonize  the 
results  of  pharmacological  experimentation.^  This  is  particularly  to 
be  seen  in  the  reactions  of  the  vegetative  nervous  system  to  certain 
products  of  the  endocrinous  glands,  i.  e.,  hormones,  and  to  certain 
toxic  and  anaphylactic  substances,  notably  nicotin.  Inasmuch  as  the 
vagus  constitutes  the  chief  representative  of  the  autonomic  system, 
the  terminology  is  applied  to  it  more  particularly. 

Thus  atropm,  its  related  alkaloids  and  the  nitrates  paralyze  the 
vagus  and  its  end-organs  (vagoparalytic) .  The  former  paralyzes  the 
positive  or  stimulating  element  of  the  autonomic  system,  causing 
mydriasis,  lessened  secretion;  the  latter  a  paralysis  of  the  negative  or 
depressing  element,  gives  rise  to  vasodilatation.  Vasospastic  drugs, 
such  as  muscarin,  pilocarpin,  picrotoxin  and  physostigmin  cause 
lowering  of  blood-pressure,  weakening  of  the  heart  action,  brady- 
cardia, myosis,  mcreased  secretions,  and  increased  peristalsis.  Morphin 
gives  similar  reactions  but  its  actions  are  extremely  complicated.^ 

A  number  of  the  products  of  the  endocrinous  system  act  as  vago- 
tonics; among  these  is  cholin,  from  the  cortex  of  the  suprarenals. 
Cholin's  hormone  autonomic  stimulation  symptoms  are  myosis, 
diminished  peristalsis,  contraction  of  the  uterus,  bladder,  bronchi, 
diminished  cardiac  force  and  rhythm,  pallor  of  skin  with  increased,  cold 
sweat  and  paresis  of  the  abdominal  bloodvessels.  The  sympathetic 
neurons  and  synapses  are  unaffected  by  cholin.     lodothyrin,  hypo- 

1  See  Higier:  Loc.  cit.,  p.  125.    Laignel  Lavastine:  Loc.  cit.    Kempf:  Loc.  cit. 

«  Petren  u.  Thorling:  Ztschr.  f.  innere  Med.,  173.    Bauer,  D.:  Arch.  f.  klin.  Med.,  107. 

2  Kraus:  Jour.  Nerv.  and  Ment.  Dis.,  1918. 


AUTONOMIC   AND  SYMPATHETIC  NERVOUS  SYSTEMS     135 

physin,  and  adrenalin  act  partly  as  sivTnpathicotonic,  partly  as  auto- 
nomic or  vagotonic.     lodothvrin,  or  the  related  hormone  from,  the 


H«t^ 


u^o-vcc: 


btoRacK 


Liver 


r(xt^c<e(xi 


K.>Vatvs 


Pvox  Colon 


CoVo(\Rfftu.ra 


Fig.  47. — Distribution  of  the  various  divisions  of  the  vegetative  system.  Sympathetic 
fibers  are  indicated  by  dotted  lines;  parasympathetic  (autonomic)  by  unbroken  lines. 
(Kraus,  modified  from  Meyer  and  Gottleib,  Jour.  Nervouts  and  Mental  Disease,  1918.) 

thjToid,  acts  upon  both  systems  to  produce  some  of  the  characteristic 
signs  of  exophthalmic  goiter  (see  later) . 


136 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


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AUTONOMIC  AND  SYMPATHETIC  NERVOUS  SYSTEMS     137 

The  active  principle  of  the  hypophysis  acts  upon  both  systems  to 
produce  pictures  of  hypo-  or  hyperpituitarism  or  a  mixture  of  both, 
dyspituitarism.  Adrenalin,  from  the  medullary  portion  of  the  adrenal, 
causes  tachycardia,  increase  of  blood-pressure  from  contraction  of  the 
bloodvessels,  mydriasis  and  exophthalmos,  paresis  and  anemia  of  the 
respiratory  and  stomach  tracts,  increase  of  sphincter  tonus  and  of  the 
secretion  of  certain  glands,  mobilization  of  glycogen,  and  increase  in 
its  oxidation.  The  amount  of  adrenalin  set  free  by  acute,  conscious 
fear  has  been  measured  by  Cannon,  the  effect  of  chronic,  unconscious 
fear  is  undoubtedly  as  forceful.  The  antagonistic  action  of  the  sym- 
pathicotropic adrenalin  and  the  vagotropic  pilocarpin  shows  itself  in 
that  adrenalin  can  counteract  a  pilocarpin  eosinophilia  and  pilocarpin 
an  adrenal  glycosuria.  Other  remarkable  opposing  reactions  are 
known  showing  the  striking  antagonistic  physiological  possibilities  of 
the  vegetative  system. 

The  chief  contrasting  activities  of  these  two  systems  are  here  showoi 
in  tabular  form  as  taken  from  the  studies  of  Frohlich,  Eppinger,  Hess, 
Lowi  and  others  (see  page  136). 

Inasmuch  as  this  system  is  very  markedly  under  psychical  influences, 
particularly  of  the  affects,  its  relations  to  what  is  know^l  as  affectivity 
and  ambivalence  in  psychoanalytic  literature  is  of  far-reaching  impor- 
tance.^ The  vagotonic  and  sympathicotonic  types  as  described  by 
Eppinger  and  Hess  have  already  been  touched  upon  in  the  chapter  on 
Examination  of  the  ^  egetative  Nervous  System.  It  is  important  to 
bear  in  mind  that  these  are  reaction  trends  rather  than  clear-cut  types. 

No  psychic  experience  of  any  kind,  joy,  sorrow  or  any  other  can 
take  place  without  there  being  reactions  of  a  definite  natiu"e  in  the 
body.  These  are  mediated  chiefly  through  the  vegetative  system. 
Strange  as  it  may  seem  the  James-Lange  hypothesis  that  emotional 
states  are  peripherally  induced  seems  to  be  clearly  established 
by  Kempf's  recent  studies.  Man  should  be  without  shame 
did  he  not  blush,  and  without  rage  if  the  muscles  did  not  con- 
tract, the  heart  beat  more  rapidly  and  thump  the  breast,  and  if 
we  had  not  all  those  other  changes  in  our  vegetative  organs  which 
accompany  emotional  activity.  "That  the  mind  acts  upon  the 
body  and  the  body  acts  upon  the  mind  in  that  important  sphere  of 
psychic  activity,  the  emotional,  is  quite  clear."  It  is  significant  that 
the  psychoreflexes  manifest  themselves  in  many  ways.  The  different 
emotional  states  have  qualitatively  different  manifestations  in  various 
parts  of  the  body.  That  which  applies  to  sensory  stimuli  and  emo- 
tions also  applies  to  every  mental  act  of  the  individual.  E\ery  psychic 
activity,  every  voluntary  impulse,  every  fixation  of  attention,  every 
stimulating  idea  brings  with  it  a  reaction,  for  all  psychic  activites  are 
accompanied  by  emotional  variations  and  feelings.  jNIan  is  not  only 
governed  by  pure  sensory  stimulation,  but  also  by  higher  intellectual, 
ethical  and  esthetic  feelings  which  are  but  compounded  symbolizations 

'  Kempf:  The  Autcnomic  Functions  and  the  Personality,  loc.  cit. 


138  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

of  simpler  affective  states.  The  continual  minute  oscillations  of  the 
vegetative  nervous  system  bear  witness  that  the  sum  of  stimuli  going 
to  the  central  nervous  system  is  always  varying;  that  the  tone  of  the 
vegetative  tracts  is  always  changing,  that  the  vegetative  balance  is 
never  quite  stationary. 

Special  Pathology. — Eye  Sympathetic. — The  ciliary,  pupillary  sphinc- 
ter and  dilator  muscles,  Miiller's  orbital  muscles,  and  the  tear  glands 
are  all  vegetative  organs  of  the  eye  which  are  mnervated  in  part 
by  autonomic  and  in  part  by  sympathetic  fibers.  The  pupillary 
innervation  is  of  special  moment.  The  synapse  of  the  dilator  sym- 
pathetic fibers  is  the  ciliospinal  center  in  Dl-3.  These  fibers  pass 
through  to  the  superior  cervical  ganglion  where  a  synapse  is  made. 
Here  fibers  pass  to  the  Gasserian  ganglion,  join  with  the  trigemmus 
and  in  the  long  ciliary  nerves,  pass  to  the  vessels,  dilator  pupillse 
and  to  Miiller's  muscle,  which  pushes  the  eyeball  forward.  An  auto- 
nomic pathway  (sphincter)  passes  by  means  of  the  oculomotorious 
and  ciliary  ganglion.  Connections  with  the  cerebrospinal  axis  are 
many.  The  central  course  of  the  precellular  fibers  of  the  smooth  ciliary 
muscle  of  accommodation  of  the  iris  sphincter  is  not  certainly  fixed, 
although  the  evidence  points  to  a  midbrain  synapse  in  the  anterior 
median  nucleus  of  the  oculomotor.     (See  Oculomotor.) 

Several  important  clinical  conditions  depend  upon  the  complicated 
pupillary  innervation,  the  chief  of  which  are:  (1)  absolutely  stiff 
pupils,  (2)  Argyll-Robertson  pupil,  (3)  sympathetic  paralysis,  (4) 
variations  and  deformities  in  pupils. 

1.  In  the  first  all  automatic  stimuli  to  the  iris  muscle  are  inoperative 
with  the  exception  of  the  sympathetic,  which  of  itself  has  a  minimal 
action.  The  pupils  are  dilated  and  distorted.  Since  the  ciliary 
gangion  serves  for  autonomic  tonic  activity,  any  disturbance  of  the 
ganglion  gives  rise  to  great  dilatation,  which  is  more  marked  than  is 
produced  by  nuclear  or  peripheral  lesions  of  the  oculomotor.  Absolute 
rigidity  is  seen  particularly  in  fainting,  high  grades  of  anxiety  or  fear, 
frequently  in  hysteria,  in  most  epileptic  convulsive  attacks,  and^in 
central  cerebrospinal  syphilis.  Pupillary  inequalities  are  frequent  in 
the  psychoneuroses  and  such  anomalies  have  special  significance  in  the 
psychoanalytic  study  of  the  repressions  of  unconscious  material. 

2.  The  Argyll-Robertson  pupil  is  a  complicated  phenomenon.  It 
has  already  been  described.  (See  Examination.)  It  is  an  extremely 
common  sign  in  cerebral  syphilis,  as  seen  in  tabes  and  paresis  par- 
ticularly. It  is  occasionally  found  in  extreme  alcoholism  (Korsakov 
particularly),  and  occurs  from  rare  and  isolated  lesions  of  the  corpora 
quadrigemina.  In  apes,  Karplus  and  Kreidl  have  shown  that  a 
severance  of  commissural  association  fibers  passing  in  the  arm  of  the 
anterior  corpora  quadrigemina  to  the  anterolateral  border  of  the 
anterior  corpus  will  cause  a  bilateral  reflex  pupillary  rigidity  with 
retention  of  pupillary  activity  for  accomodation,  convergence  and 
psychical  stimuli.  Chronic  meningeal  exudates  in  syphilis  pressing 
upon  these  fibers  may  account  for  the  frequency  of  this  symptom  in 


SPECIAL  PATHOLOGY  OF  EYE 


139 


tabes  and  paresis.  Explanations  are  numerous,  however,  and  may  be 
consulted  in  the  literature.  (Willbrand  and  Saenger,  Die  NeAirologie 
des  Auges,  see  citations  in  Examination  section.) 

3.  S\Tnpathetic  paralysis,  or  Horner's  syndrome,  is  characterized 
by  retraction  of  the  bulb,  narrowing  of  the  palpebral  fissure,  dropping 
of  the  upper  and  raising  of  the  lower  lid  and  myosis,  with  conservation 
of  the  psychical  and  light  reflexes  of  the  pupil. 


Medulla 


Citio-sjiinul  Centre- 


To  non-xtriitted  ixtrt  of  ^ 
Lev.  I'dli'.  iui>. 


W     ^.;»^Sweat 
f  y^^"^  Va»o- 


Upper  Lid 


Dilator  Pupillce 
Lonii  Ciliary  Xerves 

Gasserian  Ganylion 

uscle  of  M'uller 
(non-striated) 
To  Hyiwalossal  Xerve 
Sweat  Fibres 
motor  £ 
I  a  tor  Fibres 


Middle  Cerrical  Guiiulion 


Inferior  Cervical  Ganulion- 


Fig.  48. — Diagram  of  course  of  oculopupillary  fibers  of  cervical  sympathetic.     (Stewart.) 


Topographically  the  picture  results  from  pressure  on  the  sympa- 
thetic fibers  (goiter),  a  lesion  of  the  cervicodorsal  cord  (hematomyelia), 
gliosis  (syringoencephalomyelia),  myelitis,  especially  of  the  upper 
dorsal  region  (Dejerine-Klumpke,  Budge's  centers),  thrombosis  of 
the  posterior  inferior  cerebellar  artery,  cervicodorsal  radiculitis,  and 
in  certain  hysterical  conversions,  compulsive  tics,  and  psychotic 
projections. 

4.  Combinations  of  the  three  just  recorded. 

Tests  with  cocain  and  adrenalin  are  of  value  in  determining  the 
sympathicotropic  activity,  a  2  per  cent,  cocain  solution  stimulating  the 


140 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


dilator  fibers.  A  failure  to  cause  mydriasis  is  evidence  of  weakness 
of  the  sympathetic.  ^Yhere  such  a  paresis-producing  lesion  may  lie, 
pre-  or  postganglionic,  above  or  below  the  superior  cervical  ganglion, 
can  be  determined  by  the  use  of  a  1  per  cent,  solution  of  the  sympathico- 
tonic hormone  adrenalin.  Six  drops  in  five  minutes  normally  causes 
no  action.  If  after  fifteen  minutes,  however,  there  is  a  marked  dilata- 
tion the  lesion  is  postganglionic.  Adrenalin  mydriasis  is  frequently 
present  in  anterior  and  middle  fossa  disturbances  (orbital  disease, 
fractm*e  of  base).  This  is  through  the  activity  of  the  sympathetic 
fibers  of  the  carotid  plexus  which  joins  with  the  trigeminus  at  the 
Gasserian  ganglion.  Thus  a  combination  of  disturbances  of  the  supra- 
orbital, with  adrenalin  mydrias's  due  to  postganglionic  sympathetic 


Fig.   49. — Partial  Bernard-Horuer's  .syndrome  uf  riglu  eye  in  exophthalmic  goiter. 


paralysis,  may  give  important  evidence  as  to  the  localization  of 
a  tumor,  or  fracture  of  the  base  of  the  skull.  Double-sided  adrenalin 
mydriasis  (Lowi's  reaction)  is  also  seen  in  hyperthyroidism,  pancreatic 
diabetes,  and  in  increased  irritability  of  the  sympathetic  nervous 
system  in  general.  In  psychoses,  particularly  in  dementia  precox,  the 
reaction  is  very  variable,  and  the  test  is  complicated  by  a  variety  of 
emotional  reactions. 

Vagotonic  reactions  give  rise  to  accommodation  cramps  with 
lachrymation  which  may  be  diminished  by  atropin.  In  youth,  when 
vagotonia  is  more  pronounced,  atropin  acts  less  protractedly^  than  in 
older  people,  and  pilocarpin  in  the  eye  may  cause  von  Graefe's  sj'mptom 
as  a  sign  of  an  increase  in  the  tonus  of  the  autonomic  levator  palpebrse. 

There  are  a  number  of  eye  affections  whose  pathogeny  is  in  part 


SPECIAL  PATHOLOGY  OF  MOUTH  141 

dependent  upon  vegetative  nerve  disturbance.  Only  a  few  of  the 
more  important  can  be  referred  to  here,  and  briefly.^ 

Glaucoma. — This  serious  aflFection  of  the  eye,  speaking  of  the  acute 
inflammatory  or  congestive  type,  is  due  to  a  disturbance  in  intra-ocular 
tension  which  is  largely  dependent  upon  sympathetic  control.  The 
precise  mechanisms  are  still  incompletely  analyzed.  The  adminis- 
tration of  atropin  by  its  paralyzing  action  on  autonomic  fibers — 
paralysis  of  sphincter  iridis,  ciliary  muscle — also  produces  marked 
increase  in  intra-ocular  tension,  and  hence  augments  the  difficulties. 
Pilocarpin  and  eserine  (physostigmin),  with  their  opposing  actions 
on  the  sympathetic,  decrease  intra-ocular  tension  and  hence  alleviate 
(temporarily  in  the  early  stages)  the  syndrome.  Excision  of  the 
cervical  sympathetic  ganglion  diminishes  the  tension,  and  is  resorted 
to  in  the  treatment  of  glaucoma,  thus  showing  the  definite  part  played 
by  the  vegetative  nerves  in  this  affection. 

Herpes  corncp,  herpes  ophthalmicus,  keratitis  nevroparalytica  are 
among  the  eye  aft'ections  due  to  implication  of  vegetative  fibers, 
located  chiefly  in  the  trigeminus  sheaths,  or  in  the  Gasserian  ganglion. 
Keratitis  neuroparalytica  occurs  not  infrequently  as  a  complication 
in  resection  of  the  ganglion.  Resection  of  the  cervical  SNTupathetic 
seems  to  cure  this  keratitis  (Cimoroni). 

Acute  edemas  of  the  conjunctiva,  of  the  retina  and  iris,  analogous 
to  Quincke's  edema  are  to  be  classed  probably  with  vegetative  nerve 
disorders.  These  have  largely  been  disguised  under  pseudonyms  as 
"rheumatic,"  or  "lithemic,"  or  "gouty." 

Certain  forms  of  accommodation  disorders  (hypermetropias,  myo- 
pias, spasms,  etc.),  frequently  called  "eye-strains"  are,  properly  speak- 
ing, vegetative  disorders  of  the  ciliary  muscle.  A  few  of  them  are 
largely  psychogenic  in  origin. 

Tear  Glands. — These  are  autonomically  innervated  through  the 
superior  cervical  ganglion  and  sympathetically  through  the  spheno- 
palatine ganglion.  Irritation  of  the  neck  sympathetics  causes  increase, 
paresis  of  the  same,  diminution  in  the  secretions.  The  postcellular 
branches  of  the  neck  ganglia,  secretory  or  vasomotor  fibers,  pass  in 
the  internal  carotid  plexus  reaching  the  glands  either  by  the  way  of  the 
ophthalmic  plexus  or  through  the  cavernous  plexus  and  the  lachrymal 
sensory  branch  of  the  trigeminus.  The  secretion  is  markedly  under 
pyschical  influence  as  is  universally  recognized. 

Mucous  and  Salivary  Glands. — The  vegetative  control  of  these  is 
exercised  through  the  sphenopalatine,  otic,  submaxillary,  and  sub- 
lingual glands.  The  sphenopalatine  sends  only  autonomic  vasodilator 
fibers  through  the  ])osterior  nasal  nerves  to  the  mucous  membrane 
of  the  nose;  sympathetic  vasocontrictor  fibers  come  from  the  cervical 
sympathetics. 

The  parotid  gland  has  both  a  sympathetic  and  autonomic  supply, 
the  former  from  the  cervical  sympathetic,  the  latter  through  the  otic 

'  Contsvilt  Archaiiil)ault :  The  SymptoiiiatoloKy  of  Certain  Infectious  Procc.sses  hnolv- 
iug  the  Ciliary  Ganglion,  Joqr.  Nerv.  and  Mcnt.  Dis.,  September,  1917,  vol.  xjvi. 


142 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


ganglion.  The  small  superficial  petrosal  is  its  viator  or  precellular 
root,  the  auriculotemporalis  of  the  trigeminus  is  its  postcellular  branch. 
The  autonomic  bulbar  center  is  Kohnstamm's  nucleus  salivatorius 
inferior.  Autonomic  stimulation  delivers  a  different  type  of  secretion 
from  s^Tiipathetic  stimulation.  Lesions  of  the  tympanic  in  the  mastoid 
operation  give  rise  to  parotid  disturbance,  and.  may  be  looked  for  in 
middle-ear  disease. 


Fig.  50. — The  innervation  of  the  salivary  glands:  olp,  parotid;  gsm,  submaxillary; 
gsl,  sublingual;  ggs,  Gasserian  ganglion;  nl,  lingual  nerve;  nm,  mandibular  nerve;  nVII 
facial  nerve  nucleus;  cht,  chorda  tympani;  VII,  facial  nerve;  IX,  glossopharyngeal 
nerve;  ns,  nucleus  salivatorius;  gp,  petrosal  ganglion;  s,  sympathetic;  rs,  sympathetic 
branches;  gs,  submaxillary  ganglion;  nh,  hypoglossal  nerve;  re,  ramus  communicans. 
(Bechterew.)     (Compare  Plate  III,  p.  104). 

The  submaxillary  and  sublingual  glands  have  a  sympathetic  and  an 
autonomic  supply.  The  latter  of  these  causes  vasodilator  and  secre- 
tory stimulating  effects)  has  its  autonomic  bulbar  center  in  Kohn- 
stamm's superior  salivatory  nucleus,  its  rami  communicans  albi  in  the 
chorda  timpani  and  through  the  lingual  to  the  gland.  The  sympa- 
thetic vasoconstriction  and  secretory  stimulating  fibers  are  derived 
from  the  cervical  sympathetic.  Autonomic  stimulation  causes  the 
full,  thin,  watery,  salty  secretion,  cut  off  by  atropin;  the  sympathetic, 
the  scanty,  viscous  (organic  constituent)  secretion  acted  upon  by 
cocain,  cholin,  adrenalin.  Xerostomia  (Hadden),  xerostomia  senilis, 
xerophobia,  excessive  production  of  frothy  mucus,  constant  spitting 
of  mucus  in  schizophrenia  (often  symbolic  of  semen),  scanty  secretions 
as  seen  in  anxious  states,  in  stage-fright  and  other  types  of  fright, 


SPECIAL  PATHOLOGY— CERVICAL  SYMPATHETIC 


143 


sometimes  unconscious,  as  in  marked  depressions,  etc.,  are  among  the 
disorders  of  the  secretions  of  these  glands  of  neurological  and  psychiat- 
ric interest.  The  symbolic  significance  of  spitting  is  extremely 
complex  and  fertile  in  suggestions  and  its  study,  particularly  in  the 
psychoneuroses  and  psychoses,  only  just  beginning.  Cortical,  glosso- 
phar^Tigeal,  and  trigeminal  associations  are  the  basis  for  reflex  stimu- 
lation of  the  glands. 


i 


•3     / 


'■■:::::c^..Spl^^-''"^ 


Celiac  ^anff. 


''i.RlvicJijjf-''"" 


Fig.  5]. — Innervation  of  the  digestive  tract.     (After  Miiller.) 

Neck  Sympathetic. — The  superior  cervical  sympathetic  supplies, 
through  the  internal  carotid  nerve  and  the  internal  carotid  plexus, 
the  dilator  of  the  pupils,  Miiller's  muscle,  tear,  parotid,  maxillary  and 
lingual  glands,  the  pilomotors,  vasoconstrictors,  and  sweat  glands  of 
the  face. 

Cervical  Sympathetic.  Partial  syndromes  due  to  implication  of 
parts  of  tile  cervical  syni[)atlu'tic  fibers  have  just  l)eon  described  under 
eye,  tear  gland  and  mucous  and  salivary  gland  disturbances.  More 
extended  syndromes  both  of  stimulation  or  of  paralysis  of  the  cervical 
sympathetics  are  frequently  seen  and  are  of  considerable  importance. 


144 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


In  military  practice  cervical  sympathetic  wounds  are  frequent.  As 
has  been  pointed  out  fibers  from  many  diverse  sources  converge  within 
the  cervical  sympathetic.  The  action  of  these  fibers,  in  response  to 
stimulation  or  to  paralysis,  shows  a  great  variety  of  combinations 
such  as  vasoconstriction,  vasodilatation  of  the  cerebral  vessels  giving 
rise  to  congestion,  hyperemia,  anemia,  migraine,  epileptic  attacks,  etc., 
of  the  thyroid  vessels,  causing  varying  states  of  dysthyroidism;  of  the 

base  of  the  orbit,  causing  enophthal- 
mos,  exophthalmos;  the  fundus  of 
the  eye  (glaucoma) ;  of  the  salivary 
and  lingual  glands  (variations  in 
secretion,  xerostoma,  etc.);  of  the 
skin  of  the  face  and  head  (anidrosis, 
hyperidrosis,  seborrhea,  horripila- 
tion, skin  eruptions,  acne,  eczema, 
anomalies  in  pigmentation,  hair, 
beard,  eyebrows)  modifications  of 
active  unstriped  muscle,  partial 
ptosis,  widening  or  narrowing  of  the 
palpebral  fissures,  dilatation  or  con- 
traction of  the  pupils,  acceleration 
or  retardation  of  the  heart  action. 
These  results,  single  symptoms  or 
in  various  combinations,  may  be 
produced  by  lesions  in  the  neck, 
in  the  cerbral  cortex,  medulla,  and 
spinal  cord  (trauma,  tumor,  syphilis, 
multiple  sclerosis,  syringoencephalo- 
myelia, etc.),  in  the  brachial  plexus, 
in  the  chest  cavity  (tuberculosis, 
tumors,  pneumonia),  depending 
upon  the  anatomical  pathways  or 
synapses  implicated. 

The  typical  complete  picture  of 
a  cervical  sympathetic  stimulation 
will  show  dilatation  of  the  pupils, 
exophthalmos,  from  retraction  of 
the  lids  and  protrusion  of  the 
bulbs,  increased  intra-ocular  tension, 
myopia,  submaxillary  and  parotid 
hypersecretion  (sympathetic  saliva- 
tion, thick  and  comparatively  sparse),  cardiac  acceleration,  vaso- 
constriction of  the  skin  of  the  head,  neck;  vasoconstriction  of  the 
meninges,  the  brain,  the  eyes,  the  mucous  membrane  of  the  mouth 
and  tongue,  and  of  the  thyroid  gland. 

An  analogous  picture  of  sympathetic  paralysis  (Bernard-Horner 
syndrome),  Avell  contracted  pupil,  diminished  intra-ocular  tension, 
abolition  of  ciliospinal  reflex,  loss  of  cocain  dilatation,  retraction  of 


Fig.  52.— Schematic  arrangement  of 
cardiac  nerves :  nX,  chief  motor  nucleus ; 
X,  vagus  nerve;  gs,  stellate  ganglion; 
Is,  superior  laryngeal  nerve;  s,  sympa- 
thetic; pis,  solar  plexus;  pic,  cardiac 
plexus;  1,  upper  inner  branch  to  heart; 
2,  accelerator;  3,  internal  inferior 
branch;  4<  upper  and  inferior  external 
branch;  5,  Ansa  Vieussenii.  (Bech- 
terew.) 


GASTRO-INTESTINAL  SYNDROMES 


145 


the  eyeballs  (enophthalmos),  hypermetropia,  loss  of  luster  of  eye, 
slight  ptosis  from  palsy  of  ^Miiller's  muscle,  easily  overcome  by  volun- 
tary action  of  III  X,  dilatation  of  the  conjunctival  vessels,  homo- 
lateral rise  in  temperature  of  the  side  of  the  face,  increase  in  the 
lachrymal  secretions,  sialorrhea  (thin,  watery  secretion),  slowing  of 
the  heart,  anidrosis,  and  seborrhea  sicca. 

Gastro-intestinal  Syndromes. — Vegetative  disturbances  in  swallow- 
ing are  commonplaces  of  every-day  medical  practice.  The  universal 
so-called  "hysterical  globus,"  "the  lump  in  the  throat,"  which  Democ- 
ritus  symbolized  as  a  wandering  uterus,  is  one  of  the  most  familiar. 


Fig.  53. — Innervation  of  the  mechanism  of  .swallowing:  Sn,  substantia  nigra;  Vni, 
motor  nucleus  of  the  trigeminus;  Vn,  sensory  root  of  the  trigeminus;  IX^n,  motor  nucleus 
of  the  glossopharyngeus;  XII,  nucleus  of  the  hypoglossus;  Xs,  sensory  nucleus  of  the 
vagus,  pm,  sf>ft  palate;  apa,  palatal  vault;  app,  pharyngeal  vault.     (Bechterew.) 


It  is  a  very  complex  phenomenon,  and  its  etiology  is  multiform  (psy- 
choanalytically  speaking,  much  over-determined).  In  the  psycho- 
neuroses  it  is  often  a  symbol  of  disgust,  a  surrogate  for  vomiting,  an 
unconscious  remnant  of  infantile  food  impregnation  fantasies;  again  it 
is  a  symptom  of  fear  (inferiority  symbol)  largely  determined  l)y  the 
unconscious  peeping  and  exhibitionistic  infantile  trends. 

Local  constrictions  of  the  esophagus  are  frequently  met  with  in 

neurotic   individuals.     The   accompanying  .r-ray   photograph   shows 

such  a  variable  stricture  due  to  vagotonic  disturbance  in  a  case  of 

anxiety  hy.steria  in  which  rumination  was  a  prominent  feature  and 

10 


146 


VEGETATIVE  OR  VISCERAL  NEUROLOGY 


which  could  be  induced  by  irritating  the  patient  by  even  the  simplest 
forms  of  contradiction  (Fig.  54). 

The  vagus  plays  such  a  large  role  in  these  disturbances  that  a 
few  words  may  be  said  concerning  its  structure  (Fig.  46). 

The  vagiis  like  the  oculomotor,  glossopharyngeal  and  fifth  is  a 
mixed  nerve  and  contains  motor,  sensory  and  autonomic  fibers.     The 


Fig.  54. — Vagotonic  contraction  of  esophagus  in  rumination. 


somatomotor  nucleus  is  the  nucleus  ambiguus;  the  nucleus  solitarius 
is  the  sensory  nucleus;  the  visceral  nucleus  for  the  heart,  lungs,  and 
digestive  system  is  the  nucleus  dorsalis  vagi  lying  on  the  floor  of  the 
fourth  ventricle.  All  three  sets  of  fibers  travel  through  the  jugular 
and  nodosus  ganglia  to  form  the  vagus,  the  jugular  ganglion  probably 
forming  the  synapse  for  sympathetic  connections  and  anastomoses. 
The  two  ganglia  point  to  two  nerves  phylogenetically :    (a)  The  pure 


G ASTRO-INTESTINAL  SYNDROMES 


147 


motor  branches  are  the  rami  pharjugeus;  (6)  the  pure  sensory  branches 
are  the  meningeal  and  superior  laryngeal;  (c)  the  mixed  motor-sen- 
sory-visceral is  the  recurrent  laryngeal  sending  motor  fibers  to  the 
larynx,  receiving  sensory  fibers  from  the  trachea  and  the  visceral 
fibers  supplying  the  heart,  aorta,  and  vessels  of  the  larynx;  {d)  the 
purely  visceral  branches  pass  to  the  digestive  tract,  the  heart,  the 
liver  and  the  lungs. 

Only  the  visceral  branches  will 
be  taken  up  here,  the  motor  and 
sensory  being  discussed  later  under 
the  Cranial  Nerv^es. 

Esophagus. — ^The  entire  digestive 
tract  is  served  by  the  sympathetic 
(narrow  sense),  whereas  the  vagus 
(autonomic)  only  supplies  the  lower 
two-thirds  of   the  esophagus,   the 


TiXm 


Fig.  55. — Scheme  of  stomach  innerva- 
tion: a,  b,  ganglia  in  walls  of  the  stomach; 
nXs,  sensory  nucleus  of  the  vagus;  7iXm, 
motor  root  of  the  vagus;  ns,  splanchnic. 
(Bechterew.) 


Fig.  56. — Scheme  of  intestinal  innerva- 
tion: in,  small  intestine;  r,  lower  end  of 
the  large  intestine;  pis,  celiac  plexus;  j^lh, 
hypogastric  plexus;  spl,  splanchnic;  c,c, 
spinal  center  of  intestinal  movements; 
X,  vagus;  nXm,  motor  nucleus  of  the 
vagus;  nXs,  sensory  nucleus  of  the  vagus. 
(Bechterew.) 


stomach,  and  the  intestines  to  the  descending  colon.  The  combined 
action  is  stimulating  (autonomic)  and  depressing  (sympathetic),  whicli 
actions  are  apparently  reversed  in  the  case  of  the  heart  muscle.  Local 
ganglion  cells  seem  to  regulate  the  motorfunctions.  Tactile  and  chemical 
stimuli  are  apparently  unresponded  to  in  the  upper  part  of  the  esoph- 
agus.   Thermal  stimuli  above  40°  C,  below  30°  ('.,  are  felt  (Boring). 


148  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

Deep  pressure  sensibility  is  present,  but  the  pathways  are  not  definitely 
located.  The  lower  end  of  the  esophagus  responds  to  chemical  and 
thermal  and  possibly  other  types  of  stimuli  (Heart-burn) . 

Stomach  and  Intestines. — Local  ganglion  cells  in  the  walls  of  the 
digestive  tract  are  very  frequent  and  are  to  be  regarded  as  the  terminal 
motor  neuron  of  the  vegetative  systems.  (See  Fig.  46.)  The  stomach 
is  strongly  under  associative  relations  with  sight,  hearing  and  smell 
and  its  affect  (psychical)  reactivity  is  extremely  sensitive.  Ordinary 
sensibility  to  tactile  and  thermal  stimuli  are  lacking,  but  deep  sensi- 
bility fibers  are  present  and  carry  pressure  stimuli  (pain,  colic,  crises). 
The  pathways  used  for  these  are  probably  through  the  vagus  (stimu- 
lating) and  the  splanchnics  (inhibiting) .  Fig.  46  shows  the  distribution 
of  the  vagus  fibers  (Fig.  47).  Thus  both  autonomic  and  sympathetic 
systems  are  utilized.  Eppmger  and  Hess  have  seemed  to  show  that 
in  the  laryngeal,  bronchial,  esophageal,  gastric,  intestinal,  genital 
and  rectal  crises,  in  tabes  particularly,  the  autonomic  system  only  is 
involved;  the  vagus  bulbar  autonomic  for  the  upper  types,  the  pelvic 
autonomic  series  for  the  lower  ones.  Foerster  describes  vagus  and 
splanchnic  (?)  crises.  The  former  are  without  pain,  but  with  nausea, 
hypersecretion,  and  vomiting,  the  latter  with  pain  and  hyperesthetic 
skin  zones,  and  increased  epigastric  and  abdominal  reflexes. 

Timme^  conducted  a  series  of  experiments  by  partially  cutting  off 
autonomic  stimuli,  thus  producing  increased  sympathetic  stimuli 
action  upon  the  stomach  with  important  and  far-reaching  implications 
bearing  upon  the  problems  of  visceral  disease,  in  general,  and  its 
relation  to  vegetative  nervous  system  activity.  Such  imbalanced 
actions  are  constantly  brought  into  operation,  as  has  been  stated, 
by  mechanical  factors,  toxins,  by  hormones  and  by  mental  stimuli. 
Visceral  disease  then  should  be  studied  from  the  point  of  view  of  the 
balanced  relations  of  these  two  antagonistic  systems.- 

The  local  topographical  diagnosis  and  the  physiological  under- 
standing of  nervous  dyspepsias,  the  motility  and  secretory  anomalies 
(achylia,  hj^jersecretion,  hyperacidity,  increase  of  gas,  ptoses,  hour- 
glass contractions),  changes  induced  in  Addison's  disease  and  in  exoph- 
thalmic goiter  and  in  all  of  which  sympathetic  (psychical)  influences 
play  a  large  role  are  as  yet  not  thoroughly  analyzed.  Radiographic 
study  is  aiding  in  a  knowledge  of  these  anomalies,  especially  the 
motor  ones,  but  the  alterations  seen  must  be  interpreted  as  results 
and  not  as  cmises.  Emotions,  toxins  and  endocrine  stimuli  are  the 
causes,  the  anomalies  the  results.  After  years  of  maladjustment 
permanent  changes  result  and  a  vicious  circle  is  established  in  which 
cause  and  result  are  inextricably  interwoven  in  their  general  effects. 

Individual  and  social  adjustment  at  psychological  levels  seems  to 
influence  them  much  more  effectually  than  measures  addressed  to 
modify  the  perverted  chemisms  and  motility,  especially  at  the  begin- 

'  Experimental  Studies  on  the  Nervous  Mechanism  in  the  Production  of  Hyperplasia, 
Jour.  Ner\-.  and  Ment.  Dis.,  1913,  xl,  311. 

-  Pottenger:  Visceral  Disease  and  the  Vegetative  Nervous  System,   1919. 


GASTRO-IXTESTIXAL  S YX DROMES 


149 


ning  of  these  disorders.  There  is  little  doubt  that  long-continued 
psychical  disturbances  which  cause  very  pronounced  secretory  and 
motor  anomalies  may  ultimately  induce  definite  structural  changes. 
Many  visceroptoses  are  of  this  tji^e.  The  relaxation  is  due  to  irregu- 
larities in  the  reciprocal  innervation  of  the  sympathetic  and  autonomic 
pathways,  induced  in  many  instances  through  psychical  maladjust- 
ments. '  Possibly  toxic  factors  may  ultimately  play  an  important  role, 
or  put  in  another  way,  in  certain  patients  the  emotional  factors  are 
undoubtedly  the  primary  ones,  in  others  it  may  be,  although  this  must 
be  proved,  mere  asseveration  is  not  sufficient,  toxic  factors  (intestinal 
absorption)  may  play  the  primary  role.  Speaking  of  the  intestine  as 
a  "sewer"  is  for  the  most  part  an  incorrect  figure  of  speech. 


Fig.  57. — Duodenal  ulcer  as  end-result  of  a  vicious  psjchogenic  maladjustment. 
Unconscious  hate,  unconscious  fight,  hj'peradrenalemia,  duodenal  spasm,  finally  ulcer 
as  links  in  chain.  Curable  only  by  surgery.  Could  have  been  prevented  by  psycho- 
analysis in  earlier  years.     A,  filling  defects;  B,  incisure. 


The  vagus,  by  way  of  the  solar  ganglia,  stimulating  the  terminal 
neurons,  depresses  the  peristalsis  and  secretions  of  the  intestines.  The 
intestinal  movements,  however,  may  take  place  independently.  The 
tactile-mechanism-reflexes  are  continuously  active;  chemical  reflexes 
are  operative  during  the  passage  of  absorptive  material.  Each  have 
their  sympathetic  and  autonomic  pathways— working  independently 
one  of  the  other.  The  chief  psych oreflex  pathways  seem  to  act 
through  the  vagus;  thus  at  the  upper  end,  such  psychoreflex  activities 
show  increase  of  secretion  with  appetite,  loss  of  secretion  with  worry, 
fear,  and,  acting  on  the  autonomic  pelvic  arc,  the  various  constipations 
and  diarrheas  so  frequently  of  psychic  origin;  the  anal  erotic  and 
anxiety  neurosis  phenomena  so  well  elaborated  by  Freud  being  among 


150  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

them.  Purely  sympathetic  disturbances  with  increased  peristalsis 
and  serous  fluid  may  result  from  loss  of  function  of  the  splanchnics, 
either  as  a  vital  reaction  to  cutting,  trauma,  etc.,  or  to  psychical 
influences,  as  from  shock,  emotion,  fear  and  desire,  both  conscious, 
foreconscious  and  unconscious;  from  toxins,  hormones,  anaphylactic 
substances,^  also  intestinal  putrefactive  products,  etc. 

The  great  importance  of  the  autonomic  and  sympathetic  control 
factors  on  the  vessels  of  the  abdominal  cavity  and  organs  cannot  be 
more  than  mentioned.  Here  the  peripheral  vascular  regulations  are 
in  direct  contrast  with  the  abdominal  ones,  and  hence  the  purely 
mechanical  and  vital  process  of  adjustment  of  blood-pressure  regula- 
tion takes  place.  The  interpretation  of  the  phenomena  of  shock  must 
come  about  through  a  study  of  these  factors,  but  such  cannot  be  taken 
up  here.^ 

Another  feature  of  activity  of  the  vegetative  nervous  system  con- 
cerns itself  with  the  gastro- intestinal  ferments,  and  the  specific  secre- 
tions or  hormones  (gastrins,  gastrosecretine,  enterokinases  of  the 
various  authors).  Many  hormones  of  the  endocrinous  glands 
undoubtedly  influence  the  gastro-intestinal  functions.  The  diarrhea 
of  exophthalmic  goiter  (thyroxin)  is  a  very  classical  example  of  this 
influence.  Gastric  ulcer,  duodenal  ulcers  resulting  from  increased 
adrenalin  activities  are  other  less  comprehended  reactions  in  this  impor- 
tant field .  Here  the  adrenalemia  is  directly  under  autonomic  control  and 
is  largely  a  response  to  fear  and  hate — conscious  and  more  temporary, 
unconscious  and  usually  more  persisting.  Hence  when  it  is  said  that 
these  disorders  appear  in  asthenic  states,  this  means  the  asthenic  states 
are  usually  unconscious  fear  states  and  are  interpretable  through 
psychoanalysis^  (see  Fig.  57) . 

Westphal  has  shown  further  that  in  a  large  number  of  peptic  ulcers 
he  has  observed  the  signs  of  vegetative  disorder  such  as  dilated 
pupils,  exophthalmos,  increased  secretions,  bradycardia,  spastic  con- 
stipation, increased  vascularity  of  the  thyroid,  loss  of  abdominal 
reflexes,  increased  knee-jerks,  strong  reactions  to  adrenalin,  atropin 
and  pilocarpin.  Accompanying  symptoms  of  vegetative  nature  were 
gastrosuccorrhea,  pylorospasm,  hour-glass  contraction. 

The  gastric  mucous  membrane  secretes  a  hypothetical  hormone 
which  influences  the  activity  of  peristalsis  through  the  sympathetic 
pathways. 

Surgical  procedures  become  necessary  after  a  number  of  years  of 
faulty  vegetative  functioning.  The  vicious  cycle  set  up  by  faulty 
emotional  discharges  ultimately  causes  tissue  changes.  Premature 
surgery  is  pernicious;  too  delayed  a  relief  in  long-standing  cases  equally 

1  See  a  Comprehensive  and  Valuable  Study  on  the  Relation  of  Anaphylaxis  to  the 
Vegetative  Nervous  System  by  J.  H.  Smith,  Jour.  Nerv.  and  Ment.  Disease,  January, 
1917,  vol.  xlv. 

^  Consult  Crile:  Study  of  the  Emotions,  Philadelphia,  1915. 

'  For  suggestive  studies  on  changes  in  vegetative  ganglia,  visceral  disease  and  psychoses, 
see  Morse,  M.  E.,  Jour.  Nerv.  and  Ment.  Dis.,  January,  1917,  xlv.  Laignel  Lavastine: 
Loo.  cit. 


GASTRO-INTESTI NAL  S YN DROMES 


151 


foolhardy.     Autonomic  cases  are  apt  to  respond  well  to  many  surgical 
procedures.     The  atonic  or  sympathicotonic  cases  do  badly  as  a  rule.^ 

Rectum. — The  chief  innervation  is  through  the  hemorrhoidal  plexus 
and  the  inferior  mesenteric.  Both  autonomic  and  cerebrospinal  influ- 
ences are  active.  Voluntary  muscle  activities  play  a  large  role  in 
defecation,  the  grade  of  tension  in  the  rectum,  however,  is  registered 
by  the  autonomic  system,  which  is  responsible  for  the  original  impulses, 


Fig.  58. — Scheme  of  pelvic  innervation;  eg,  spinal  genital  center;  In,  first  lumbar; 
s,  sympathetic;  ct,  conus;  ilh,  ileohypogastric;  gmi,  inferior  mesenteric  ganglion;  ghp, 
hypogastric  ganglion;  plh,  hypogastric  plexus;  ghm,  hemorrhoidal  ganglion;  Jih  (above), 
hypogastric  nerve;  ne,  sacral  nerve;  s,  erigens;  npc,  common  pudendal;  nh  (below), 
hemorrhoidal  nerve;  ndp,  dorsalis  penis;  npp,  deep  perineal;  vu,  bladder;  scr,  scrotum. 
(Bechterew.) 

after  which  voluntary  and  involuntary  activities  are  operative.  The 
spinal  autonomic  center  is  located  in  the  limibosacral  spinal  segments. 
The  cortical  association  connections  are  thought  by  Bechterew  to  be 
in  the  sigmoid  gyrus.  Frontal  association  pathways  are  also  present, 
interference  with  which  causes  involuntary  defecation,  as  in  frontal 
tumor,  general  paresis,  epileptiform  convulsion,  profound  stupor, 
emotional  loss  of  control,  etc. 

'  Hutchinson,  R.:  Disappointments  after  Gastro-enterostomy,  British  Med.  Jour., 
May  3,  1919.  Also  see  L'ulc^re  chronique  de  I'estomac  et  du  duodenum.  Verhandl. 
d.  Schw.  Gesellschaft  f.  Chir.  Correspblt.  f.  Schw.  Aerzte,  xlix,  1919,  pp.  708  and  709. 


152  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

Interruption  of  spinal  pathways  may  cause  obstij)ation  or  diarrhea 
(tabes,  poHomyelitis,  multiple  sclerosis,  tumor,  syphilis  of  cord, 
hematomyelia,  syringomyelia,  etc.).  Here  deep  sensibility  conducting 
fibers — autonomic  and  cerebrospinal — are  interfered  with  and  the  auto- 
nomic reflexes  fail  to  establish  the  psychical  connections  either  for 
compulsion  (discharge)  or  control  of  sphincter  (retention).  The  anal 
reflex  here  is  of  great  localizing  value,  its  positive  appearance  ruling 
out  somatic  disease  of  the  lower  sacral  and  coccygeal  segments. 
Lesions  of  the  cervical  or  dorsal  cord  interfere  with  the  voluntary 
activities  of  the  abdominal  muscles  in  defecation,  while  lesions  of  the 
lower  lumbar  cord  cause  changes  in  the  voluntary  sphincters.  In 
sacral  lesions,  with  involvement  of  the  external  sphincter  nucleus,  the 
anus  remains  wider  open,  not  so  sharply  corrugated,  not  as  vigorous  in 
closing  and  there  is  loss  of  the  anal  reflex.  Notwithstanding  the  loss 
of  the  voluntary  sphincter,  autonomic  closure  is  possible.  There  is 
therefore  no  single  defecation  center  in  the  lower  cord. 

Severe  constipation  may  be  of  purely  autonomic  origin.  This  con- 
stipation reaction  may  occur  at  physicochemical  levels,  such  as  those 
due  to  excessive  absorption  of  water;  at  vital  levels,  i.  e.,  resulting 
from  pain  from  kidneys,  gall-bladder,  peritonitis,  chronic  appendicitis, 
hemorrhoids;  or  at  purely  psychical  levels,  where  infantile  pleasure 
fantasies  may  play  a  large  role,  i.  e.,  anal  erotic  in  displacement  of 
affects,  birth  fantasies,  etc.  Birth  and  death  fantasies  which  deal 
with  feces,  and  which  are  concealed  behind  constipations  and  diarrheas 
are  very  frequent  among  psychoneurotics  and  psychotics,  particularly 
in  schizophrenics,  (g.  i\)  In  schizophrenics  frequent  fecal  discharges, 
fecal  smearings,  fecal  eating  are  symbolic  activities  for  which  the 
psychoanalytic  technic  often  reveals  the  psychical  equivalents.  The 
anatomical  pathways  which  make  such  relationships  comprehensible 
exist  in  the  autonomic  fibers.  Constipation  as  a  correlate  of  miserli- 
ness is  an  instance  in  point,  and  is  more  fully  discussed  in  the  chapters 
on  the  Psychoneuroses.  Most  constipation  is  purely  psychogenic  and 
the  habitual  use  of  cathartics,  mineral  oils,  enemas,  etc.,  tends  to 
increase  it  since  it  provides  a  recurring  anal  erotic  satisfaction  through 
large  bowel  movements. 

Vagotonic  manifestations  within  the  gastro-intestinal  tract  are  of 
considerable  importance,  although  as  yet  far  from  being  definitely 
analyzed.  Pilocarpin  and  physostigmin  increase  them,  whereas 
adrenalin  and  atropin  diminish  them.  In  vagotonic  individuals  there 
are  increased  esophageal  cardiac  spasms,  tendency  to  increased 
salivation  and  to  increased  secretions  from  the  nose  and  eyes.  There 
is  slowness  in  the  peristalsis,  as  shown  by  radioscopic  examination, 
due  to  increased  muscular  tonus.  This  latter  causes  the  stomach 
form  of  hyperkinetic  motility  gastroneurosis.  It  may  arise  from 
disorder  at  the  physicochemical  or  psychical  levels.  Hypersecretion 
and  hyperacidity  are  accompaniments  with  pylorospasm.  Certain 
cerebrospinal  levels  may  also  be  involved,  as  shown  by  the  Head  hyper- 
sensitive skin  areas.     Membranous  enteritis  or  colitis  with  mucus  and 


G ASTRO-INTESTINAL  SYNDROMES  153 

many  eosinophile  cells  in  the  blood  and  mucous  secretions  is  associated 
with  this  condition  summarized  as  vagotonia.  Here  psychical  influ- 
ences are  of  great  moment.  The  constipation  just  spoken  of  may  be 
arranged  in  this  vagotonic  group.  Eenal  and  biliary  colics,  spasmodic 
jaundice,  reflex  anuria,  eosinophilia,  and  increased  glucose  tolerance 
are  to  be  found  in  this  vagotonic  group.  This  type  of  vagotonics  or  of 
regression  tends  to  develop  the  depressed  phases  of  a  manic  depressive 
psychosis  if  their  (usually  paranoid)  compensations  break  down. 

Diarrheal  states  in  hyperthyroidism,  in  anxiety  neurosis  and  in 
various  systemic  toxemias  (acidosis  in  children)  are  mediated  through 
vegetative  mechanisms.  Involvement  of  the  sympathetic  nuclei  in 
the  cord  by  poliomyelitis  may  cause  severe  diarrhea  and  constipation 
symptoms. 

Genito-urinary  System. — Here  autonomic  and  cerebrospinal  controls 
are  in  evidence.  The  former  act  through  the  mesenteric,  hj'pogastric 
and  hemorrhoidal  autonomic  sacral  ganglia,  supplying  with  non- 
meduUated  fibers  the  involuntary  muscles  and  the  mucous  membranes, 
N.  hypogastricus  to  muscles  of  colon  and  bladder  (sphincters),  the 
plexus  cavernosus,  and  ner\Tis  erigens  to  the  genital  vasomotors. 
The  latter  act  through  meduUated  fibers  to  the  voluntary  muscles  and 
adjacent  skin  areas.  The  nervus  pudendus  communis  supplies  the 
external  sphincter  ani,  external  sphincter  vesicae,  compressor  urethrse, 
deep  perinei,  etc. 

A  series  of  autonomic  reflexes  are  here  met  with,  the  most  important 
being : 

1.  Scrotal  reflex:  Stroking  of  perineum  or  femoral  skin;  con- 
traction of  dartos. 

2.  Bladder,  reflex:  Stretching  or  stimulus  (mechanical,  psychical) 
of  bladder  wall;  contraction  of  bladder  (mechanical,  psychical). 

3.  Rectal  reflex:  Stretching  or  stimulus  of  rectum;  contraction  of 
rectum. 

4.  Genital  reflex:  Psychical  or  mechanical  stimulus;  erection  and 
hx-peremia;  corpus  cavernosus. 

5.  Uterus  reflex:    Stretching  or  irritation  of  uterus;  contraction. 

6.  Anal  reflex:  Stretching  of  anus;  psychical;  contracture  of 
sphincter  ani. 

All  of  these  reflexes  act  through  psychical  levels  as  well  as  through 
peripheral,  i.  e.,  somatic  ones. 

Bladder. — The  general  mechanisms  of  the  bladder  pattern  after 
those  of  the  rectum  and  quite  analogous  symptoms  follow  disturb- 
ances of  homologous  relationships  of  the  autonomic  and  spinocerebral 
pathways.  The  chief  autonomic  series  travel  in  the  sacral  vesical 
nerves  to  and  from  the  inferior  mesenteric  and  hypogastric  ganglia. 
Sympathetic  fibers  are  also  functionating  through  the  hypogastric, 
to  and  from  the  inferior  mesenteric  ganglia.  Thus  the  bladder  has  a 
vegetative  mechanism  comparable  to  that  of  the  pupils. 

Emptying  of  the  bladder  follow's  similar  lines  to  that  of  emptying 
of  the  rectum.     Section  of  the  cord  to  abo^'e  the  mid-dorsal  region 


154  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

brings  about  automatic  emptying.  Psychical  influences  are  here 
active  as  in  the  case  of  the  rectum — urethral  erotic  with  retention  and 
incontinence  of  purely  psychical  character.  These  are  discussed  under 
Psychoneuroses,  whereas  the  more  mechanical,  neurological  features 
are  taken  up  under  Diseases  of  the  Spinal  Cord. 

Sexiial  Organs. — Autonomic  and  sympathetic  supplies  are  present. 
The  former  carry  stimuli  through  the  nervi  erigentes  from  the  sacral 
cord,  producing  vasodilatation  and  erection,  turgor  in  the  female, 
nipple  erection,  etc.  The  latter  carry  stimuli  through  the  hypogastric 
nerve  to  cause  vasoconstriction  and  contraction  of  the  unstriped 
musculature  of  the  sexual  glands  and  discharge  channels.  In  the 
act  of  copulation,  desire,  erection  and  ejaculation  (orgasm)  may  show 
separate  mechanisms.  At  the  physicochemical  level  the  concretization 
of  sexual  desire  usually  reaches  an  active  adult  stage  with  the  onset  of 
puberty.  It  is  assumed  that  chemical  stimuli — hormones — act  at  this 
level  to  cause  tension — tumescence — within  the  organs  themselves, 
and  also  possibly  working  upon  higher  level  nervous  structures  cause 
an  increase  in  vital  and  psychical  tension,  thereby  causing  increased 
sensitization  to  sensory  contacts  and  to  mental  stimuli.  Thus  the 
love  impulse  springs  up  at  a  touch  or  under  the  influence  of  a  symbolic 
expression,  as  in  poetry,  or  other  artistic  creation. 

The  whole  impulse  of  life  and  of  the  principle  of  race-preservation, 
i.  e.,  immortality,  is  bound  up  in  the  instinct  of  reproduction.  The 
energy  of  this  instinct  has  been  termed  libido  by  various  writers,  by 
others  the  word  is  used  in  a  wider  sense,  as  synonymous  with  the  life 
energy  wherein  one  can  distinguish  a  nutritive  or  self-preservative  and 
a  sexual  or  race-propagation  component. 

Certain  hints  obtained  from  the  study  of  the  processes  of  reproduc- 
tion in  lower  organisn-s — protozoa,  protophyta — tend  to  show  that 
the  continuance  of  the  life  of  the  individual  and  of  the  species  has  been 
obtained  through  a  sacrifice  of  the  ego.  Purely  individual  reproduction 
gave  way  to  gametic  reproduction.  The  principle  of  fertilization 
by  sexual  processes  replaced  the  principle  of  individual  reproduction, 
as  by  budding,  for  example. 

To  put  the  matter  in  a  few  words,  the  process  has  been  something 
like  this:  The  original  unicellular  organism  was  all-sufficient;  the 
ameba,  for  example,  performs  all  of  the  functions  of  ingestion,  diges- 
tion, egestion  and  reproduction  with  practically  no  structural  differen- 
tiation. To  be  sure,  there  are  certain  differences  in  different  parts  of 
the  protoplasm,  but  they  are  relatively  mconsiderable,  and  after  all, 
all  of  these  functions  are  carried  on  in  the  single  cell.  Correspondingly 
this  single  cell  is  practically  immortal,  that  is,  it  only  dies  as  a  result 
of  accident.  The  immortality  is  secured  at  the  expense  of  differentia- 
tion of  structure.  Immortality  can  only  be  attained  in  simple,  all- 
suflScient,  unicellular  organisms.  In  developing  from  this  primary 
condition  one  of  the  first  steps  is  a  union  of  a  group  of  cells,  forming  a 
more  or  less  loosely  integrated  organism.  As  evolution  proceeds, 
however,  this  integration  becomes  much  more  definite  and  along  with 


GASTRO-INTESTINAL  SYNDROMES  155 

it  there  goes  difFerentiation  in  the  functions  and  correspondingly  in  the 
structures  of  the  different  cells,  so  that  there  begin  to  be  cells  which 
are  set  aside,  so  to  speak,  for  digestion,  others  that  are  set  aside  for 
reproduction,  etc.  The  cells  so  differentiated  are  very  much  more 
efBcient  in  the  performance  of  their  several  functions  than  the  original 
undifferentiated  cell  was,  but  each  cell  so  differentiated  has  reached 
its  excellence  at  the  expense  of  giving  up  (sacrif  cing)  its  other  functions 
and  developing  in  this  one  particular  way.  The  advantage  gained  has 
been  that  each  cell  of  the  group  received  better  service,  so  far  as  each 
function  was  concerned,  than  before,  but  each  cell  had  to  sacrifice 
something  of  its  own  independence  in  order  to  get  this  advantage,  and 
in  making  this  sacrifice  perhaps  the  most  important  thing  which  it 
gave  up  was  its  practical  immortality.  Viewed  from  this  angle  it  is 
made  apparent  that  death  itself  has  been  acquired  by  natural  selection 
because  of  its  advantages.  Highly  complex  individuals  soon  accumu- 
late so  much  past  that  it  is  distinctly  advantageous  to  scrap  them  and 
make  a  new  start;  hence,  out  of  death  comes  life,  a  contrast  constantly 
met  with  at  the  s\Tnbolic  level.  In  the  highest,  most  complicated 
organizations,  therefore,  each  cell,  while  it  has  a  certain  individuality, 
is  highly  specialized  and  therefore  has  only  a  relatively  short  span  of 
life.  It  has  given  up  a  great  deal  in  order  that  the  community  of  cells 
of  which  it  is  a  part  may  profit.  In  the  progress  of  evolution  the 
process  of  selection  is  being,  so  to  speak,  slowly  transferred  from  the 
single  cell  to  the  larger  group.  Each  function,  therefore,  whether  of 
an  organ  or  only  of  a  single  cell,  may  be  looked  at  from  the  double 
point  of  view  as  to  whether  it  ministers  to  the  preservation  of  the 
individual  organ  or  cell  as  such,  or  whether  it  ministers  to  the  preserva- 
tion of  the  whole  organism,  and  therefore  it  may  again  be  seen  that 
both  nutritive  and  reproductive  activities  are  represented  at  all  three 
levels,  the  physicochemical,  the  sensorimotor  and  the  psychic. 

Inasmuch,  however,  as  vital  energy  acting  solely  through  physico- 
chemical  processes  does  not  afford  any  adequate  explanation  for  all 
and  least  of  all  for  the  most  important  of  the  phenomena  of  evolution, 
an  adequate  hj-pothesis  must  also  include  similar  activities  at  higher 
levels,  i.  e.,  vital  and  psychical.  The  out-and-out  materialist  stops 
at  the  lowest  levels,  the  vitalist  midway,  the  evolutionist  argues  for 
the  leadership  of  the  psychical,  but  needs  the  interrelationship  of  all. 
Psychical  impotence  with  intact  organs,  for  instance,  is  inexplicable 
on  materialistic  hypotheses. 

Seen  from  another  angle  this  vexed  subject  of  interrelationships  is 
well  illustrated  in  the  large  disease  group  of  schizophrenia  (dementia 
precox).  From  the  psychical  side  alone  some  have  endeavored  to 
explain  it  as  a  series  of  reactions  to  repressed  and  unconscious  sexiial 
activities — repressed  and  unconscious  because  of  higher  cultural 
demands  and  inability  on  the  part  of  the  patient  to  sublimate,  i.  e., 
employ  his  libido  in  its  numerous  useful  socialized  transformations 
arrived  at  in  the  course  of  cultural  development.  A  compromise 
situation  adopts  the  interrelatory  hypothesis.    Reasoning  in  such  terms 


156  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

the  interrelationist  says  that  schizophrenia  is  a  disorder  occurring 
in  certain  inferior  indi\'iduals;  inferior  because  of  certain  structural 
defects  of  the  gonadal  or  other  endocrinous  systems  (testes,  ovaries, 
pituitary,  etc.).  These  disorders  induce  changes  at  the  metabolic 
(physicochemical)  level  and  thus  bring  about  the  disease,  which  because 
of  inferiority  in  these  endocrinous  glands,  chiefly  gonadal,  cause 
a  symptomatology  which  is  largely  tinctured  with  sexual  concepts. 
The  Abderhalden  pregnancy  reaction — ovarian,  testicular,  hormone 
changes — shows,  in  some  cases,  a  peculiar  activity  with  schizophrenics 
pointing  to  some  disturbance  at  the  physicochemical  level,  although 
all  of  the  confusing  contradictions  in  technic  have  not  yet  permitted 
any  positive  statements  even  as  to  these  results.  To  the  student  of 
the  problem  from  the  psychical  side  it  is  difficult  to  comprehend  how 
changes  in  chemical  reactions  will  determine  a  fairly  constant  mental 
picture  which  psychoanalysis  shows  to  be  largely  occupied  with  sexual 
symbolization  with  its  consequent  emotional  reactions.  The  increas- 
ing evidence  showing  that  emotional  reactions  can  produce  somatic 
modifications,  as  seen  in  hysterical  conversions,  compulsive  substitu- 
tions, and  psychotic  projections,  tends  to  throw  the  proof  over  to  the 
psychical  side,  with  the  compromise  situation  that  both  somatic 
inferiority  and  psychical  symbolizations  are  interrelated  and  more 
or  less  reciprocal  phenomena.  The  student  by  keeping  his  mind  open 
and  thinking  in  these  various  terms  will  certainly  gather  more  real 
information  from  his  patient  than  if  he  held  to  one  side  only  of  the 
problem. 

In  the  phenomenon  of  erection  one  sees  these  principles  at  work. 
The  cerebral  or  psychical  is  the  most  frequent  source  of  origin  for  vaso- 
dilation. The  pathways  are  by  means  of  the  cord  to  the  upper  lumbar 
segments  and  by  way  of  the  erector  nerves.  In  severe  spinal  injuries 
psychical  erection  may  remain  intact.  Severe  continuous  priapism 
is  not  infrequently  of  purely  cerebral  origin,  either  organic  as  in 
encephalitis,  non-purulent  or  purulent,  syphilitic  (paresis),  or  possibly 
purely  psychical  as  in  some  manic  states,  some  schizophrenics. 

Sensorimotor  levels  respond  to  the  sensory  stimuli  of  the  skin  of 
the  penis  or  adjacent  organs,  and  the  reflex  pathways  are  made  up  of 
the  spinal  sensory  nerves,  the  second  sacral  segment  and  the  dorsalis 
penis  and  pudendis  communis  nerves  acting  through  synaptic  junc- 
tures in  the  sympathetic  ganglion. 

The  physicochemical  levels  respond  to  the  tension  stimuli  from  the 
bladder,  seminal  glands,  etc.,  acting  through  the  hypogastric  plexus. 

In  ejaculation,  sympathetic  and  cerebrospinal  pathways  are  utilized. 
A  summation  of  stimuli,  acting  through  the  sympathetic,  forces  the 
threshold,  setting  free  a  peristaltic  contracture  of  the  vasa  deferentia 
with  the  accumulation  of  genital  secretions  in  the  prostatic  portion  of 
the  urethra.  A  spinal  reflex  causes  the  contraction  of  the  bulbo-  and 
ischiocavernous  muscles  with  the  ejaculation  of  the  semen. 

Sympathetic  disturbances  are  rare,  spinal  ones  not  infrequent  in 
conus  lesions,  either  being  traumatic  or  due  to  new  growth  or  infil- 


G ASTRO-INTESTINAL  SYNDROMES 


157 


trating  disease,  tumor,  syphilis,  etc.  Ejaculation  in  coitus,  in 
masturbation,  or  in  pollution  dreams  is  usually  accompanied  by 
other  autonomic  signs,  such  as  mydriasis,  hyperidrosis  and  cardiac 
palpitation.  Pollution  dreams  have  determinants  at  all  the  levels 
mentioned.  They  are  usually  not  harmful.  When  frequent  and  evi- 
dently pathological  they  may  arise  from  lower  level  stimuli  (prostatic 
disease,  etc.),  but  are  more  often  of  psychical  origin — usually  accom- 
plishing the  repressed  and  unconscious  wish  for  culturally  forbidden 
sexual  activities  (masturbatory,  homosexual,  incestuous,  or  bestialit}' 
fantasies).  Impotency,  partial  or  complete,  is  most  frequently 
chiefly  a  psychogenic  complaint.  Psy- 
choanalysis shows  that  copulation  is 
a  highly  complex  act  and  that  com- 
plete potency  is  only  attained  when 
all  of  the  partial  libido  trends  are  sub- 
limated in  a  real  love  act,  i.  e.,  one 
that  is  essentially  constructive. 

Respiratory  Apparatus.  —  Complete 
data  are  not  available  for  definite  plot- 
ting of  the  vegetative  nerve  physiology 
in  this  region.  Autonomic  vagus  fibers, 
acting  through  the  superior  laryngeal, 
tracheal,  and  bronchial  nerves,  induce 
reflex  coughing,  inflammatory  reactions 
with  increase  of  mucus,  etc.  Somatic 
fibers  are  concerned  as  well.  Potten- 
ger  has  shown  the  great  importance 
of  the  study  of  the  vegetative  nervous 
system  in  tuberculosis.^  His  palpation 
sign  is  one  of  the  proofs  of  the  pro- 
tective autonomic  response.  Psychical 
determiners  probably  play  a  large  part 
in  this  disease  in  affecting  the  vegeta- 
tive resistance  to  the  tubercle  bacillus. 
Hysterical  coughing  utilizes  the  auto- 
nomic pathways.  Asthmatic  attacks, 
with  spasm  of  the  bronchi,  difficulty 

in  breathing,  slowing  of  respiratory  phases,  emphysema  and  eosino- 
philic sputum,  are  illustrations  of  increased  vagotonia,  hence  re- 
lieved in  part  by  adrenalin.  Here  the  exciting  causes  may  also  lie 
at  any  of  the  three  levels.  Physicochemical  (parathyroid  with  tetany, 
calcium  metabolism)  sensorimotor  (from  pressure  phenomena  on 
laryngeal  and  bronchial  nerves,  reflexes  from  nose),  or  psychical 
(emotions,  sexual  excitement,  repressed  sexuality).  The  problem  in 
treating  asthma  is  therefore  to  find  which  nervous  system  level  is 
chiefly  implicated.     Psychoanalysis  would  be  folly  for  those  asthmatic 


Fig.  59. — Scheme  of  innervation 
of  breathing:  D,  diaphragm;  nf, 
phrenic  nerve;  X,  sensory  vagus 
branches  to  the  lungs;  nr,  respira- 
tory nucleus  in  medulla;  ivXs,  sen- 
sory nucleus  of  the  vagus;  nrs, 
respiratory  center  in  midbrain 
region.     (Bechterew.) 


'  See  Pottenger:  Clinical  Tuberculosis,  1917,  Mosby,  St.  Louis. 


158 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


attacks  which  are  due,  for  instance,  to  cheesy,  tuberculous  deposits 
pressing  upon  nerve  structures  in  the  posterior  mediastinum,  while 
it  alone  would  remedy  those  asthmas  that  are  of  psychical  origin 
solely.     Combined  therapy — interrelational — is  of  greatest  value. 


Fig.  60. — Scheme  of  cardiac  innervation:  A.d, 
right  auricle;  A.s,  left  auricle;  V.d,  right  ven- 
tricle; V.s,  left  ventricle;  v.c.s,  and  v.c.i,  superior 
and  inferior  vena  cava;  s.v.c,  sinus  venosus;  F.o, 
foramen  ovale;  K.F.,  Keith- Flack  sinus  node; 
A.T,  Aschoff-Tawara  auriculoventricular  node; 
Th,  Thorel's  superior  vena  cava  bundle;  Tho, 
Thorel's  intermediary  node  bundle;  W.  Wenck- 
enbach's  auriculovenous  bundle;  k.f.,  Keith- 
Flack  fibers  between  K.F.  and  F.o.,  a.  t.,  Aschoff- 
Tawara  bundle  between  A.t.  and  F.o.,  P.H. 
Paladino-His  bundle.     (Janowski.) 


Fig.  61. — Scheme  of  innervation 
of  the  vasomotors:  c»,  main  center 
of  vasomotors  in  the  medulla;  cs, 
spinal  vasomotor  centers;  s.  sym- 
pathetic; nX,  vagus  nucleus;  X, 
vagus;  nV,  trigeminus  nucleus;  plb, 
brachial  plexus;  plv,  vesical  plexus; 
pulm,  lungs;  stom,  stomach;  diiod, 
duodenum;  hep,  liver;  spl,  spleen; 
ren,  kidney;  r,  rectum;  ves,  bladder; 
scr,  scrotum;  pn,  penis.  (After 
Langley.) 


Cheyne-Stokes  respiration  is  found  in  a  great  variety  of  patho- 
logical states  such  as  high  cervical  myelitis,  hemorrhage  of  the  medulla, 
hemorrhage  of  the  base,  tumors  of  the  midbrain  region,  and  occasion- 
ally in  certain  cortical  atrophies  or  hemorrhages.  It  is  rarely  present 
in    certain    hysterics.     Snorting,    barking,    coughing,    sneezing,    hie- 


VASCULAR  APPARATUS  159 

coughing  and  yawning  are  frequent  respiratory  affections.  They  are 
for  the  most  part  psychical,  but  not  always. 

Vascular  Apparatus. — Only  a  brief  outline  is  possible,  although  the 
study  of  the  cardiac  activities  lies  mostly  in  the  vegetative  field. 

Heart. — S\Tnpathetic,  autonomic  and  intraganglionic  mechanisms 
are  present.  The  s\Tnpathetic  pathways  arise  from  D  1-5.  Wrisberg's 
ganglion  is  the  first  synapse,  the  postganglionic  fibers  passing  to  the 
heart  musculature.  The  vagus  is  active  through  three  main  branches, 
one  arising  below  the  superior  laryngeal,  a  second  from  the  recurrens, 
a  third  from  the  thoracic  part  of  the  vagus.  The  deeper  layers  of  the 
heart  are  supplied  through  the  right  vagus,  the  superficial  cardiac 
plexus  supplying  through  the  left.  The  sympathetic  fibers  which 
accelerate  the  heart's  action  are  in  relation  with  the  extracardiac 
ganglia,  the  end  branches  of  the  vagus  in  connection  with  the  intra- 
cardial  ganglion,  ^.  e.,  cells.  The  intracardial  cells  are  here  assumed 
to  be  visceromotor,  and  are  thickest  at  the  origin  of  His's  bundle, 
Tawara's  nodes  and  at  the  origin  of  the  Keith-Flack  bundle.  The 
activity  of  the  vagus  upon  the  muscles  seems  to  manifest  itself  chiefly 
through  the  ganglion  cells. 

The  gray  matter  of  the  midbrain  in  the  neighborhood  of  the  floor 
of  the  third  ventricle  is  thought  to  be  a  higher  coordinating  switch- 
board— the  nucleus  dorsalis  vagi,  an  end  station.  Through  this  por- 
tion of  the  mechanism,  psychical  influences  are  switched  in,  modifying 
the  tonus  through  emotions,  pain  and  local  stimuli. 

Bradycardia  appears  through  a  number  of  influences,  chiefly  follow- 
ing acute  infectious  intoxications,  by  increased  intracranial  pressure, 
in  hypothyroidism,  digitalis  and  allied  glucosidal  actions.  Trigeminal 
reflexes  through  the  nose,  eyes  (pressure)  may  also  cause  brady- 
cardia. The  various  arrhythmias,  dislocations,  and  blocks  cannot  be 
discussed  here. 

The  relation  of  changes  in  or  due  to  His's  bundle  cannot  be  entered 
into  here,  although  they  may  properly  be  discussed  in  a  text-book 
on  neurology. 

Angina  pectoris,  in  some  of  its  forms  at  least,  is  due  to  autonomic 
overestimulation  whereby  vascular  cramp  states  are  brought  about; 
vagus  paralyzing  and  Aasodilator  drugs  therefore  aid  in  overcoming 
the  condition,  particularly  in  the  vasomotor  types  of  angina.  Cardiac 
discomfort  so  frequent  in  visceral  heart  disease,  as  well  as  in  psychical 
disorders  is  carried  to  consciousness  chiefly  through  communicating 
sympathetic  branches  through  the  spinal  ganglia,  or  directly  to  the 
spinal  systems.  Head's  hyperalgesias  are  explained  in  this  manner. 
The  vagus  (autonomic)  fibers  are  not  implicated.  They  are  involved 
in  the  cardiac  crises  of  tabes. 

In  the  anxiety  neurosis,  cardiac  disturbances  are  extremely  frequent. 
Pseudoangina  pectoris  is  usually  a  symptom  of  this  state,  the  further 
elucidation  of  which  will  be  found  in  later  chapters.  Neurotic  car- 
diac disturbances  are  frequent.  In  the  great  world  war  many  pros- 
pective soldiers  developed  cardiac  irregularities.     Many  showed  extra- 


160  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

systoles  which  graphic  tracings  separated  from  heart-block.  A  systolic 
murmur  at  the  apex  growing  louder  on  exertion  with  a  positive  Was- 
sermann  will  likely  prove  a  precursor  of  angina  pectoris.  The  use  of 
adrenalin  and  a  study  of  the  blood  will  aid  in  the  clinical  diagnosis  of 
organic  cardiac  difficulties.  Thus  increased  adrenalin  mydriasis  and 
positive  lymphocytosis  with  cardiac  irregularity  is  usually  organic 
from  a  dysth\Toid  state.  In  determining  military  capacity  the  test  is 
valuable. 

Bloodvessels:  Vasomotor  Neuroses. — The  anatomy,  physiology  and 
clinical  disturbances  of  the  bloodvessels  make  a  large  chapter  in  con- 
temporary neurology.  Cassirer  has  devoted  a  monograph  of  1000 
pages  alone  to  their  consideration.  Only  the  briefest  sketch  is  offered 
here. 

The  bloodvessels  of  the  face  are  innervated  from  the  upper  cervical 
sympathetic  fibers  passing  over  the  internal  carotid  plexus  to  the 
Gasserian  ganglion,  and  with  the  pathways  to  the  sweat  glands  pass 
with  the  sensory  fibers  of  the  face.  ThoSe  of  the  upper  extremities 
are  supplied  chiefly  from  C5  to  D7,  mostly  leaving  by  way  of  the  D3 
to  D7  root  segments.  Those  of  the  lower  extremities  arise  from  Dl-2 
to  L3.  In  the  spinal  axis  are  located  only  the  local  segmental  func- 
tions. Bulbar  centers  are  present  in  the  nucleus  dorsalis  vagi,  which 
is  an  autonomic  synapse  zone  for  peripheral  vessels  as  well  as  those 
of  the  intestines.  Stimuli  in  the  bulbar  centers  tend  to  cause  con- 
traction of  the  peripheral  vessels  and  dilatation  of  the  visceral  ones. 
Intracranial  bloodvessels  have  vasodilator  and  vasoconstrictor  fibers 
conveyed  through  the  cervical  sympathetics. 

Cortical  centers  have  been  placed  in  the  frontal  areas  (Lewandowsky, 
Weber;  denied  by  Miiller  and  Glaser,  who  claim  the  midbrain  as  the 
highest  center),  from  which  the  pathways  pass  through  the  internal 
capsule,  caudate  nucleus,  thalamus,  hypothalamus,  pons,  central  gray 
of  fourth  ventricle,  oblongata,  Helweg's  triangular  bundle,  antero- 
lateral bundle  to  lateral  horns — the  fibers  crossing  in  the  posterior 
commissure  (Helweg).  The  autonomic  and  s;sTnpathetic  fibers  appar- 
ently follow  different  pathways  in  the  cord,  the  vasodilator  auto- 
nomic pathways  following  the  course  of  the  sensory  roots,  the  vaso- 
constrictor sympathetic  by  way  of  the  anterior  roots,  the  motor  nerves 
and  the  sympathetic  ganglion.  Thus  irritation  of  the  posterior  roots 
causes  hyperemia  (vasodilatation)  with  pain,  paralysis  of  the  same 
causes  anemia  with  anesthesia. 

Within  the  bloodvessels  themselves  ganglion  cells  are  found,  save 
perhaps  in  those  whose  vasomotors  run  in  the  spinal  nerves  (Miiller 
and  Glaser),  and  reflexes  occur  here  exactly  as  in  all  of  the  skin  and 
tendon  reflexes  from  terminal  stimuli.  Hence  an  analysis  of  vascular 
disturbances  must  include  a  study  of  the  sensory,  motor  and  central 
portions  of  the  reflex  arc — the  last  including  both  medullary  and 
corticospinal  reflex  pathways. 

Disturbances  of  the  peripheral  mechanisms  of  the  vasomotor 
pathways  have  been  more  completely  analyzed  than  those  resulting 


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TONIC  HYPEREMIAS  161 

from  lesions  in  the  spinal,  bulbar,  thalamic,  or  cortical  portions  of  the 
same.  Of  these,  more  detailed  mention  may  be  made  of  the:  (1)  tonic 
hAperemias  (erythromelalgia) ,  (2)  spastic  anemias  (pseudosclerosis, 
Pa^iiaud's  disease,  migraine,  intermittent  claudication),  and  (3)  vaso- 
motor irritability,  as  in  acute  angioneurotic  edema,  multiple  gangrene 
of  the  skin,  etc. 

Physiological  alterations  in  the  tonus  of  the  peripheral  vessels  are 
seen  in  sleep,  emotional  states,  active  digestion,  overexercise,  over- 
heating, in  collapse  and  in  fatigue  states. 

1.   Tonic  Hyperemias. 

These  consist  in  prolonged  irritability  of  the  peripheral  vasomotors. 
In  certain  individuals  (sympathicotonic)  a  diminished  alkalinity  of  the 
blood  is  thought  to  bring  about  such  a  stimulation  of  the  sympathetics. 
These  tonic  hyperemias  are  seen  more  particularly  in  neuralgias,  neu- 
ritides,  in  infections,  or  toxic  erythemas,  and  reach  a  pronounced  grade 
in  the  s,Midrome  known  as  erythromelalgia.  Thymic  persistence  should 
always  be  inquired  into. 

Erythromelalgia. — Two  main  trends  may  be  distinguished — those 
with  pain,  as  described  by  ^Yeir  Mitchell,  and  those  without  pain  but 
with  hyperidrosis  and  hyperalgesia  (Hess) . 

Weir  Mitchell,  in  1878,  described  a.  paroxysmal  disorder  of  the 
extremities  which  was  marked  by  a  painful  redness  and  swelling  of 
the  feet.  Lannois,  in  1880,  wrote  an  important  monograph  on  the 
subject,  and  Cassirer,  in  the  second  edition  of  his  V asomotorisch- 
trophischen  Nenrosen,  1912,  has  given  a  complete  description  of  the 
general  groiip  to  which  the  name  erythromelalgias  may  be  given.  He 
was  able  to  gather  reports  of  about  130  cases.  One  may  conclude  it 
to  be  rare.  Only  2  in  Oppenheim's  25,000  dispensary  patients  are 
reported,  while  in  JelliflFe's  statistics  of  Starr's  dispensary  service  of 
18,000  patients  21  were  observed,  15  in  males  and  6  in  females.  It 
is  more  often  observed  in  the  later  years  of  life,  although  six-  to  ten- 
year-old  (Baginsky)  patients  are  recorded. 

Causes  are  difficult  to  run  doAMi.  Thermic  influences  apparently 
play  some  role  as  exciting  agents  at  most.  Psychical  factors  may 
determine  an  attack. 

Hypothetically  erythromelalgia  is  a  pure  sympathetic  affection, 
an  angioneurosis,  due  to  prolonged  sympathetic  stimulation.  Prac- 
tically it  shows  itself  in  combined  forms,  being  an  accompaniment 
of  spinal  disorder  (involving  the  sympathetic  cell  groups)  in  multiple 
sclerosis,  and  in  tabes;  it  may  occasionally  be  seen  in  cerebral  disorders, 
hemiplegia,  thalamic  involvement,  or  may  be  a  part  of  a  peri])heral 
nerve  disorfler,  accompanying  a  neuritis,  or  it  may  be  a  cause  of  or  a 
part  of  a  chronic  vascular  disease  of  an  obliterating  or  spasmodic  type. 
In  each  of  these  the  chief  action  is  directed  upon  the  sympathetics. 
Thus  a  number  of  gradations  and  variants  exist  which  are  discussed  in 
the  works  already  cited,  j)articularly  in  Cassirer  and  Oppenheim. 
11 


162  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

Symptoms. — The  chief  symptoms  are  heat,  redness  and  pain  in  the 
extremities,  either  localized  along  a  definite  nerve  distribution,  often 
following  a  root  area,  or  peripheral.  It  is  usually  intermittent,  worse 
at  night,  and  the  patient  suffers  tortures.  Heat  usually  makes  it 
worse,  so  also  does  movement,  especially  walking,  whereas  any  position 
reducing  passive  congestion,  thus  overcoming  the  tonic  hyperemia, 
affords  relief.  Severe  grades  of  the  disorder  show  a  purple  cyanotic 
skin,  with  erythema,  usually  due  to  transudation  following  stasis  from 
slowed  circulation  in  dilatated  area.     Hyperidrosis  may  be  present. 

Accessory  symptoms,  such  as  headache,  palpitation,  and  fainting 
are  reactions  to  the  pain,  and  in  part  to  the  fear,  or  may  be  another 
facet  of  a  psychoneuroses  in  which  the  erythromelalgia  is  also  a  symp- 
tom. Trophic  changes  in  the  skin,  hair  and  nails  may  take  place 
which  are  either  a  part  of  the  sympathetic  disturbance  itself  or  are 
results  common  to  the  angioneurosis,  and  a  producing  or  accompanying 
lesion — tabes,  multiple  sclerosis,  paresis,  etc. 

Course  and  Therapy. ^ — The  outcome  depends  much  upon  the  causa- 
tion. An  erythromelalgia  due  to  spinal  changes  may  get  better  if 
these  do  (syphilis)  or  not  (tumor,  multiple  sclerosis).  The  therapy 
will  be  determined  by  the  cause.  Palliatives,  such  as  the  use  of 
high  frequency  current,  violet  rays,  cold,  antipyrin,  are  valuable  as 
well. 

A  neuritic  erythromelalgia  will  improve  or  not  as  the  neuritis  does; 
similarly  an  arteriosclerotic  one;  but  it  usually  gets  worse.  The 
therapy  is  for  the  more  fundamental  condition.  A  psychically  deter- 
mined erythromelalgia,  possibly  a  hysteria,  needs  psychoanalysis. 

2.  Spastic  Anemic  Group. 

Here  the  chief  results  are  due  to  persistent  or  intermittent  vaso- 
constriction. The  syndromes  are  numerous  and  confusing,  but  among 
them  a  few  are  sufficiently  distinct  or  constant  to  be  given  diagnostic 
titles  such  as  Raynaud's  disease,  intermittent  claudication,  acro- 
paresthesia, migraine,  pseudosclerosis,  asphygmia  alternans,  etc.  Only 
the  chief  types  can  be  taken  up;  the  purely  tentative  nature  of  the 
classification  must  be  emphasized. 

Certain  of  these  cases  are  unquestionably  related  to  underlying 
endocrinopathies  (thyroid,  adrenal),  others  are  primary  and  secondary 
neuritic  syndromes,  or  are  related  to  anatomical  changes  involving 
the  sympathetic  synapses  in  the  lateral  horns  of  the  cord,  syringo- 
myelia, poliomyelitis,  often  overlooked  if  slight  attack,  multiple 
sclerosis,  spinalsyphilis,  etc.,  others  are  exclusively  psychogenic,  chiefly 
hysterical  conversions,  or  schizophrenic  defense  substitutions. 

Raynaud's  Disease. — Raynaud's  disease  is  also  known  as  sym- 
metrical gangrene,  local  asphyxia.  This  syndrome,  like  the  preceding, 
one,  may  be  of  many  origins.  It  may  be  psychical  (shock,  hysteria, 
schizophrenia),  cerebral,  or  spinal  organic  (capsular  (thalamic)  hemor- 
rhage, trauma,  paresis,  multiple  sclerosis,  tabes,  syringomyelia,  tumors) 


RAYNAUD'S  DISEASE 


163 


or  peripheral  in  nerve  or  bloodvessel,  neuritis  of  all  various  etiologies, 
mild  endocrinopathies,  arteriosclerosis  either  peripheral  or  of  the  large 
vessels  (aorta). 

It  may  readily  be  seen  that  from  such  a  polyetiological  viewpoint, 
there  is  no  true  Raynaud's  disorder.  Hence  Cassirer's  attempt  to 
make  true  sympathetic  types  and  those  due  to  complicating  disorders, 


Fig.   62. 


Figs. 


Fig.  63. 
62  and  63. — Raynaud's  disease,  showing  superficial  gangrene. 


(Osier.) 


such  as  the  local  gangrenes  due  to  diabetic  neuritis,  or  to  arterio- 
sclerosis, etc.  Even  this  is  difficult  to  accomplish.  Thus  a  spinal  gliosis 
(syringomyelobulbia)  may  invade  the  sympathetic  cells  in  a  given 
segment  and  bring  about  a  local  paroxysmal  gangrene.  This  is  truly 
a  vegetative  affection,  but  it  is  to  be  considered  as  the  very  beginning 
symptom  of  the  syringomyelia.  Some  patients  after  a  mild  ])olio- 
myelitis,  i.  e.,  mild  sofar  as  motor  involvement  is  concerned,  show 


164  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

marked  vascular  instability  and  develop  RaAiiaud's  syndrome  very 
readily  from  a  variety  of  disturbing  factors — cold,  alcohol,  fright,  etc. 

Symptoms. — The  attacks  are  paroxysmal.  The  fingers  or  toes  begin 
to  get  cold,  and  have  the  feeling  of  prickling  and  of  "going  asleep." 
They  become  pale  and  waxy  from  the  vasoconstriction.  Pain  is  fre- 
quently felt  and  local  coldness  is  present.  An  attack  of  this  kind  may 
come  and  go  in  a  few  hours. 

]\Iore  persistent  attacks  lead  to  more  marked  grades  of  local  asphyxia, 
with  cyanosis,  or  bluish-red  discoloration  of  the  extremities.  Pain  is 
extreme.  Vesicles  may  form — the  fingers  may  even  get  bluish-black — 
and  gradually  disappear  after  a  few  days,  or  leave  slowly  healing, 
broken  vesicles,  or  more  deeply  lying  trophic  ulcers  (protopathic 
nerve  fiber  injury).  Other  tApes  of  sensibility  also  suffer.  Epicritic 
touch  and  thermal  as  well  as  protopathic  pain,  thermal  and  deep 
sensibility,  may  also  be  involved.  Gangrene  is  a  severe  grade  with 
loss  of  fingers  or  finger-tips. 

Accessory  symptoms  (such  as  trophic  changes  in  the  nails,  in  hair, 
in  the  bones,  etc.),  which  are  due  to  the  different  etiological  factors, 
syringomyelia,  neuritis,  arteriosclerosis,  etc.,  need  not  be  entered  into. 
Attacks,  with  recovery,  may  persist  as  long  as  three  or  four  months. 

Treatment. — The  therapy  is  often  without  avail,  as  the  underlying 
condition  is  unmodifiable  (syringomyelia,  multiple  sclerosis,  etc.). 
As  a  rule,  however,  the  attack  subsides,  although  to  appear  again. 
Then  attention  should  be  addressed  to  the  general  health  of  the 
patient,  especially  to  emotional  features  which  produce  vascular 
instability.  Mild  massage,  local  warmth  and  Bier's  h^'peremic  treat- 
ment are  of  value  during  the  attack,  strong  analgesics  being  necessary 
for  the  pain  at  the  time.  Endocrinopathic  cases  do  well  with  thyroid. 
In  psychogenic  cases  psychotherapy  is  alone  available. 

Intermittent  Claudication. — ^This  is  an  angiospastic  syndrome  and 
rests  upon  a  number  of  foundations.  Clinically  it  consists  of  a  spastic 
vascular  state  with  weakness,  pain,  and  coldness  in  the  affected  region. 
In  the  majority  of  cases  it  appears  in  the  leg  or  legs.  After  the  patient 
has  walked,  perhaps  rapidly,  the  leg  or  legs  begin  to  be  fatigued,  and 
commence  to  feel  numb  and  painful  until  it  is  impossible  to  keep  up 
the  pace  or  walk  at  all.  After  a  rest  the  patient  may  resume  his  walk 
for  a  time  free  from  distress,  but  the  state  of  pain  and  fatigue  recurs  to 
be  again  relieved  following  rest.  There  is  a  later  tendency  for  the 
state  to  recur  when  the  limbs  are  at  rest.  Cyanosis,  coldness,  paleness 
are  accompanying  phenomena.  There  is  mild  hj^peresthesia  of  the 
affected  part  but  no  other  sensory  signs.  The  chief  vessels  may  be 
pulseless.  These  should  be  tested  by  touch  and  the  eye  aided  by  the 
sphygmograph. 

The  chief  sites  are  the  vessels  of  the  legs  but  the  arms  may  be 
involved.  Any  muscular  group  may  show  the  symptoms.  Lumbago- 
like forms  occur  in  the  back  muscles.  The  vessels  of  the  intestines, 
internal  organs,  brain,  and  spinal  cord  may  be  involved. 

The  chief  lesion  is  arteriosclerosis,  but  others  are  operative.    The 


OPHTHALMIC  MIGRAINE  165 

arteriosclerosis  itself  may  be  secondary  to  s^'philis,  alcoholism,  to 
chronic  nicotine  poisoning.^ 

Oppenheim  has  called  attention  to  the  frequency  with  which  these 
arterial  changes  are  found  in  Russian  Jews.  This  disorder  is  mostly 
confined  to  the  men  of  this  people.  Here  flat-foot  probably  plays  a 
role — excess  in  walking  (peddlers)  may  aid.  Psychoneurotic  factors 
also  may  play  a  part  in  the  causation  of  these  arterial  cramps  inde- 
pendent of  any  definite  arteriosclerosis.  The  complicated  question 
of  altered  chemism  within  the  vessel  walls  cannot  be  entered  into. 
Hereditarily  inferior  vascular  systems  are  factors. 

Therapy. — Rest,  warm  applications  to  the  parts,  and  high-frequency 
current  application  are  of  value  in  treating  the  attack  in  its  acute 
stage.  Treatment  of  the  condition  rests  upon  the  proper  conception 
of  the  individual  provocative  disorder.  Arteriosclerotic  cases  need 
treatment  for  this;  psychoneurotics  require  psychotherapy.  Of  the 
more  fundamental  therapy  of  the  vegetative  system  which  permits  the 
spasticities  as  well  as  modifies  the  calcium  metabolism  in  the  vascular 
walls  nothing  as  yet  can  be  laid  down. 

Ophthalmic  Migraine.^This  is  also  known  as  sick  headache; 
megrims;  hemicrania;  bilious  headache. 

This  protean  affection  is  difficult  to  define.  It  may  be  a  simple 
or  an  extremely  complex  condition.  Migraine  may,  however,  be 
defined  as  a  periodical  abnormal  state  in  which  the  patient  suffers 
from  a  peculiar  oppressive  pain  in  the  head,  unilateral  or  bilateral, 
localized  or  general,  which  develops  \eiy  gradually  from  heaviness  to 
dulness,  to  pain  that  is  splitting,  and  is  accompanied  or  more  often 
preceded  by  characteristic  visual  signs,  such  as  scotomata,  flying  specks, 
or  partial  blindness.  Chilliness,  depression,  and  sensory  disturbances, 
particularly  in  the  stomach,  and  which  may  lead  to  nausea  or  vomiting, 
are  also  usually  present.  An  attack  may  be  terminated,  after  a  few 
minutes,  by  vomiting,  or  it  may  persist  hours  or  even  days.  After  a 
variable  length  of  time,  usually  following  a  heavy  sleep,  the  patient 
regains  his  previous  condition  of  well-being.  Nearly  every  one  has  an 
attack  or  attacks  of  migraine  during  his  life-time,  hence  its  extended 
description  here. 

History. — A  heritage  of  the  rich  and  the  poor,  the  great  and  the  small 
alike,  it  has  numbered  among  its  sufferers  many  of  the  master  minds  of 
all  times,  and  no  disorder  can  vie  with  it  in  richness  of  description  from 
medical  writers  who  have  been  themselves  subject  to  its  vagaries.^ 
Aretaeus  is  credited  with  having  given  the  first  description  of  migraine. 
C'elsus  gave  a  description  which,  while  not  corresponding  in  many 
details  with  what  is  now  understood  to  be  migraine,  is  nevertheless 
very  suggestive.  Caelius  Aurelianus  noted  for  the  first  time  that  the 
Greeks  called  it  hemicrania.  Lepois,  in  the  seventeenth  century, 
gave  his  personal  experiences  through  fourteen  years,  and  called  atten- 
tion to  the  fact  that  the  usual  after-effects  of  vomiting  and  sopor  might 

•  Frankl-Hochwart,  Dcutsoh.  Zeit.  f.  Nervenheilk.,  1913,  \()ls.  xlvii  and  xlviii. 

*  See  article  by  Jelliffe:  Osier,  Modern  Medicine. 


166  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

come  on  without  the  presence  of  the  headache.  Wepfer  in  the  same 
century  seems  to  have  more  clearly  appreciated  the  eye  symptoms. 

Tissot's  description,  1784,  remained  authoritative  up  to  the  appear- 
ance of  Liveing's  monograph.  On  Megrim,  Sick  Headache  and  Some 
Allied  Disorders  (1873),  although  in  the  interim  the  symptomatology 
was  becoming  richer  and  the  case  analyses  more  exliaustive.  Thus, 
Vater,  Hennicke,  and  Heberden  made  observations  upon  the  scotomata. 
Plenck,  Parry,  Wollaston  drew  from  personal  experiences  the  picture 
of  half-sided  blindness.  Schonlein  and  Romberg  introduced  the 
neuralgic  theories,  while  Dubois-Reymond,  influenced  by  the  newer 
work  of  Claude  Bernard,  developed  the  hypothesis  of  arterial  spasm 
which  Mollendorf  controverted,  and  postulated  a  sympathetic  paraly- 
sis, both  of  which  views  were  conciliated  by  Jaccoud  and  by  Eulenberg 
(1867),  who  described  angiotonic  and  angioparalytic  conditions. 

Etiology. — Migraine  is  a  vasomotor  disturbance  due  to  a  great  variety 
of  possible  stimuli  acting  upon  the  vegetative  nervous  system.  These 
stimuli  may  be  physical,  as  seen  in  attacks  following  severe  blows, 
falls,  fast  movements,  sudden  alteration  in  temperature,  of  pres- 
sure— high  mountains,  caisson,  deep  diving,  lumbar  puncture,  etc. 
They  may  be  of  chemical  origin,  nicotine,  alcohol,  endocrinological 
(adrenalin,  thyroid),  morphin,  protein  sensitization,  toxic  substances 
from  various  sources.  They  are  infrequently  of  somatic  reflex  char- 
acter, fatigue,  neuritic,  tumor  formations,  meningitis,  etc.  They 
may  be  emotional,  great  anger,  fear  (which  may  act  by  producing 
metabolism  disorders — acidosis),  disappointment,  chagrin,  which 
psychical  stimuli  may  be  conscious  or  unconscious.  One  or  more 
exciting  factors  may  cooperate.  Those  mediating  in  the  cerebral 
sympathetic  nervous  system  cause  vasomotor  spasms  and  paralyses 
with  hyperemia  and  pressure  in  the  brain  substance  and  cerebral 
ventricles  usually  of  a  temporary  and  transient  nature.  At  times  the 
pressure  produces  persistent  or  more  or  less  persistent  sequelae,  such 
as  ophthalmoplegia,  hemianopsia,  hemiplegia,  aphasia,  optic  nerve 
lesions,  etc. 

Milder  Attacks. — Incomplete  or  abortive  attacks  may  be  said  to  be 
the  rule  rather  than  the  exception  and  attempts  to  classify  the  disorder 
according  to  the  number  of  symptoms  present  offer  no  help  in  the  under- 
standing of  the  complete  picture. 

Mobius  suggests  that  the  parents  of  patients  suffering  from  migraine 
with  scotomata  often  have  suffered  from  migraine  without  scotomata, 
but  he  also  speaks  of  the  reverse  as  happening.  The  extreme  preva- 
lence of  migraine  makes  many  of  the  conceptions  regarding  its  neces- 
sary hereditary  nature  very  dubious,  and  the  extreme  variability  of 
the  individual  attacks  in  the  same  patient  makes  general  hereditary 
features  extremely  improbable.  It  is  by  no  means  infrequent  to 
find  patients  that  show  at  one  time  or  another  almost  every  symptom 
mentioned  in  the  voluminous  literature  of  migraine.  Thus  one  patient 
under  personal  observation  had  about  two  attacks  weekly  for  a  year. 
He  then  went  two  years  without  a  single  attack,  and  he  then  had 


PLATE    III 


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MJhamcaJsegm- 


schematic  Representation  of  the  Vegetative  Nerve  Innervation  of  the  Head.     (MuUer  &  Dahl.) 


OPHTHALMIC  MIGRAINE  167 

several  severe  ones  with  aphasia  and  psychical  symptoms,  interspersed 
with  abortive  attacks,  with  hardly  any  two  alike.  He  was  a  veritable 
musemn  of  migraine  attacks  in  the  fifteen  years  that  he  was  under 
observation. 

Many  families  are  known  in  which  both  parents  have  been  sufferers 
from  chronic  migraine  for  years,  and  yet  none  of  the  children,  now  in 
some  instances  over  forty  years  of  age,  have  ever  had  more  than  one 
or  two  attacks.  The  high  percentage  of  incidence  makes  it  almost 
impossible  to  calculate  a  hereditary  factor.  Again,  it  may  be  borne 
in  mind  that  as  there  are  many  kinds  of  epilepsies,  so  also  there  are 
undoubtedly  many  migraines.  Some  are  due  to  hereditary  anomalies, 
while  others  have  nothing  to  do  with  anything  of  a  hereditary  char- 
acter. Thus,  one  can  speak  of  migraines  that  are  possibly  hereditary 
and  others  that  are  not. 

The  commonest  milder  attacks  are  those  that  begin  in  the  classical 
manner,  with  chilliness,  perhaps  with  pinched  face,  and  cold  extrem- 
ities. The  patient  then  has  the  scotomata  and  wTetchedness,  depres- 
sion and  apprehension,  and  then  while  waiting  for  the  headache  he 
notices  that  it  does  not  come,  and,  although  he  may  still  have  heavi- 
ness and  a  sense  of  discomfort,  the  feeling  of  relief  is  sufficient  to 
make  him  feel  well. 

Others  have  added  the  sensation  of  prickling  in  the  fingers,  numbness 
in  the  hand  or  arm,  or  other  sensory  disturbances  without  the  headache. 
In  some  the  entire  attack  will  consist  of  a  disturbed  painful  sense  of 
discomfort,  without  sensory  symptoms,  scotomata,  or  headache,  but 
they  feel  sick  at  the  stomach,  and  have  an  attack  of  what  they  term 
"biliousness,"  which  clears  up  after  vomiting.  This  feeling  will  recur 
with  sufficient  frequency,  and  at  times  be  combined  with  such  other 
symptoms  of  a  migraine  attack,  in  its  varying  aspects,  as  to  stamp 
the  whole  process  as  a  variant  of  a  severe  attack.  Isolated  attacks  of 
vomiting  as  the  sole  expression  of  a  migraine  are  known. 

Attacks  of  scotomata  occur  alone,  without  antecedent  distress,  and 
no  after-attacks  are  noted.  These  are  not  uncommon.  Historically 
it  may  be  noted  that  Parry  and  Airy  had  such  attacks.  It  is  highly 
probable  that  the  majority  of  patients  who  have  had  many  migraine 
attacks  will  have  had  some  of  this  nature.  Attacks  of  scotomata  and 
vomiting  occur  without  headache.  In  many  on  the  contrary  headache 
is  the  only  symptom. 

Some  patients  have  attacks  of  hemiparesthesia  with  no  other 
symptoms  of  migraine.  These  generally  occur  at  night,  and  usually 
follow  severe  mental  exertion;  in  one  patient  under  observation  a 
severe  ordeal  in  playing  a  difficult  piece  of  music  will  bring  on  such  an 
attack  without  other  signs.  This  patient's  severe  attacks  are  very 
extreme,  being  associated  with  hemiedema,  hemiparesis,  hemianesthesia 
and  marked  hysteromaniacal  outbursts. 

Under  the  heading  of  equivalents,  Liveing  speaks  of  stomach  attacks 
associated  with  some  of  the  vascular  phenomena  of  migraine;  glossal 
spasms  are  also  mentioned  by  him.     Attacks  of  giddiness,  vertigo, 


168  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

intestinal  colic,  mental  anxiety  and  depression  which  occur  period- 
ically in  partial  association  with  migraine  symptoms,  are  also  noted 
as  equivalents.  There  is  need  of  further  study  of  these  isolated  phe- 
nomena associated  with  vasomotor  disturbances. 

Attempts  have  been  made  to  determine  the  relative  frequency  of 
migraine  attacks  with  and  without  the  visual  signs.  These  are  not 
overreliable,  because  of  the  vast  preponderance  of  abortive  attacks 
over  those  of  the  complete  classical  t^'pe. 

Mobius  expresses  the  opinion  that  the  percentage  of  visual  accom- 
paniments of  the  attacks  is  usually  overstated.  His  statistics  show 
130  cases,  with  14  visual  aura.  In  Liveing's  60  patients,  37  suffered 
from  scotomata.  Gowers  says  that  the  cases  are  about  half  and  half, 
with  and  without  eye  signs.  Galezowski  maintains  that  the  visual 
aura  migraines  appear  later  in  life,  thirty  to  fifty  years,  than  ordinary 
migraines. 

It  is  difficult  to  state  an  individual  position,  the  results  of  personal 
inquiries  having  been  so  diverse.  Close  questioning  has  revealed 
the  fact  that  at  some  time  or  other  in  the  course  of  the  disease  the 
majority  of  patients  have  had  visual  symptoms,  and  it  is  not  improb- 
able that  the  usual  statistics  are  largely  derived  from  studies  of  too 
few  attacks,  i.  e.,  largely  from  the  severer  attacks  only.  Some  notes 
on  individual  histories  are  of  interest.  Several  patients  have  kept 
fairly  accurate  records  of  their  migraine  attacks  for  several  years. 
One  shows  168  attacks  in  a  period  of  about  ten  years;  of  these,  about 
100  were  milder  attacks,  the  vast  majority  of  which,  60  per  cent,, 
consisted  of  scotomata  alone.  Of  the  68  remaining  attacks,  about 
50  per  cent,  were  ordinary  hemicrania,  lateral  or  bilateral,  without 
scotomata,  the  others  ophthalmic  migraine,  usually  unilateral  and  with 
scotomata.  Not  one  of  the  attacks  was  ever  accompanied  by  vomit- 
ing. Two  were  associated  with  aphasia,  fifteen  with  sensory  tactile 
associations;  there  were  five  or  six  attacks  of  hemiparesthesia,  one 
in  the  daytime,  the  rest  at  night.  Spasms  of  the  orbicularis  were  a 
common  accompaniment.  Every  attack  sufficiently  severe  to  require 
an  analgesic  was  promptly  relieved  by  from  5  to  10  grains  of  either 
antipyrin,  acetanilid,  or  phenacetin. 

Severe  Migraine, — Early  Symptoms. — These  may  be  termed  pre- 
cursors of  a  full  attack  of  migraine,  or  they  may  constitute  the  symp- 
toms of  an  abortive  attack.  The  most  striking  are  a  sense  of  heaviness, 
with  yawning,  chilliness,  dizziness,  or  depression,  motor  twitching, 
even  sharp  spasmodic  closure  of  the  eyelids,  sensory  phenomena, 
chiefly  paresthesia,  occasionally  anesthesia,  and  affections  of  the 
eyes  or  other  sensory  organs,  ringing  in  the  ears,  blowing,  whistling, 
modifications  of  taste,  of  smell,  of  touch,  etc.  There  may  be  failure 
of  appetite,  constipation,  diarrhea,  vascular  instability,  hot  flashes 
chasing  here  and  there  over  the  body,  throbbing  in  the  carotids,  etc. 
The  temporal  arteries  are  often  smaller,  the  saliva  diminished,  and 
the  pupils  narrowed. 

The  premonitory  signs  which  show  a  great  deal  of  variability  in 


PLATE    IV 


From  Dr.  Hubert  Airy's  Paper  on  a  Distinct  Form  of  Transient 
Hemiopsia,  Philosophical  Transactions,  1870,  p.  247. 

Figs.  1  to  4. — Early  stages  of  Fortification  Spectra  as  seen  in  dark.     0  =  sight  point. 

Figs.  5  to  8. — Similar  series,  beginning  lower. 

Fig.  9 — Fully  developed.     i?  =  secondary  attack  within.  ^ 


OPHTHALMIC  MIGRAINE  169 

different  individuals,  and  also  in  different  attacks  in  the  same  indi- 
vidual, may  be  felt  several  minutes  before  the  attack,  in  some  rare 
instances  even  days.  This  is  frequently  the  case  in  women  in  whom 
the  onset  of  the  menstrual  function  seems  to  bear  some  relation  to  the 
attack.  The  ordinary  depression  felt  at  this  time  is  a  thing  apart 
from  this  special  type  of  depression  that  pervades  them.  At  times 
such  attacks  of  depression  and  anxiety,  combined  with  a  sense  of 
chilliness  and  dizziness,  will  constitute  the  entire  picture  of  the  abor- 
tive attack.  Many  attacks  come  apparently  without  the  slightest 
warning. 

Many  patients  having  attacks  at  night  find  themselves  heavy  and 
tired,  with  sore  spots  on  the  scalp  in  the  morning.  ]M6bius  relates  a 
case  in  which  the  patient  dreamed  of  having  swallowed  a  rabbit, 
which  ate  its  way  out  through  the  stomach  wall.  After  this  unpleasant 
dream  the  patient  had  a  severe  migraine  on  awakening. 

In  all  probability  premonitory  s\Tiiptoms  of  some  type  are  invariably 
present;  when  thought  to  be  absent  it  is  because  the  patient  has 
overlooked  them,  either  by  reason  of  their  mild  character,  because  the 
symptoms  appeared  in  a  dream,  as  in  Mobius's  patient,  or  because 
of  naturally  poor  powers  of  observation.  Many  patients,  who  have 
had  headaches  for  years,  have  never  noticed  their  one-sided  localization 
or  the  well-known  fortification  spectra,  until  their  attention  has  been 
directed  specifically  to  them.  ]Many  patient's  will  deny  ever  having 
had  zig-zags  of  light,  etc.,  until  shown  Airy's  pictures,  when  they 
remember  having  seen  such  phenomena.  It  is  because  of  such  poor 
observation  that  many  cases  of  true  migraine  are  overlooked,  which 
fact  lends  further  support  to  the  belief  that  this  disorder  is  very  much 
more  prevalent  than  is  usually  supposed. 

Sensory  Symptovis. — In  the  more  classical  attacks  the  patient  has 
preliminary  sensory  symptoms.  These  are  spoken  of  by  Mobius  in 
the  sense  of  an  aura.  If  the  term  aura  be  used  as,  for  instance,  the 
term  "fever"  is  used,  there  can  be  no  objection,  but  if  by  an  aura  is 
meant  a  restricted  phenomenon  essentially  related  to  an  epileptic 
aura,  the  term  should  be  eliminated. 

A  sense  of  coldness  and  chilliness  is  one  of  the  commonest  sensations. 
This  is  usually  general,  and  is  associated  with  a  pale  countenance, 
gooseflesh,  perhaps  clammy  hands,  and  a  sense  of  misery.  Cases 
are  known,  and  are  by  no  means  uncommon,  in  which  the  chilliness 
has  been  one-sided,  and  is  accompanied  by  other  phemonena  involving 
one-half  of  the  body,  including  the  face,  of  the  same  side.  Yawning  is 
a  common  early  sign. 

Unilateral  paresthesia  is  not  an  uncommon  early  sign.  Many 
patients  note  a  tingling  or  numbness  in  the  fingers  of  one  hand;  this 
may  spread  up  the  arm,  and  in  rare  instances  general  unilateral  pares- 
thesia of  a  very  uncomfortable  nature  may  be  i)resent.  In  some 
instances  such  unilateral  paresthesias  have  constituted  the  sole  symp- 
tom of  an  attack,  save  for  the  heaviness  and  usual  discomfort.  Occur- 
ring at  night,  such  attacks  are  often  extremely  wearing,  keeping  the 


170  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

patient  awake.  Photophobia,  flow  of  tears,  strange  sounds,  tinnitus, 
peculiar  odors,  queer  peppery  or  flat  tastes,  may  be  noted. 

Anesthesia  is  less  often  observed,  largely  because  of  the  negative 
character  of  the  symptom.  When  involving  the  face  or  mouth  it  is 
complained  of.  Anesthesia  frequently  follows  the  tingling  of  the  early 
paresthetic  disturbances.  Franz^  has  shown  that  there  is  a  very 
evident  decrease  in  the  pain  threshold,  especially  after  the  headache 
has  set  in. 

The  visual  phenomena  are  the  most  striking,  and  hence  held  to  be 
of  the  most  frequent  occurrence.  The  ease  of  observation  in  part 
accounts  for  the  usually  accepted  opinion  that  they  are  the  commonest 
of  the  early  symptoms.  Very  few  individuals  have  been  subjected  to  a 
careful  sensory  examination.  If  more  were  investigated,  it  is  probable 
that  other  slight  sensory  signs  would  be  found  to  be  equally  prevalent 
and  as  evanescent.  The  visual  signs  have  been  described  by  many 
writers,  and  many  illustrations  have  been  made  showing  their  chief 
characteristics.  The  extreme  uniformity  of  their  general  character  is 
striking,  as  well  as  the  variations  of  the  same  pattern. 

As  a  rule  the  patient  notices  a  slight  blurring  of  his  vision  if  reading 
or  a  slight  flicker  of  light  located  in  one  eye,  to  one  side  of  the  center. 
Closer  observation  reveals  either  a  slight  cloudy  spot,  which  seems 
to  follow  the  eye  in  reading,  cutting  out  the  after-images,  or  a  letter 
or  so  from  the  center  of  clear  vision.  The  slight  subjective  sense  of 
difficulty  in  reading  may  precede  the  discovery  of  a  scintillating  spot 
which  becomes  visible  on  closing  the  eyes.  Little  by  little  this  spot 
spreads  out,  usually  in  a  crescent-like  fashion.  General  statistics  are 
thus  far  unavailable,  but  a  special  study  has  shown  that  the  majority 
of  these  scotomata  have  begun  in  the  left  eye,  are  situated  to  the  left 
of  the  middle  line,  w^th  the  convexity  of  the  crescentic  border  to  the 
left.  As  the  crescent  gradually  grows  larger,  the  difficulty  in  seeing 
clearly  becomes  more  marked,  especially  on  the  periphery  of  the  visual 
field.  For  most,  the  scotomata  are  in  constant  motion,  flashing  in  its 
spectral  zig-zag  fashion,  thus  causing  the  classical  name  "fortification 
spectrum"  from  the  play  of  colors,  and  the  fortress-like  "ins  and  outs" 
of  the  outline. 

After  a  variable  time,  from  five  to  twenty  minutes,  the  scotomata 
gradually  subside,  or  suddenly  disappear,  to  be  followed  by  the 
headache.  Not  infrequently  the  headache  never  comes,  and  the  pre- 
liminary sensory  phenomena  of  chilliness,  heaviness,  and  scotomata 
constitute  an  abortive  attack.  A  description  of  the  scotomata  of 
migraine  might  fill  a  volume.  The  classic  of  Liveing  reproduces  the 
excellent  illustration  of  Airy's,  which  is  here  reproduced. 

Occasionally  the  right  half  of  the  field  is  involved.  Sometimes  it  is 
the  upper  half,  one  of  Mobius's  patients  saying  that  everybody  seemed 
headless.;  occasionally,  it  is  the  lower.  In  rare  instances  the  patients 
complain  of  total  blindness,  i.  e.,  central  scotomata.    Berbez  reports 

'Amer.  Jour.  Physiol.,  1905. 


OPHTHALMIC  MIGRAINE 


171 


an  interesting  case  of  a  ring-like  scotoma — ^the  patient,  on  looking 
at  his  watch,  could  see  only  the  central  pin  where  the  hands  were 

THE  DEVELOPMENT   OF  SCOTOMATA   IX  MIGRAIXE   WHILE   READIXG. 


have  not  studied  immigrati 
:ers  into  which  immigrati 
esire  for  larger  opportuniti 
always  h^pA  the  prime  fore 
,  This  'jMjr^-fnas  proved 
ie  any  reg^tion  of  immigr 
in  for  the  selection  of  imni 
'  desirable  it  is  to  apprecia 
colonization  and  to  examif 
Kij  place  in  the  causes  at 


There  has  been  no  sue 
the  United  States  as  tW 
longingfor  liberty  and  fl 
for  lai^Qmmigration  at 
settlen»H  gt  America;  i 
Island  yjfce  present  tim 
and  th^frontiersman  of : 
that  a  very  large  numbe' 
lured  by  purely  economic 
rights,  many  immigrants 


Fig.  64. — Stage  of  blurring  three  minutes. 


Fig.  65. — First  outlines  of  scintillations. 


>.t7Z>    cUMi,     fW   ) 


'  utnujix  xn 


questions  is  usually  introducec 
were  jmmigrantg  and  the  chi 
following  theCtomial  period . ' 
their  kinsm^^^hat  immigrab 
the  West^^fl|?Bat  we  are  indel 
iiidusjtrian^fl  conylercial  gre 
ments  ar^^and  ifis  difficul 
owes  to  ^H^ants.  It  is,  h< 
of  presen#5fiQig:ration  eis  sin 
inspired  by  iikfcse  longing  fo 
find,  it  even  at  the  cost  of  da 
part  of  the  civiUzation  of  the 
period  and  m  the  years  foUowi 


Fig.  66.- 


-Five  to  ten  minutes  growing 
scotoma. 


tiiu>.      i-'ibuussion  01  immigratjon 
statements  that  our  forefathers 
century 
(ig  to  join' 


»f .  inpnigrants, 
y.' new  general 


e  back  the  I 
efly  lo  immi, 

•For  the 
rstate  the  d 

a  very  serii 
continuation 
oal  liberty  am 
d  privation,  whii 
orld  to  the  New  in 


and  peopled 

or  our  present 

;  thesAstate-' 

'""^'i  country 

conceive? 

t  Hiovemefat, 

rminatien  tff 

Ted  a 

lonial 


Revolutionary  War.    Such  a  con* 
Fig.  67. — Ten  to  fifteen  minutes. 


!?^ 


I  had  J 


twe 


of  arrivals  at  Ellis 
.    The  addition 
lie  racial  or  econi 

those  wto 
ship  as  weQ  as 
jg  the  next 

of  immigran 
in  Ireland  in 
t  illustratio 
ms  in  Euro 
America.   It 
•eloped  by  i 
definite  relatio 
the  number  of 
ji  Europe.    T 
on  on  account 
"Dsiderable  ext 
It  it  was  influenced 


igle  mont 
aliens 
iges.    Nearly 
_7fore  and  many  ^ 
'those  with  whom  tt 
iars  there  was  a  ste« 
the  latter  part  of 
iddenly  caused  a  larg 
unportant  new  social 
..  definite  relation  to  tn 
ieny  years  later  than  ai 
Slion— that  econbmic  p 
>te>e  volume  of  immigra 
this  country  who  r 
TOm  Ireland  WB! 
•epftte  potato  c 
ihropic  indiv 
tooT'by%We1ame  motiv 


itedout,  the  English, Di 
1  wl\'<^S^tyt&^  practically  all  arrivals  i 
roluntionary  War  were  clos 
one  Germanic  race  in  tl 
ley  colonized  the  Atlantic' 
the  thin  line  of  civilizat 
of  the  same  race.    The  "imir 
ir^  many  years  after  the  RevMi 
,„    j£an  those  who  had  preceded  thei 
wA^ve  considered  as  that  of  contin 
he  Civil  War.   During  that  period  ab 
4k,  It  is  not  possible  to  learn  the  > 
Ills  country  before  1820  for  in  i 
to  record  their  number  and  to  a: 
'e  know,  however,  that  the  popi 
ally  increased  by  immigratic 
oktionary  War.    During 


Fig.  68. — Fifteen  to  twenty  minutes. 


Fig.  69. — Just  before  disappearing, 
twenty  to  thirty  minutes,  and  begin- 
ning of  headache.  (Jelliffe;  personal 
observation.) 


united;  the  figures  on  the  dial  were  all  obscured  by  the  scintillating 
scotoma.    These  scotomata  are  usually  bilateral  phenomena.    They 


172  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

may  begin  in  one  eye  before  appearing  in  the  other,  and  be  some- 
what different  in  the  two  eyes,  and  may  disappear  in  one  eye  sooner 
than  the  other.    Scotomata  limited  to  one  eye  are  probably  rare. 

The  retinal  appearances  during  the  time  of  these  scotomata  are 
uncertain.  Blanching  of  the  papillte  has  been  observed  by  some 
(Galezowski) ;  pulsation  of  the  retinal  arteries,  with  dilatation  by 
others.  Personal  experience  has  shown  similar  dilatation  in  a  few 
cases,  but,  as  a  rule,  a  normal  fundus  is  found.  The  picture  seen 
will  depend  upon  the  stage  of  the  attack  and  its  severity. 

Pupillary  dilatation  occurs  late.  Slight  irregularity  of  the  pupils 
during  a  severe  attack  of  an  ophthalmic  migraine,  dilatation  being 
usual  on  the  affected  side,  is  not  unusual.  Bilateral  pupillary  con- 
traction is  the  rule  in  the  headache  stage. 

During  the  onset  of  the  fortification  spectra  it  not  infrequently 
happens  that  mild  motor  phenomena  occur  in  the  eyelid  of  the  side 
to  be  affected.  The  eyelids  droop  a  little,  and  Gowers  and  others 
report  double  vision,  interpretable  as  a  sign  of  paresis  in  an  ocular 
muscle. 

Motor  Disturhances. — Speech. — This  may  be  considered  as  both  a 
motor  and  sensory  phenomenon,  for  the  most  frequent  type  of  change 
is  a  transitory  sensory  aphasia.  Anarthrias  are  known,  especially  in 
the  ophthalmoplegic  variety,  but  for  ophthalmic  migraine  the  type 
of  aphasia  found  is  very  characteristic.  As  described  by  Charcot, 
it  is  an  intermittent  halting  aphasia.  At  one  moment  the  patient  can 
get  the  right  word,  at  the  next  he  cannot.  He  stumbles  on  a  word; 
uses  madame  for  monsieur,  etc.  In  Liveing's  cases  15  out  of  20  had 
speech  disturbances;  one  on  hearing  clock  bells  was  unable  to  inquire 
what  they  were.  Fere  cites  the  case  of  a  coachman  who  forgot  where 
he  was  going  to  drive  his  passengers;  Berbez  a  like  case  in  which  a 
pedestrian  lost  his  way,  as  he  could  not  read  the  street  signs  under- 
standingly.  Gowers  speaks  of  a  case  of  word-deafness.  Cases  of 
agraphia  are  also  known.  Mobius  reports  a  case  with  t\^ical  scintillat- 
ing scotomata  at  one  time  on  the  right  side,  at  another  on  the  left. 
When  the  patient  suffered  from  a  right-sided  scotoma  he  had  sensory 
aphasic  signs,  but  they  were  not  present  when  the  scotoma  was  on 
the  left  side. 

Other  observers  have  noted  the  same  phenomena,  while  contra- 
dictory observations  are  also  recorded.  The  speech  disturbance 
sometimes  resembles  a  paraphasia,  the  patient  using  a  jumble  of 
words.  In  a  personal  case  the  patient  could  not  sing  a  well-known 
tune  correctly,  his  sense  of  musical  values  having  been  interfered 
with. 

The  onset  of  the  aphasic  disturbance  may  var\'  greatly.  It  is 
usually  temporary,  persisting  at  times  for  only  a  few  minutes,  again 
persisting  a  few  hours.  It  frequently  antedates  the  headache,  or  is 
coincident  with  it.  In  a  case  reported  by  Meige  the  aphasia  persisted 
as  long  as  the  headache,  and  disappeared,  as  a  rule,  when  that  dis- 
appeared.   The  patient  showed  a  loss  of  ability  to  say  certain  words 


OPHTHALMIC  MIGRAINE  173 

and  a  tendency  to  the  emplo^'ment  of  incorrect  words.  There  was  no 
anarthria. 

Cerebellar  Symptoms. — Oppenheim  has  called  attention  to  a  cere- 
bellar hemicrania  in  a  patient  in  whom  every  attack  of  migraine  was 
accompanied  by  tj^pical  cerebellar  symptoms.  The  patient  was 
uncertain  in  his  gait,  walked  like  a  drunken  man,  was  dizzy,  and  had 
the  sensation  that  his  body,  or  individual  parts  of  it  were  doubled. 
The  sense  of  equilibrium  was  disturbed  in  each  attack.  Dizziness 
and  loss  of  the  sense  of  equilibrium  are  not  infrequent  but  such  a 
complete  syndrome  has  been  described  only  by  Oppenheim. 

Paralytic  Phenomena. — Attention  has  already  been  called  to  the 
rare  occurrence  of  hemiparesis,  which  may  even  involve  the  facial 
muscles.  Up  to  the  present  time  no  instances  of  crossed  hemiplegic 
t^-pes  have  been  found  in  the  literature.  This  is  of  interest  in  con- 
nection with  the  hypothesis  of  the  bulbar  origin  of  migraine,  especially 
of  the  ophthalmoplegic  variety.  Other  palsies  are  known,  monoplegias 
of  the  extremities,  ophthalmoplegias,  etc.  Topical  and  minute  brain 
swellings  may  produce  a  great  variety  of  paralytic  phenomena  usually 
of  a  transitory  nature  but  at  times  persisting. 

Headache. — This  is  the  most  common  feature  and  exhibits  a  great 
amount  of  variability  as  to  location,  quality,  intensity,  and  duration. 
In  the  more  classical  attacks  the  headache  begins  on  the  average  about 
fifteen  to  thirty  minutes  after  the  appearance  of  the  scotomata  or  other 
sensory  phenomena.  It  frequently  begins  on  one  side,  and  may  remain 
so  or  become  bilateral.  As  a  rule  it  is  frontal,  or  occupies  the  vertex, 
but  may  involve  the  temporal  regions,  the  occiput,  sometimes  as  low 
down  as  the  neck.  Gower's  experience  points  to  the  parietal  region  as 
being  oftenest  affected,  and  usually  over  a  small  area.  Henschen,  in 
123  patients,  shows  the  pain  to  have  been  located  110  times  in  the 
forehead,  100  times  in  the  parietal  region,  and  54  times  in  the  occiput. 
There  is  usually  pain  over  the  eyes,  and  the  eyeballs  are  usually  painful 
to  pressure.  In  a  few  instances  pressure  over  the  malar  bones  is 
painful,  and  occasionally  there  is  a  well-marked  jawache. 

Statistics  of  the  percentage  of  different  locations  are  uncertain  since 
one  individual  will  have  all  the  different  varieties.  Thus,  in  a  case 
already  cited,  in  which  the  abortive  attacks  were  so  frequent,  the 
headaches  comparatively  rare,  the  strictly  unilateral  headaches  were 
only  5  per  cent,  of  the  entire  number.  In  others  the  hemicranic  type 
runs  much  higher.  In  Henschen's  records  of  123  cases,  56  had  one- 
sided attacks,  in  67  both  sides  were  involved.  In  Liveing's  61  patients, 
17  had  one-sided  attacks,  in  7  the  attacks  were  variable,  while  in  34 
both  sides  were  involved.  Mobius  and  others  note  that  the  headache 
often  appears  on  the  side  opposite  to  that  affected  by  the  sensory 
aura.  Personal  studies  do  not  confirm  Mobius's  statement.  It  does 
seem,  however,  as  first  noted  by  Liveing,  that  one-sided  sensory 
symptoms  are  oftener  accompanied  by  one-sided  than  by  bilateral 
headaches.  With  bilateral  sensory  phenomena,  scotomata,  etc., 
bilateral  pains  are  the  commonest. 


174  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

In  many  attacks  the  pains  are  limited  to  the  eyes,  the  feehng  of 
soreness  of  the  eyeballs  being  so  very  marked  that  it  is  painful  to  move 
them.    Pain  in  the  neck  may  also  cause  the  desire  to  hold  the  neck  rigid. 

The  relationship  of  these  pains  to  psychical  symbolizations  has  not 
yet  been  investigated.  In  a  few  cases  thus  far  analyzed,  left-sided 
symptoms  are  apt  to  symbolize  the  unconscious  sex  conflicts,  right- 
handed  ones,  the  nutritive.  Pains  in  the  back  of  the  head  are  fre- 
quently associated  with  unconsciously  repressed  hate  complexes,  as  are 
also  jaw  pains. 

The  character  of  the  pain  defies  analysis,  since  descriptive  phrases  are 
used  in  such  various  ways  by  different  observers.  In  some  attacks,  the 
head  simply  feels  slightly  sore,  or  heavy,  or  dull,  or  thick;  "like  a 
block  of  wood,"  is  a  frequent  expression.  "Filled  with  sawdust," 
one  patient  says.  Again,  the  pain  is  agonizing,  impossible  to  describe. 
Some  patients  shriek  with  the  pain,  become  hysterical,  and  roll  about 
the  floor,  grasping  the  head  between  the  hands,  wishing  to  beat  their 
brains  out.  Between  these  extremes  numberless  variants  are  found 
among  different  individuals,  and  in  different  attacks  in  the  same 
individual.  Nearly  all  patients  will  say  that  the  severe  pains  are 
throbbing  or  thumping,  usually  indicating  great  pressure  from  within 
or  without;  as  Mobius  has  said,  "some  patients  think  the  head  will 
burst,  others  that  it  is  being  squeezed  in  a  vise."  Descriptions  of 
bursting  are  more  common.  The  pain  is  an  all-pervading  one,  gradu- 
ally mounting  to  a  maximum,  then  running  along  continuously  with- 
out any  let-up,  with,  at  all  times,  sudden  accessions,  especially  on 
movement,  if  one  leans  over,  or  is  forced  to  sudden  exertion.  In  but 
the  rarest  instances  is  it  described  as  lancinating  in  quality.  It  is 
the  type  of  pain  apparently  seen  in  cerebral  tumor,  in  acute  hydro- 
cephalus, in  cerebrospinal  meningitis,  and  is  allied  to  the  pain  of 
opium  poisoning,  or  of  sea-sickness;  all  pointing  in  the  direction  of  a 
modification  of  intracerebral  pressure,  at  times  an  increase,  or  it  may 
be  a  decrease,  either  of  which  may  cause  severe  pain.  Occasionally 
the  phenomenon  of  a  bilateral  headache  with  marked  predominance 
of  one-sided  pain  will  be  observed. 

The  severity  of  the  pain  may  be  conditioned  by  a  number  of  factors. 
Movement  uniformly  increases  it.  Bending  over  becomes  impossible. 
The  first  movement  on  lying  down  is  usually  accompanied  by  a  sudden 
rise  in  severity,  but  this  gradually  subsides.  The  taking  of  alcohol, 
usually  increases  the  severity  of  the  pain,  as  does  also  the  use  of  tobacco. 
Eating,  if  possible,  may  help  somewhat,  but  usually  augments  the 
pain,  and  is  avoided.  Strong  sensory  impressions  invariably  increase 
the  pain.  Noises  of  various  kinds  often  aggravate  the  pain  tremen- 
dously and  cause  certain  patients  marked  distress.  The  "Fourth  of 
July"  in^'ariably  dri^'es  many  migrainous  patients  to  some  quiet  spot 
in  the  country,  free  from  crackers  and  bombs.  Mobius  notes  that 
the  rage  of  migrainous  parents  directed  toward  their  noisy  children 
often  resembles  a  pathological  hatred.  Strong  light  is  invariably 
avoided,  because  of  its  tendency  to  increase  the  pain.    The  movements 


OPHTHALMIC  MIGRAINE  175 

of  the  eyeball  and  attempts  at  visual  accommodation  cause  an  increase 
in  the  pain. 

Psychical  effort  is  often  impossible ;  in  milder  attacks  the  awakening 
of  a  strong  mental  stimulus  may  make  one  forget  the  pain.  Mobius 
says  that  his  attacks,  usually  light  ones,  are  frequently  forgotten  during 
an  interesting  visit  to  the  Polyclinik,  to  be  once  more  prominent 
afterward.  One  of  us  had  frequently  begun  a  lecture  with  a  severe 
migraine  to  find  it  almost  forgotten  until  the  close,  when  it  reappears, 
usually  with  renewed  vigor. 

The  movements  of  straining  at  stool,  and  vomiting,  coughing,  etc., 
invariably  cause  a  rapid  and  sharp  rise  in  the  severity  of  the  pain. 
Sensory  stimuli  may  have  an  unpleasant  effect  on  the  psyche.  Thus, 
certain  odors  cause  distress;  the  smell  of  cooking  acts  much  as  it  does 
on  shipboard;  it  accelerates  vomiting.  Certain  skin  phenomena,  such 
as  sore  spots,  are  frequent  after  the  headaches. 

In  certain  personal  experiments  with  drugs  the  following  have 
invariably  increased  the  headache  within  a  few  minutes:  A  few 
whiffs  of  chloroform  or  of  ether,  adrenalin  by  mouth,  digitalis,  stro- 
phantin,  and  ergot.  Drugs  that  raise  the  blood-pressure,  in  general, 
increase  the  pain  when  taken,  especially  at  the  beginning  of  the  head- 
ache. The  headache  may  clear  away  very  suddenly  after  an  attack 
of  vomiting,  or  it  may  pass  without  vomiting;  in  some  it  fades  away 
gradually.  It  may  last  a  few  minutes,  a  few  hours,  or  a  few  days. 
Some  cases  of  what  jNIobius  chooses  to  call  status  hemicranicus  are 
recorded. 

Vasomotor  Disturbances. — Practically  all  attacks  of  mi'i-raine  are 
accompanied  by  visible  vasomotor  disturbances.  In  most  cases 
vasoconstrictor  phenomena  (coldness,  paleness,  gooseflesh,  etc.)  pre- 
cede, to  be  followed  later  by  vasodilator  changes.  Thomas  and  Cornu 
both  point  this  out  as  a  result  of  their  experiences.  Thomas  con- 
tributes a  statistical  study  of  107  cases  in  support  of  the  early  pallor, 
small  pulse  and  coldness,  which  pass  over  to  the  phenomena  of  warm, 
red,  flushed  face  and  skin,  and  full  pulse.  The  period  of  initial  con- 
striction may  be  unnoticed  by  reason  of  its  transitory  character.  In 
some  instances  this  initial  vasoconstriction  may  be  very  marked  and 
give  rise  to  the  phenomena  of  localized  cyanosis,  even  advancing  to 
the  picture  of  the  constriction  phase  of  the  Raynaud  disease  type. 
(Vagotonic.) 

In  the  same  manner  the  secondary  vasomotor  dilatation  may 
pass  the  bounds  ordinarily  observed  and  lead  to  localized  edema, 
to  the  erythromelalgic  type,  or,  exceptionally,  to  hemorrhagic  phe- 
nomena in  the  conjunctiva,  ocular  tissues,  or  even  in  the  walls,  of  the 
stomach.     (Sympathicotonic.) 

Secretions. — Alteration  in  secretory  functions  are  frequently  observed 
early  or  late  in  the  attacks.  Reference  has  been  made  to  the  excess 
of  secretion  of  tears  as  a  frequent  precursor.  Vomiting  of  frothy 
mucus,  serous  diarrhea,  increase  of  sweat,  coryza  (Calmeil),  or  inces- 
sant salivation  (Liveing,  Tissot),  are  common  phenomena. 


176  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

The  changes  in  urinary  secretion  have  attracted  careful  attention. 
The  early  vasoconstriction  of  the  periphery,  coldness,  lack  of  secretion 
of  perspiration,  etc.,  account  in  a  purely  mechanical  way  for  the 
increase  of  urinary  secretion  in  the  early  stages.  Metabolic  studies 
show  no  fundamental  disturbances.  Bioglio  was  unable  to  show 
constant  changes.  Although  it  is  not  possible  to  exclude  metabolic 
disturbance  as  causing  changes  in  vegetative  control,  probably  it  is 
more  true  that  emotional  influences  cause  the  metabolic  disturbance. 

Trophic  Disturbances. — These  have  been  reported  by  several 
observers.  Cornu  says  that  nearly  all  of  his  cases  of  migraine  show 
facial  asymmetry,  and  facial  atrophy  is  recorded.  These  instances 
are  nearly  always  coincidences  and  are  not  necessarily  attributes 
of  the  migraine.  A  facial  atrophy  which  can  be  interpreted  only  on 
the  basis  of  a  migrainous  disturbance  of  the  vasomotor  apparatus  is 
very  problematic,  and  certainly  Cornu's  results  are  not  confirmed 
by  others.  Loss  of  weight  in  the  severe  rapidly  recurrent  cases  is  due 
to  disturbance  in  general  nutrition  due  to  gastric,  rather  than  to  other 
causes.    Herpes  is  a  not  infrequent  accompaniment  in  some  patients. 

Psychical  Dishtrhances. — These  have  been  noted  by  many  observers, 
Liveing  being  one  of  the  first  to  point  out  the  relationship  of  disturbed 
psychical  states  to  the  attacks  of  migraine. 

In  the  majority  of  migraine  attacks  there  are  few  conscious  mental 
changes  before,  during,  or  after  the  attacks.  Mild  depression,  hope- 
lessness, despondency  with  clear  consciousness,  are  frequent  mental 
states.  With  very  severe  pains  Mobius  admits  clouding  of  conscious- 
ness, and  is  not  sure  that  severe  stuporous  states  are  not  due  to  Dain 
as  well.  Mingazzini,  on  the  other  hand,  believes  there  is  justification 
for  erecting  a  special  group,  which  he  has  termed  the  hemicranie 
dysphrenias,  and  distinguishes  a  transitory  and  a  more  permanent 
variety.  Recent  observers  are  practically  in  accord,  in  showing  that 
severe  mental  disturbances  varying  in  character  and  intensity  may 
be  part  of  a  migraine  attack. 

Guidi  has  amplified  these  observations  by  reporting  the  history  of 
a  number  of  cases  in  which  the  patients  suffered  during  the  day  before 
the  onset,  in  a  much  more  decided  manner  than  by  feelings  of  anxiety 
or  depression  as  described  by  Liveing.  Thus  Guidi  calls  attention 
to  grave  alterations  in  the  psychical  state  of  a  number  of  his  patients. 
In  one  the  entire  character  of  the  personality  would  change  preceding 
the  attack.  A  patient  who  had  always  been  calm,  reserved,  quiet 
and  modest,  suddenly  became  much  agitated,  was  forward,  noisy, 
and  loquacious,  and  told  salacious  stories,  which  was  far  from  his 
usual  behavior.  While  in  health  a  spare  eater,  preceding  an  attack 
he  suddenly  became  hungry  and  hankered  especially  for  starchy  foods. 
During  the  attack  the  patient  had  glycosuria,  which  disappeared 
later. 

With  the  onset  of  pain  the  picture  is  less  clear,  yet  there  is  little 
doubt  that  many  patients  suffer  from  profound  psychical  disturb- 
ances, which  arise  independently  of  the  pain.    One  such  case,  under 


OPHTHALMIC  MIGRAINE  111 

personal  observation,  would  be  interpreted  by  Mobius,  and  rightly 
so,  as  one  in  which  the  pain  is  the  first  link  in  a  hysterical  reaction. 
But  there  are  other  cases  which  do  not  belong  to  this  group.  Mingaz- 
zini's  hemicranic  dysphrenias  may  be  cited  as  examples,  in  part,  at 
least.  In  others  severe  disturbances  have  occurred,  such  as  states  of 
anxiety,  rising  to  actual  anguish  (Charcot);  phobias  o_f  inability  to 
perform  acts  (Cornu-Charcot) ;  terror  (Liveing,  Fere,  Kraft-Ebing) ; 
hallucinations  of  sight  (phosphenes,  colored  lights,  animals)  and  hear- 
ing with  mental  confusion  (Forli,  ]Mingazzini) ;  maniacal  excitement 
(Mingazzini,  Jelliffe)  and  stupor;  unconsciousness  (many  authors). 

Liveing  reports  that  25  per  cent,  of  his  cases  showed  psychical 
s}Tnptoms.  The  Italian  observers  record  fewer,  but  it  appears  that 
at  least  from  10  to  15  per  cent,  of  the  cases  of  grave  hemicrania  show 
some  distinct  mental  disturbance  in  some  one  or  more  of  their  attacks 
which  is  more  significant  than  the  usual  depression  which  is  so  uni- 
versal. These  severe  t^qjes  only  emphasize  the  fact  that  unconscious 
conflict  is  an  important  etiological  factor  in  the  migraines. 

Symptomatic  Headaches. — The  occurrence  of  migraine-like  attacks 
accompanying,  or  due  to,  definite  disease  conditions,  notably  organic 
disease  of  the  brain,  is  well  known.  The  association  of  migraine  with 
gout  and  malarial  affections  has  been  noted.  It  is  very  well  known 
that  attacks  of  migraine  may  be  very  frequent  during  the  continuance 
of  a  malarial  infection.  Such  may  disappear  for  months  after  quinine 
therapy,  and  then  reappear  at  the  time  of  a  later  malarial  infection. 

I\Iigraine-like  attacks  are  not  infrequent  in  cerebral  tumor;  they 
may  appear  periodically,  as  in  cases  fully  reported  by  Abercrombie 
and  ]\Iobius,  or  they  may  be  continuous  and  distinguishable  with 
great  difficulty  from  the  pain  of  tumor,  as  in  cases  reported  by  Wer- 
nicke, who  has  said  that  such  attacks  may  be  quite  readily  confused 
with  those  more  typical  of  tumors.  In  tumors,  however,  vomiting 
brings  little  or  no  relief;  quiet  gives  less  relief,  and  the  fluctuation  in 
intensity  of  the  pain  is  less  prominent.  A  primary  onset  of  migraine- 
like attacks  in  adult  life  should  always  awaken  the  suspicion  of  an 
organic  brain  lesion. 

The  headache  of  pituitary  disease,  either  as  a  pure  pituitary  syn- 
drome, or  arising  from  functional  hyperplasia  of  the  pituitary  as  a 
compensatory  relation  in  thymic  disease,  or  from  other  tx'pes  of 
imbalanced  endocrine  function — adrenalin — pituitary  balancing,  etc., 
is  frequent.     A'-ray  study  of  the  sella  turcica  shows  this. 

Oppenheim  has  called  particular  attention  to  the  occurrence  of 
migraine-like  attacks  at  the  onset  of  tabes;  Mobius  is  inclined  to  think 
it  a  rare  combination,  and  regards  it  either  as  a  pure  coincidence 
or  a  migraine-like  neuralgia.  In  general  paresis,  migraine-like  attacks 
may  be  an  initial  symptom.  ^ligraine  attacks  are  not  infrequent 
throughout  the  early  stages  of  the  disease,  but  the  anatomical  correla- 
tions arc  still  hjiDothetical. 

Diagnosis." — The  difficulties  appear  in  the  consideration  of  ordinary 
lieadaches  and  in  neurasthenic  headaches;  in  distinguishing  between 
12 


178  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

the  scotomata  of  migraine  and  other  scotomata;  the  paresthesia  of 
migraine  and  other  paresthesias;  the  aphasia,  the  vomiting,  etc., 
as  seen  in  migraine,  and  the  same  as  due  to  other  causes.  In  most 
individuals  abortive  and  incomplete  attacks  are  the  rule,  and  it  is 
often  extremely  difficult  to  determine  their  precise  significance. 

Mobius  has  suggested  that  the  problem  is  not  only  whether  the 
case  is  one  of  migraine  or  not,  but  whether  it  is  migraine  alone,  and 
not  something  additional.  This  author's  contention  that  migraine  is 
hereditary  and  begins  in  youth,  would  seem  to  make  it  a  simple  matter, 
but  clinical  experience  shows  that  real  migraines  do  appear  in  later 
years,  apart  from  other  affections,  and  as  for  the  hereditary  factor, 
the  extreme  prevalence  of  the  affection  makes  it  hard  to  accurately 
weigh  this  factor.  The  periodic  recurrence  is  a  difficult  criterion. 
There  is  usually  no  difficulty  in  diagnosing  the  classical  attacks  from 
simple  headache,  but  at  times  such  differentiation  is  impossible.  Many 
chronic  sufferers  from  migraine  know  well  their  real  attacks,  are  able 
to  distinguish  abortive  attacks,  and  also  have  headaches  of  an  entirely 
different  nature.  The  simplest  test  in  separating  abortive  migraines 
from  simple  headaches  is  the  occurrence  of  sensory  phenomena,  other 
than  pain,  which  have  their  main  origin  in  vasomotor  disturbances. 
It  is  on  this  account  that  the  severe  headaches  following  the  use  of 
alcohol,  ether,  chloroform,  opium,  or  analogous  drugs  should  be  allied 
to  the  migraines  rather  than  to  simple  headaches.  The  headaches  of 
neurasthenia,  anemia,  syphilis,  lead  poisoning,  nasal  sinus  involvement, 
supra-orbital  neuralgia,  nephritis,  eye-strain,  glaucoma,  etc.,  should 
present  little  difficulty. 

Treatment. — The  treatment  of  the  migraine  attack  is,  for  the  most 
part,  fairly  satisfactory.  There  are  few  patients  for  whom  some 
relief  cannot  be  obtained,  both  with  reference  to  the  diminution  of 
the  number  of  attacks,  and  to  the  mitigation  of  the  severity  of  the 
attacks  themselves.  The  migraine  habit,  constitution,  or  liability — 
call  it  what  one  will — exists  in  very  varying  degrees;  in  some  a  very 
slight  disturbance  is  sufficient  to  set  free  those  forces  which  culminate 
in  an  attack;  for  others  it  requires  a  very  much  greater  maladjustment. 
If  the  general  reflex  vascular  hypothesis  be  taken  as  a  tentative  expla-- 
nation  it  is  very  readily  understood  why  the  taking  away  of  various 
forms  of  peripheral  rritation  may  result  in  eliminating  one  or  more, 
and  in  certain  instances  all,  of  the  causes  which  set  the  migraine 
reaction  in  operation. 

It  is  folly  to  shut  one's  eyes  to  the  very  evident  clinical  fact  that  a 
few  migraines  are  relieved,  if  not  entirely  wiped  away,  by  the  correction 
of  some  peripheral  disorder,  sometimes  more  than  one,  which  has  had 
definite  effect  on  the  nervous  system.  Just  what  the  interrelation 
may  be  between  the  severity  of  the  irritant  and  the  mildness  of  an 
attack  it  is  impossible  to  judge,  but  certainly  the  relief  from  eye- 
strain, from  diseased  turbinates,  from  adenoids,  from  constipation, 
from  dysmenorrhea,  from  a  number  of  minor  yet  definite  peripheral 
irritations,  will  relieve  a  certain  number  of  patients.     Perhaps  they 


OPHTHALMIC  MIGRAINE  179 

are  the  very  slight  migraines,  perhaps  not ;  one  is  not  yet  in  a  position 
to  say.  One  should  therefore  eliminate  at  the  onset  such  of  these 
structural  defects  as  are  shown  to  have  some  influence  on  the  nervous 
system.  In  denying  any  possibility  to  these  influences  in  the  causation 
of  a  migraine  attack,  one  errs  as  badly  as  when  maintaining  some  one 
of  them  to  be  the  only  and  invariable  element  in  the  case,  as  faddists 
are  doing  and  always  have  done. 

Gastro-intestinal  factors  are  closely  analogous  to  those  just  men- 
tioned. In  the  minds  of  most  clinicians,  and  certainly  as  generalized 
in  the  feelings  of  those  most  affected,  it  is  in  the  stomach,  liver,  or 
intestines  that  the  main  seat  of  the  trouble  is  to  be  sought.  The 
gastro-intestinal  factor  is  undoubted  in  many  cases;  it  may  be  exclu- 
sively gastric  or  colonic ;  perverted  chemism,  perverted  bacterial  action 
(primary  or  secondary  factors,  no  one  can  yet  say).  The  significance 
of  chemical  features,  resulting  from  altered  gastric  secretions  or  from 
toxic  bacterial  products,  is  not  known  positively.  It  is  certain  that 
none  of  the  products  which  have  been  held  responsible  as  auto-intoxi- 
cants are  universal  causes.  At  any  rate,  the  general  features  of  gastro- 
intestinal hygiene  should  be  carried  out.  Constipation  is  to  be  avoided, 
and  such  diet  taken  as  experience  has  shown  is  individually  applicable. 
Excesses  in  certain  articles  of  diet  are  held  by  many  as  exciting  causes; 
such  empirical  feelings  should  be  respected;  the  patient  often  knows 
himself  better  than  does  the  physician. 

In  some  excessive  carbohydrate  intake  acts  disastrously;  in  others 
wine,  whisky,  or  gin.  The  history  of  inability  to  eat  fatty  food,  par- 
ticularly sausages,  is  not  infrequent. 

In  rarer  instances,  one  notes  that  certain  auditory  stimuli  may 
bring  on  a  migraine.  To  attend  certain  fatiguing  and  thrilling  operas 
is  followed  in  some  by  migraine  attacks.  Here  psychical  mechanisms 
are  at  work. 

If  the  varying  elements  mentioned  have  any  real  relation,  it  is 
evident  why  such  a  variety  of  measures  will  be  of  help  to  a  few,  and 
why  so  many  more  will  be  worthless  for  many  but  useful  for  some. 
Medication  between  attacks  is  largely  useless,  save  naturally  in  the 
symptomatic  migraines.  General  medication,  for  no  definite  purpose 
but  just  in  the  hope  that  it  may  do  good,  as  iodides,  bromides,  strych- 
nine, etc.,  is  senseless.  If  definite  factors  are  found  that  need  correc- 
tion, and  can  be  so  modified  by  drugs  in  the  desired  direction,  then 
they  will  prove  useful.  Thus  iodides  will  undoubtedly  help  many 
presenile  arteriosclerotic  migraines;  bromides  are  useful  for  sleepless 
and  irritable  conditions  which  provide  a  good  foundation  for  the 
nervous  instability  that  permits  an  attack;  laxatives  are  called  for 
if  persistent  constipation  bears  any  causal  relationship,  and  thyroid 
is  invaluable  in  certain  hypothyroid  states  which  are  very  liable  to 
affect  the  tonus  of  the  vegetative  nervous  system,  particularly  in  that 
tjT)e  known  as  sympathicotonic.  Very  minute  doses  are  useful  in 
certain  mild  hyperthyroid  migraines,  as  is  also  belladonna,  or  violet- 
ray  treatment  of  the  thyroid.    In  the  migraines  related  to  menstrual 


180  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

disorders  in  women  a  careful  analysis  of  psychical  and  gonadal  factors 
is  essential. 

Complicated  systems  of  diet  have  been  devised.  Usually  such  are 
more  prolific  in  engendering  semi-invalidism  than  useful  for  migraine. 
Here  and  there  a  patient  derives  benefit  from  a  strict  dietary  regimen, 
but  unless  there  are  real  reasons  why  a  patient  should  not  eat  red 
meat,  or  tomatoes,  or  sundry  other  articles,  as  determined  by  actual 
experience  and  under  repeated  experimental  trials,  in  order  to  elimi- 
nate faddists'  errors,  the  patient  is  better  off  without  a  diet  card. 
The  reasons  sought  for  are  not  those  contained  in  many  treatises  on 
dietetics,  in  which  primitive  notions  concerning  differences  in  red  meat 
and  white  meat,  vegetables  growing  under  the  ground  and  those  above 
the  ground,  are  foolishly  perpetuated.  The  only  satisfactory  manner 
to  attack  the  metabolic  problem  is  to  carry  out  a  complete  metabolism 
analysis.  Haphazard  attacks  here  and  there  lead  only  to  premature 
and  insecure  judgments. 

Complete  formulas  for  attacking  excessive  bacterial  putrefaction 
are  applicable  only  when  it  is  proved  that  such  excessive  bacterial 
action  exists  and  has  a  relation  to  the  migraine.  The  hj'pothesis 
cannot  be  excluded  ex  cathedra,  but  it  remains  unproved  for  most 
cases,  and  of  doubtful  applicability  in  a  few.  The  belief  that  the 
presence  of  indicanuria  is  an  infallible  index  of  harmful  putrefactive 
products  is  not  well  founded. 

The  avoidance  of  alcohol  and  tobacco,  while  advisable,  is  so  only 
relatively.  The  individual's  reaction  to  all  influences  should  be 
rigidly  estimated  before  those  usually  self-evident  restrictions  are 
imposed  in  the  name  of  health. 

In  certain  individuals  a  change  of  occupation  may  be  absolutely 
necessary,  but  here  again  .one  must  be  wisely  conservative,  and  not 
consign  all  migraine  patients  to  an  outdoor  life.  Outdoor  workers  are 
by  no  means  exempt  from  migraine;  such,  perhaps,  should  be  clerks. 
The  character  of  the  work  is  to  be  borne  in  mind.  The  elements  of 
haste,  of  pressure,  and  of  lack  of  leisure  are  to  be  weighed. 

Psychoanalysis  should  be  advised  for  severe  recurrent  migraines  in 
neurotic  individuals.  Chronic  headaches  are  most  frequently  psycho- 
genic in  origin  and  need  psychoanalysis.  INIigraine  is  one  of  the  most 
universal  scapegoats  for  meliorizing  psychological  conflicts. 

For  the  treatment  of  the  attack  itself,  one  finds  that  a  like  fitting 
of  remedies  to  the  individual  is  called  for.  In  the  initial  phase  of 
vasoconstriction  a  number  of  vasodilators  are  of  service,  although 
their  action  is  extremely  unequal.  The  nitrites  and  nitrates  have  been 
employed  for  years,  and  usually  with  a  fair  degree  of  success  if  the 
dosage  and  individual  member  of  the  group  be  correctly  chosen  with 
reference  to  the  severity  of  the  attack.  A  mixture  is  of  greatest  value; 
nitroglycerin  and  erythrol  tetranitrate  give  the  best  combination,  for 
following  the  very  evanescent  and  powerful  action  of  the  former,  the 
more  prolonged  and  steady  action  of  the  latter  maintains  the  effect. 
The  slower  acting  nitrates  are  practically  useless.    Nature's  readjust- 


OPHTHALMIC  MIGRAINE  181 

ment,  vasodilatation  by  vomiting,  etc.,  has  already  reduced  the  cerebral 
pressure,  and  the  stage  has  passed  when  the  dilating  remedies  might 
be  useful.  It  is  practically  only  in  the  vasoconstriction  stage  that  the 
nitrites  are  worth  much;  and  in  many  they  are  inefficient,  the  reason 
for  this  being  as  yet  unappreciated.  Given  too  late,  they  overdo 
the  dilatation  and  increase  the  difficulty. 

The  analgesic  vasodilators  have  come  to  occupy  the  front  rank. 
The  precise  pharmacology  of  each  must  be  appreciated  in  order  to 
obtain  the  best  results.  Solubility,  time  of  absorption,  slight  differ- 
ences in  the  chemical  formula  and  'n  action,  continuance  of  effect  with 
minimum  by-effects,  are  all  to  be  studied.  The  list  is  a  long  one  and  is 
constantly  on  the  increase.  Antipyrin,  acetanilid,  phenacetin,  and  the 
related  salicylic  acid  (aspirin,  etc.)  compounds  are  the  chief  members. 
It  is  to  be  remembered  that  while  their  general  action  is  closely  related, 
there  are  specific  differences  in  the  working  of  each,  and  the  measure 
of  success  that  one  has  in  mastering  the  majority  of  migraines  depends 
upon  a  knowledge  of  these  factors,  Antipyrin,  by  reason  of  its  rapid 
solubility  and  quick  action,  occupies  an  important  place,  but  is  not 
always  applicable.  Acetanilid,  alone  or  in  combination  with  other 
analgesics  of  related  type  (salicylic  acid  derivatives),  bromides,  and 
caffeine,  is  also  valuable.  The  dosage  should  be  graded  according 
to  the  usual  severity  of  the  attacks.  Tolerance  is  established  in  the 
quickly  recurring  attacks,  and  changes  must  be  made.  It  is  not  yet 
certain  what  part  is  played  by  the  respective  analgesic  and  vasodila- 
tation actions  of  this  group.  They  have  robbed  migraine  of  most  of 
its  terrors,  and  tended  to  diminish  the  use  of  morphine  and  its  deriva- 
tives very  markedly. 

Caffeine  is  a  much  overrated  drug.  In  the  abortive  attacks  and  in 
the  morning  remains  of  a  migraine  it  is  useful;  but  for  a  full-fledged 
attack  it  is  not  efficient.  Similarly,  bromides  alone,  chloral,  and  other 
widely  used  drugs  are  valuable  only  in  mild  attacks.  They  should  be 
used  in  preference  to  other  more  potent  remedies,  which  should  be 
reserved  for  the  severer  attacks,  in  order  that  one's  therapeutic  meas- 
ures may  more  correctly  approximate  the  needs  of  each  individual 
occasion. 

The  use  of  aconite  and  Cannabis  indica  is  more  restricted  now  that 
really  efficient  analgesics  are  known.  Aconite  is  rarely  called  for, 
while  Cannabis  indica  or  Cannabis  americana  has  a  limited,  though 
no  less  definite,  place.  In  attacks  associated  with  much  mental 
depression  the  addition  of  cannabis  is  often  useful.  The  often  experi- 
enced inefficacy  of  this  latter  remedy  is  largely  due  to  its  extreme 
A'ariability.  (ireat  care  is  therefore  to  be  exercised  in  the  selection  of  a 
proper  preparation.  Tablet  preparations  are  usualh'  worthless.  This 
is  equally  true  of  the  volatile  nitrite  preparations.  0])ium,  or  its 
main  derivative,  morphine,  should  be  used  only  as  a  last  resort.  It  is 
rarely  really  needed. 

Lying  down  in  a  quiet,  darkened  room — a  brisk  saline  laxative  taken 
as  early  as  possible,  the  patient  being  undressed  and  well  covered — 


182  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

these  are  essential  in  the  severe  exhausting  attacks.  A  very  hot  bath 
often  aids  very  materially  in  restoring  the  patient  to  comparative 
freshness.    Cold  is  to  be  avoided. 

The  greatest  folly  of  all  is  to  treat  all  patients  and  every  attack  alike. 

Periodic  Palsies:  Ophthalmoplegic,  Facioplegic,  Hemiplegic  Mi- 
graine.— It  is  known  that  in  the  ordinary  attack  of  ophthalmic 
migraine  there  may  occur  various  sensory  or  motor  phenomena, 
among  which  anesthesias  or  paralyses  are  the  most  marked.  These 
sensory  and  motor  changes  are  extremely  diverse  when  the  entire 
range  of  the  migraine  symptomatology  is  brought  into  review,  but 
there  is  one  symptom  grouping  which,  by  reason  of  its  comparative 
frequency  and  close  similarity,  was  set  apart  from  others  occurring 
in  this  affection  and  named  by  Charcot  ophthalmoplegic  migraine 
in  order  to  distinguish  it  from  its  more  classical  relation.  It  consists 
in  a  paresis  or  a  paralysis  of  one  or  more  muscles  of  the  eye,  inner- 
vated chiefly  by  the  oculomotorius,  w^hich  comes  on  either  following 
or  during  a  migraine  attack. 

Inasmuch  as  oculomotor  pareses  or  paralyses  may  occur  from  a 
great  diversity  of  causes,  apart  from  a  migraine,  and  may  appear 
periodically,  it  has  been  held  by  many  that  the  term  ophthalmoplegic 
migraine  has  no  particular  right  to  exist,  but  the  evidence  is  too  great 
to  eliminate  migraine  as  a  competent  producing  cause  for  these  periodic 
oculomotor  paralyses. 

Furthermore,  evidence  is  accumulating  that  indicates  that  paralyses 
of  the  oculomotorius  are  not  the  only  paralytic  syndromes,  and  it  is 
recognized  that  a  number  of  other  muscles  suffer  from  similar  affections, 
which  are  more  or  less  transitory  in  their  character  and  which  are  in 
all  probability  due  to  vegetative  nerve  disorders  chiefly  of  a  vascular 
character.  To  these  changeable  and  flitting  palsies  the  name  periodic 
palsies  is  given.  Some  are  marked  in  certain  families  and  hence  have 
been  termed  familial.  Among  the  rarer  of  these  migraine  equivalents, 
or  periodic  palsies  are  the  facioplegic  and  hemiplegic  tj'pes.  Monoplegic 
syndromes  are  still  rarer. 

Etiology. — Whether  heredity  plays  any  greater  part  here  than  in 
migraine  in  general  is  difficult  to  decide.  Certain  periodic  palsies 
not  usually  classed  with  migraines  show  marked  heredity. 

Symptoms. — Leaving  aside  for  the  moment  the  atjT3ical  and  symp- 
tomatic periodic  oculomotor  paralyses  due  to  other  causes  than 
migraine,  one  finds  in  these  patients,  usually  during  or  after  a  severe 
attack  of  unilateral  migraine,  with  headache,  nausea,  vomiting,  etc., 
a  ptosis  of  the  eyelid  on  the  same  side,  and  a  loss,  partial  or  complete, 
of  the  upw^ard,  downward,  and  inward  movements  of  the  eye  of  the 
same  side.  This  eye  is  usually  directed  outward  and  downward,  and 
the  patient  sees  double.  This  may  or  may  not  be  accompanied  by 
sensory  disturbances  in  the  superior  branch  of  the  trigeminus,  just  as 
may  be  observed  in  ordinary  ophthalmic  migraine. 

After  a  variable  length  of  time,  a  few  days,  a  week  or  more,  the 
paralysis  disappears,  usually  gradually,  and  the  patient  suffers  no  incon- 


PERIODIC  PALSIES  183 

venience  from  the  ocular  palsies  or  the  ptosis.  In  some  individuals 
such  palsies  accompanying  a  migraine  have  come  on  comparatively 
early  in  life,  almost  with  the  beginning  of  the  migraine  attacks;  for 
the  majority,  however,  they  follow  several  years  after  the  establish- 
ment of  a  migraine,  in  some  instances  as  late  as  sixty  years.  In  some 
only  a  very  severe  attack  will  be  accompanied  by  the  oculomotor  signs, 
or  only  slight  palsies;  transitory  ptosis  may  occur  frequently.  But  in 
others  the  palsies  deA'elop  with  each  attack  of  migraine  and  often  in 
increasing  severity.  The  effects  may  persist  longer  and  longer  between 
the  attacks,  until  in  a  few  they  become  permanent  palsies.  This  t\'pe, 
however,  often  permits  of  other  interpretations. 

A  double  lesion  can  be  understood,  although  it  rarely  occurs.  Iso- 
lated abducens  palsy  has  been  described,  also  isolated  trochlearis; 
and  complete  ophthalmoplegia  is  reported  in  a  single  case,  but  in  view 
of  the  many  possible  contributory  factors  it  perhaps  is  preferable  to 
view  such  a  case  from  another  standpoint. 

Ophthalmoplegic  Migraine. — There  has  been  much  speculation  con- 
cerning the  central  or  peripheral  nature  of  this  form  of  third-nerve  palsy. 
The  present  view  regarding  migraine  in  general,  that  it  is  due  to  a  dis- 
turbance in  cerebral  pressure  secondary  to  vascular  modifications,  is 
sufficient  to  account  for  the  oculomotor  palsies  as  well,  in  view  of  the 
location  of  the  peripheral  branches  of  the  third  nerve  in  relation  to 
the  cerebral  vascular  plexuses.  In  fact,  the  occurrence  of  the  ophthal- 
moplegic type  is  one  of  the  strong  arguments  for  the  general  pressure 
hypothesis,  as  Spitzer  has  well  argued.  If,  as  has  been  shown  by 
several  autopsies,  to  these  considerations  additional  local  causes  be 
added,  which  increase  or  permanently  maintain  such  pressure  effects, 
this  interpretation  is  made  more  certain.  Thus,  exudates,  fibrous 
processes,  swelling  in  the  cavernous  sinuses,  swelling  of  the  hypophysis, 
tumor  formation,  gummata,  etc.,  have  been  found  in  patients  suffering 
from  periodic  oculomotor  paralyses  associated  with  migraine. 

It  is  true  that  some  of  these  are  to  be  interpreted  as  symptomatic 
migraines,  in  which  the  foreign  body  acts  primarily  as  an  irritant  to 
cause  the  vascular  disturbance  which  sets  free  the  migraine  reaction, 
and  secondarily  serves  as  an  additional  cause  of  pressure  to  bring 
about  the  palsy.  In  a  personally  observed  case,  with  basal  gummata, 
the  periodic  oculomotor  palsy  and  migraine  attacks  had  occurred  for 
a  period  extending  over  four  or  five  years,  usually  with  every  men- 
strual period.  Here  were  three  interplaying  factors,  and  the  exact 
part  played  by  each  can  only  be  inferred.  The  slight  disturbance  of 
menstruation,  usually  adjusted,  in  this  case  was  not  by  reason  of  the 
exudate.  A  migraine  was  set  up,  the  acute  pressure  of  which,  added 
to  that  of  the  exudate,  caused  the  ophthalmoplegia.  This  ophthal- 
moplegia has  become  fairly  persistent  in  the  intermigrainous  periods 
in  recent  years. 

Facioplegic  and  Hemiplegic  Types. — These  are  much  rarer  complica- 
tions. Some  observers  would  rule  out  the  facial  type,  but  there  is 
no  good  reason  for  this.    It  does  occur,  and  certain  recurrent  facial 


184  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

palsies  which  Bernhardt  in  his  vokiminous  study  has  shown  to  occur 
in  7  or  8  per  cent,  of  the  cases  are  of  this  type.  Hemiplegic  attacks 
accompanying  migraine  are  also  rare.^  Hemianopsia  and  optic  neuritis 
(probably  edematous  or  hemorrhagic)  are  still  rarer  complications. 

vSome  periodic  palsies  belong  in  this  group.  Vasomotor  palsies 
occur  in  the  spinal  cord  as  well  and  are  accompanied  by  edematous 
infiltrations  usually  of  a  mild  grade.  They  are  occasioned  by  much 
the  same  combination  of  stimuli  as  are  the  migraines  but  they  are 
apparently  much  rarer.  The  resulting  palsies  are  known  as  periodic 
palsies  and  are  discussed  in  the  next  group. 

Diagnosis. — Every  patient  should  be  regarded  as  one  suffering  from 
something  more  than  the  migraine,  until  all  accessory  causes  are 
excluded.    What  these  may  be  have  been  mentioned  already. 

Treatment.^ — Little  needs  to  be  added  to  the  therapy  outlined  under 
migraine.  Syphilis  as  a  cause  for  both  a  migraine  and  an  exudate 
should  be  treated,  and  the  Wassermann  reaction  utilized  to  clear  up 
the  diagnosis  and  therapeutic  indications. 

3.  Vasomotor  Irritability  Group. 

Angioneurotic  Edema. — This  condition  is  better  described  as  acute 
circumscribed  edema  (Quincke),  since  such  a  name  does  not  commit 
one  to  its  being  a  vascular  neurosis,  although  this  is  probable. 

These  most  striking  skin  edemas  were  described  as  early  as  1778 
by  Salpertus.  Crichton,  in  1801,  also  observed  them,  and  Graves,  who 
gave  such  an  excellent  outline  of  exophthalmic  goiter  in  1848,  described 
a  patient  with  localized  swelling  of  the  face,  forehead,  and  eyes,  in 
whom  the  edema  persisted  only  a  few  hours.  Various  aberrant  local- 
izations have  been  described  often  under  different  names.  Natu- 
rally hysteria  bulked  large  in  the  diagnosis  in  the  earlier  days.  Other 
synonyms  indicate  under  what  different  diagnostic  groups  these  cases 
may  be  found:  urticaria,  urticaria  edematosa,  epidermolysis  bullosa, 
urticaria  tuberosa,  urticaria  gangrenosa,  and  giant  urticaria.  Further, 
one  finds  rheumatic  'edema,  arthritic  edema,  repeating  rheumatic 
edema,  hydrops  articulOrum,  intermittent  rheumatic  edema,  neuro- 
arthritic  edema,  in  the  period  when  the  cases  were  grouped  among 
the  "rheumatisms."  Gastrosuccorrhea  periodica  is  a  stomach  locali- 
zation. Probably  there  are  others  of  obscure  nature.  Acute  brain 
swelling,  meningitis  serosa,  spinal  swelling,  local  transient  edema, 
intermittent  edema,  and  periodic  paralysis  are  among  them. 

Quincke,  in  1882,  described  it  as  acute  circumscribed  skin  edema, 
while  in  a  Kiel  dissertation,  one  of  his  students,  Dinkelacker,  brought 
together  many  of  the  older  descriptions,  and  showed  the  unity  of  several 
apparently  dissimilar  processes.    He  termed  it  acute  edema. 

Occurrence. — The  disorder  is  not  frequent,  yet  it  is  not  rare.  Men 
and  women  appear  about  equally  involved.     It  may  be  present  in 

1  Jelliffe:    New  York  Med.  Jour.,  January  6,  1906. 


VASOMOTOR   IRRITABILITY  GROUP 


185 


young  children — one  and  a  half  months  (Crozer  Griffith);  three 
months  (Dinkelacker).  After  forty  it  appears  very  rarely,  as  an 
initial  development,  although  in  affected  individuals  it  may  persist 
until  late  in  life.  Cassirer  reported  histories  of  patients  of  seventy- 
nine  and  sixty-nine,  in  "which  the  disease  appeared  comparatively  late 
in  life.    Raven  reports  a  case  in  a  woman  of  eighty-six. 

Occupation  apparently  plays  no  role.  Heredity,  on  the  other  hand, 
is  conspicuous.  Many  authors  have  mentioned  this  feature.  Osier's 
family  tree  has  been  freely  cited,  and  is  here  reproduced  in  slightly 
changed  form.  Ensor  reports  a  family  of  eighty  members,  with 
thirty-three  affected  individuals,  twelve  of  whom  died  of  edema  of  the 
glottis.  Similar  hereditary  features  are  reported  by  several  observers. 
The  question  of  its  transmission  has  not  been  completely  cleared  up. 
In  Apert  and  Delille's  families  only  the  males  were  affected,  but  this 
does  not  seem  to  be  the  rule. 


INHERITANCE  IN  ANGIO-NEUROTIC   CEDEMA 
"T"  FAMILY 


"  6r~5 


-a 


III 


IV 


£Wk  ^ 


¥^ 


rii46i44*4i4 


ri 


6% 


|~[  MALE 

O  female)  o 
Fig.  70. — Chart  showing  heredity  in  an  angioneurotic  edema  family.     (Osier.) 


In  many  families  similar  types  of  localized  edema  prevail  in  the 
members,  while  in  others,  apparently  more  often,  all  of  the  possible 
variants  disappear.  Other  nervous  system  involvements  appear 
associated  with  many  of  the  families;  how  much  of  this  is  largely 
coincidence,  how  much  general  neuropathic  causal  relationship  is 
difficult  to  determine  from  the  studies  at  hand. 

The  syndrome  is  associated  infrequently  with  tabes,  myasthenia 
gravis,  spinal  cord  tumor,  exophthalmic  goiter,  myxedema,  periodic 
palsies,  while  it  seems  very  frequently  associated  with  many  so-called 
functional  neuropathic  states — hysteria,  compulsion  neuroses,  migraine 
etc. — and  in  certain  psychotic  individuals  with  schizophrenia,  manic- 
depressive  psychosis,  feeble-mindedness. 

Local  traumata  play  a  role  at  times,  particularly  in  determining 


186  VEGETATIVE  OR   VISCERAL  NEUROLOGY 

the  location  of  the  swelhng.  Emotional  shock  bulks  large  as  a  direct 
etiological  factor,  as  does  also  the  action  of  thermal  influences.  Cold 
is  very  frequently  an  exciting  factor  in  the  reaction.  Menstrual 
factors  seem  to  enter  into  the  etiology  of  certain  cases. 

A  moment's  reflection,  therefore,  will  show  that  under  the  term 
acute  circumscribed  edema,  one  is  dealing  with  phenomena  of  great 
variability  and  multiform  genetic  pathogeny.  In  discussing  the 
pathology,  a  return  will  be  made  to  this  many-sided  etiology. 

Symptoms. — The  original  conception  of  Quincke  has  been  much 
employed,  and  Cassirer  in  his  large  monograph  shows  the  present- 
day  trend  to  include  a  large  number  of  acute  edematous  swellings 
within  the  nosological  group.  Thus  one  distinguishes  localized  edema 
of  the  skin,  edemas  of  the  mucous  membrane,  of  the  eyelids,  mouth, 
glottis,  esophagus,  stomach,  intestines,  respiratory  tract,  edemas  of  the 
joints,  the  meninges,  the  tendinous  aponeuroses,  of  the  spinal  cord,  of 
the  brain,  of  the  kidneys,  with  polyuria,  albuminuria,  hemoglobinuria, 
diminished  secretions,  and  edema  of  other  structures. 

The  07iset  is  usually  acute,  with  some  initial  prodromal  signs  of 
malaise,  fatigue,  chilliness,  anorexia,  nausea,  and  slight  rise  in  tem- 
perature. The  symptoms  that  develop  will  depend  upon  the  localiza- 
tion of  the  process. 

In  the  skin  there  are  isolated  swellings.  These  are  localized,  variable 
in  size,  at  times  small,  resembling  urticarial  blotches  (intermediary 
forms)  but  usually  as  distinct  swellings,  with  an  elastic  feel,  and  due 
to  local  accumulations  of  clear  serum  within  the  skin.  The  color  of 
the  swelling  is  usually  that  of  the  skin,  or  paler,  rarely  red  or  reddish. 
The  swelling  comes  on  with  great  rapidity,  in  a  few  moments,  and 
remains  a  few  hours,  mostly  a  few  days,  and  then  disappears  without 
leaving  any  trace.  They  are,  as  a  rule,  non-irritating,  painless,  and 
only  cause  discomfort  as  a  result  of  the  tension.  Certain  patients 
experience  burning,  itching,  and  intense  pain. 

The  size  of  the  edematous  patches  varies  greatly.  At  times  very 
small — one-half  inch — they  are  more  apt  to  be  three  or  four  inches  in 
diameter,  or  at  times  involve  the  larger  part  of  a  limb.  The  scrotum 
may  at  times  swell  up  to  the  size  of  a  foot-ball.  The  penis,  in  cases 
reported  by  Borner,  has  swollen  to  double  its  diameter.  The  end 
body  was  swollen  also  in  a  remarkable  case  reported  byDiethelm. 
At  times  the  swellings  are  numerous,  polymorphous,  semiconfluent. 
They  rarely  rise  more  than  one-quarter  to  one-half  centimeter,  but 
swellings  two  to  four  inches  above  the  skin  occur.  The  margins 
of  the  swellings  are  usually  sharply  circumscribed,  but  at  times  may 
shade  oft'  imperceptibly  into  normal  areas.  The  swellings  are  usually 
described  as  circular  or  sausage-shaped.  The  swellings  invade  almost 
any  layer  in  the  skin,  the  musculature,  or  they  may  even  invade 
the  periosteum.    Some  have  been  termed  pseudolipomata. 

The  consistency  is  semisolid,  non-pitting,  or  slightly  so.  The  color 
as  stated  is  usually  that  of  the  normal  skin,  or  it  may  be  paler,  or  have  a 
cadaveric  hue.    Again  it  is  pinkish  to  red,  or  even  deep  red.    Often 


VASOMOTOR  IRRITABILITY  GROUP  187 

the  color  disappears  on  pressure.    The  color  may  change  during  the 
rise  of  the  swelling. 

Local  temperature  varies.  At  times  the  skin  is  colder,  again  it  is 
warmer  than  that  of  the  non-affected  parts.  Exact  studies  are  want- 
ing. It  seems  not  unlikely  that  there  is  an  initial  increase  in  the 
local  temperature. 

Sensory  changes  are  not  present  as  a  rule.  Certain  cases  have 
shown  preliminary  neuralgic  twinges,  no  definite  sensory  defect 
has  been  noted,  but  refined  methods  of  examination,  such  as  those 
demanded  by  Head,  have  not  yet  been  made.  There  is  frequently 
the  subjective  sense  of  great  discomfort,  especially  in  marked  swellings 
about  the  face. 

There  are  rarely  any  residuals,  although  occasionally  scaling  or 
peeling  has  been  observed,  probably  for  the  more  superficially  lying 
edemas. 

Secretory  symptoms  have  not  been  carefully  recorded.  Local  hj'perid- 
rosis,  dermographia,  increased  tear  secretion  have  been  noted. 

The  location  of  the  swelling  may  be  almost  anywhere,  it  cannot 
be  said  that  one  place  more  than  another  is  a  favorite  site  (statistically) . 
Exposed  portions  of  the  body  seem  to  be  more  often  involved,  but 
when  on  the  hand  or  hands,  the  distribution  is  not  of  the  glove  type, 
nor  are  the  swellings  apt  to  be  symmetrical,  nor  do  they  seem  to  follow 
radicular  or  peripheral  distributions.  There  is  a  distinct  tendency 
for  a  recurring  edema  to  occupy  the  position  involved  during  a  former 
attack. 

Periarticular  swelling  constitutes  a  peculiar  type,  so  also  do  parotid 
and  salivary  gland  edemas. 

Mucous  Membranes. — These  are  frequently  involved.  The  lips, 
mouth,  soft  palate,  tongue,  pharyngeal  pillars,  nasal  membrane, 
larynx  are  all  sites  of  election.  The  last  is  particularly  frequent  and 
is  dangerous  to  life.  In  these  cases  other  structures  than  the  larynx 
are  implicated,  especially  the  epiglottis  and  closely  associated  structures. 
In  the  larynx  the  mucous  membrane  is  swollen  and  tense;  the  edema 
infiltrates  throughout. 

When  the  larynx  is  involved,  the  symptoms  are  apt  to  be  very 
marked.  There  is  beginning  tickling,  and  rapidly  oncoming  difficulty 
in  breathing,  until  marked  dyspnea  may  supervene,  with  death,  unless 
intubation  or  tracheotomy  is  performed.  Some  of  these  patients 
die  within  a  few  hours.  Many  cases,  on  the  other  hand,  clear  up  in 
an  hour,  after  severe  dyspneic  symptoms.  Acute  conjunctival  edema 
is  not  infrequent. 

Edemas  within  the  bronchi  occur  in  perhaps  20  per  cent,  of  the 
cases.  They  make  up  a  certain  percentage  of  the  cases  of  asthma. 
Certain  hay  fevers  possibly  belong  in  this  group.  Lung  edemas  have 
been  described. 

In  edemas  of  the  stomach  (gastrosuccorrhea  periodica)  external  signs 
are  also  usually  present.  There  may  be  intermittent  vomiting,  or 
sudden  acute  pains  and  anorexia.    The  attack  may  last  a  few  hours 


188  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

with  severe  pain,  and  finally  more  or  less  continuous  vomiting  of  clear  or 
bile-colored  watery  masses,  marked  thirst,  and  gradual  disappearance 
of  all  of  the  symptoms.  Bits  of  gastric  mucosa  have  been  accidentally 
dislodged  which  showed  marked  edematous  swelling. 

In  intestinal  localizations  profuse  diarrheas  are  present,  with  colicky 
pains,  meteorism,  tenderness  of  the  abdomen,  diminished  urination 
great  thirst,  and  collapse.  The  diarrheas  are  purely  neurotic  diarrheas, 
so-called,  and  occur  in  association  with  other  signs  of  a  circumscribed 
edema. 

Rarer  localizations  present  in  the  tendons  have  been  described, 
particularly  by  Schlesinger.  Muscle  edemas  are  also  rarely  described, 
although  it  is  probable  that  they  are  of  frequent  occurrence.  Lumbago 
is  possibly  of  this  type.  Articular  edemas  have  been  mentioned.  They 
are  frequently  of  psychical  origin,  as  for  instance  in  the  classical  associ- 
ation of  attacks  of  gout  and  of  anger. 

Optic-nerve  edema  is  one  of  the  rarer  localizations,  as  is  also  an 
edema  in  the  labyrinth  leading  to  a  Meniere  syndrome. 

The  bladder,  kidney,  and  heart  structures  are  among  the  rarest 
localizations.  Meningitis  serosa,  aphasia,  hemiplegia  and  monoplegias 
are  among  some  of  the  more  problematical  occurrences  reported  and 
periodic  paralyses  are  included  here  as  well  as  in  the  previous  group 
allied  to  the  migraines. 

Transition  forms  are  common,  especially  urticaria-like  eruptions. 
Acroparesthesias,  Raynaud-like  attacks,  local  asphyxias  of  the  extrem- 
ities, paroxysmal  hemoglobinuria,  acroasphyxia  chronica,  erythro- 
melalgia,  periodic  paralysis,  epidermolysis  bullosa  hereditaria,  synovial 
serositis,  fibrous  serositis,  herpes  zoster  are  all  affections  with  which 
attacks  have  been  combined,  singly  or  in  groups  of  two  or  three.  Occa- 
sionally edema,  acroparesthesia,  and  erythromelalgia  may  alternate 
in  one  and  the  same  patient. 

Prognosis.- — In  general  this  is  not  good.  The  tendency  to  larjTigeal 
localization  must  always  be  viewed  with  gravity.  A  great  many 
individuals  have  died  from  edema  of  the  glottis.  Remissions  are  to 
be  expected.  Some  patients  suffer  many  years,  others,  but  the  min- 
ority it  would  appear,  have  but  few  attacks.  There  is  some  general 
tendency  for  the  disorder  to  become  milder  as  the  affected  individual 
grows  older. 

Pathogenesis. — Recent  conceptions  concerning  edema  are  undergoing 
such  radical  modifications  that  it  is  practically  impossible  to  interpret 
the  findings  here  outlined  along  those  lines  that  regard  all  edemas  as 
cell  phenomena  solely,  independent  of  the  action  of  the  vegetative 
nervous  system  acting  on  the  bloodvessels.  The  studies  of  edema  made 
by  Fischer  and  others  emphasize  only  the  physicochemical  side  of  the 
problem.  They  neglect  the  role  of  the  vegetative  nervous  system  in 
regulating  tissue  tension  and  cellular  chemism.  The  statement  that  the 
disorder  is  an  angioneurosis  by  no  means  clears  the  situation,  although 
it  is  certain  that  the  sjinpathetics  are  media  from  cause  to  effect. 
The  study  of  anaphylactic  phenomena,  especially  as  seen  in  the  so-called 


VASOMOTOR  IRRITABILITY  GROUP  189 

anaphylactic  serum  reactions,  or  serum  diseases,  has  oflFered  suggestive 
ghmpses  indicating  certain  analogies  with  the  series  of  changes  here 
outlined.  Wherein  are  the  proteids  supposed  to  cause  these  related 
to  the  endocrinous  hormones?  It  can  only  be  stated  that  precisely 
similar  processes  and  appearances  are  found  in  the  serum  reactions, 
and  that  it  is  not  without  profit  to  enquire  more  into  the  mechanism 
of  their  production  in  an  attempt  to  understand  acute  circumscribed 
edema.  Unfortunately  the  mechanisms  of  the  changes  in  the  ana- 
phylactic reactions  are  still  much  in  the  dark.  There  is  a  distinct 
tendency  to  include  the  anaphylactic  reactions  under  the  phenomena 
regulated  by  the  vegetative  nervous  system.^ 

Acute  circumscribed  edema,  has  also  been  interpreted  as  a  modified 
colloid  absorption  reaction,  due  to  toxic  influences  brought  to  the  cells 
of  the  deeper  layers  of  skin,  muscle  or  mucous  membrane.  The  view 
here  tentatively  adopted  is  that  it  is  a  neural  reaction  brought  about 
through  the  ^■egetative  nervous  system,  which  controls  reciprocal 
tension  relations,  or  cellular  chemical  composition  relations. 

It  is  not  improbable  that  there  are  a  series  of  reactions  represented 
in  the  acute  circumscribed  edemas.  It  is  not  a  unicum,  and  analysis 
will  show  that  a  number  of  different  pathological  processes  may  underlie 
precisely  similar  phenomena,  be  they  in  any  vascular  area  of  the  body. 

Cassirer  adopts  this  vie\\ix)int,  but  consents  to  make  only  two  groups 
of  cases:  (a)  a  toxic,  autotoxic  group,  in  which  the  poison  works  in 
some  mysterious  way,  which  a  wealth  of  language  can  conceal,  better 
than  it  can  reveal,  and  (b)  a  heredofamilial  or  constitutional  neuro- 
pathic group,  which  he  regards  as  intimately  associated  with  instability 
in  certain  parts  of  the  vegetative  nervous  system.  This  may  be,  he 
says,  associated  in  some  manner  with  modifications  in  the  internal 
gland  secretions.  Here  another  dark  portal  is  entered.  At  all  events, 
Cassirer  is  loath  to  permit  so-called  angioneurotic  edema  to  wander 
from  the  neurological  fold,  and  concludes  that  the  disease  is  con- 
ditioned— at  least  his  group  (h) — by  the  lability  of  the  vegetative 
nervous  system.  Our  own  view  is  to  emphasize  the  importance  of 
psychogenic  components  in  the  etiology. 

Treatment. — This  is  purely  empirical.  One  should  not  eat  any  of 
those  things  which  experience  has  shown  to  be  hazardous. 

If  one  of  the  more  pronounced  toxic-anaphylaxis-like  types  be 
present,  careful  study  must  be  made  of  all  of  the  patient's  protein 
reactions,  and  attempts  made  calculated  to  regulate  the  diet  accord- 
ingly. It  seems  possible  that  it  is  through  the  gastro-intestinal  canal 
that  such  products  gain  entry,  particularly  in  food,  yet  some  may 
enter  the  respiratory  tract,  as  seems  to  be  the  case  in  the  related  hay- 
fever  reactions  which  are  thought  to  follow  certain  contacts,  such  as 
rag\veed,  rose,  hay,  and  other  pollens,  or  even  the  emanations  from 
lower  animals. 

'  Roncoroni:  Ergebiiisse  d.  Neurolopie  u.  P.sychiatrie,  vol.  ii.  No.  I.  .\lso  see  Noel 
Paton:  Nervous  Regulators  of  Metabolism.  Bayliss:  Principles  of  General  Physiology. 
Also  Smith's  Studies  on  Anaphylaxis,  loc.  cit. 


190  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

From  specific  exclusion  of  certain  proteins  one  passes  to  the  general 
hygiene  of  the  intestine.  This  means  a  sort  of  search  in  the  dark 
for  effective  agencies  by  chemical  means.  One  is  justified  notwith- 
standing in  trying  to  bring  about  altered  bowel  conditions,  which 
empirically  may  do  some  good,  when  a  laisser-faire  attitude  seems  to 
perpetuate  the  disturbance.  Naturally  one  should  avoid  intestinal 
therapy,  should  the  patient  be  of  an  entirely  different  type,  say  the 
intensely  neurotic  forms  with  familial  hereditary  burdens,  and  emo- 
tional shock  reactions.    These  patients  need  a  psychoanalysis. 

Of  the  gastro-intestinal  antiseptics  so-called,  few  are  of  value. 
INIenthol,  saline  laxatives,  carbonated  waters,  careful  dieting  (?)  may 
be  found  among  the  conventional  remedies  in  the  books.  The  taking 
of  a  milk-vegetable  diet  has  been  coincident  with  betterment  in  some 
individuals  and  coincident  with  getting  worse  in  others. 

In  certain  cases  with  associated  toxemias,  such  as  malaria,  etc.,  a 
specific  therapy  is  indicated. 

On  the  supposition  that  the  bloodvessels  need  bracing  up  to  pre- 
vent transudation  through  their  walls,  also  a  hypothetical  postulate, 
apparently  inadequate,  such  drugs  as  strychnine,  ergot,  arsenic, 
atropine,  morphine  have  been  recommended.  While  all  of  these  will 
bring  about  vasoconstriction  it  is  not  apparent  whether  they  can  alter 
a  hj^^othetical  transudability  or  not.  Calcium  lactate  is  the  modern 
weapon  for  this  latter.  The  authors  have  not  seen  it  recommended, 
but  it  may  be  of  service  in  preventing  transudates,  as  such  are  thought 
to  be  conditioned  by  a  diminution  in  the  calcium  content  of  the  body 
plasma.  Cassirer  mentions  calcium  chlorate.  At  all  events  the  vaso- 
constricting  drugs  have  not  been  of  any  particular  service  clinically. 
Now  and  then  they  seem  of  service;  none  have  been  proved  of  pro- 
phylactic value  which  is  a  stricter  test  of  their  usefulness,  since  the 
disease  is  so  self -limited.  Atropine  is  of  great  value  at  times.  It 
should  always  be  tried  in  the  cases  which  seem  to  show  sympathetic 
let-down — hyperthyroid  types . 

In  cases  associated  with  laryngeal  symptoms,  intubation  may  be 
necessary — even  tracheotomy.  There  are  records  of  certain  patients 
condemned  to  the  persistent  use  of  the  tracheotomy  tube. 

In  the  more  strictly  neurotic  t}q^e — C^ssirer's  group  (6) — it  is  impor- 
tant that  the  patient  be  taught  a  healthy  morale.  The  substitution 
of  reasonable  and  intelligent  actions  for  purely  instinctive  and  emo- 
tional reactions  must  be  acquired  by  them,  if  they  can  hone  to  in  any 
way  control  their  hair-trigger  vegetative  nervous  system.  Perhaps 
it  was  so  given  to  them,  defective  and  badly  coordinated;  even  then  a 
rational  pedagogy  will  prove  of  service.  Many  will  be  helped  by  the 
methods  outlined  by  Dubois  or  Dejerine;^  others  will  need  a  psycho- 
analysis. Increasing  experience  and  the  literature  are  serving  to 
establish   the   fact   that  psychogenic   factors   amenable   to   psycho- 

i  Dubois:  Pyschic  Treatment  of  Nervous  Disorders.  Dejerine:  Psychoneuroses  and 
Psychotherapy,  Philadelphia,  1915.  Jelliffe:  Technic  of  Psychoanalysis,  New  York, 
1918. 


SCLERODERMA  191 

analysis  are  responsible  for  a  number  of  these  phenomena.  Asthma, 
hay  fever,  rose  colds,  protein  sensitizations,  urticarias,  synovial  swell- 
ings, arthritis  deformans,  migraine  and  other  edematous  states  causing 
acute,  even  chronic  disorders  have  been  in  most  instances  greatly 
relieved,  in  others  cured,  by  the  altered  emotional  attitude  taken  by 
analyzed  patients.  Just  how  the  vegetative  pathways  become  involved 
in  their  complex  neurobiochemical  relations  has  been  shown  in  the 
pages  preceding,  also  in  the  chapters  on  the  neuroses  and  psycho- 
nem-oses,  under  which  groups  also  many  of  these  patients  are  classified. 

Vegetative  Skin  Syndromes. — The  vegetative  nervous  disorders  of 
the  skin  is  still  an  unwritten  chapter  in  medicine.  It  is  only  possible 
here  to  sketch  rapidly  a  few  skin  sjTidromes  which  are  spoken  of  in 
dermatological  literature  as  "neurotic,"  or  as  "trophic  dermatoses," 
etc.  The  skin  is  peculiarly  under  the  control  of  the  vegetative  nervous 
sj'stem  and  a  careful  study  of  skin  phenomena  with  an  eye  to  their 
neurological  significance  will  yield  many  fruitful  suggestions.  The 
text-books  of  dermatology^  speak  of  the  extreme  frequency,  and  chron- 
icity  of  such  disorders  as  simple  acne,  as  psoriasis,  eczema,  and  a 
number  of  others.  They  often  are  treated  for  decades  without  success 
under  the  present-day  dermatological  concepts.  Scarcely  any  derma- 
tologist goes  behind  the  term  "neurotic,"  or  "nervous,"  Herein  is  a 
rich  field  for  vegetative  nervous  s\'stem  disturbances  on  an  emotional 
basis  that  can  be  reached  by  psychoanalysis.  The  field  of  skin  s^th- 
bolisms  in  the  neuroses,  psychoneuroses,  and  psychoses  has  hardly 
been  touched  by  the  dermatologists.^  For  the  neurologist  there  are 
numerous  fruitful  problems. 

Scleroderma.— Hippocrates  described  an  Athenian  who  had  a  hard, 
indurated  skin  all  over  his  body,  and  Galen  spoke  of  patients  whose 
skin  was  hard  and  leathery,  with  the  pores  all  stopped  up.  Thirial,  in 
1845,  gave  the  earliest  good  descriptions  of  scleroderma,  and  the  French 
school,  with  Ball,  Charcot,  and  Hallopeau,  fashioned  the  present-day 
descriptions  of  this  disorder.  Grasset  and  Brissaud,  in  1890,  were 
among  the  earlier  advocates  that  it  was  a  vegetative  nerve  disorder, 
while  Striimpell  first  correlated  some  of  the  endocrinous  cases,  observ- 
ing a  patient  with  scleroderma  and  acromegaly. 

Its  description,  occurrence,  and  differential  diagnosis,  its  numerous 
forms  and  variations  are  best  to  be  found  in  dermatological  literatu*e. 
The  neurological  interest  focusses  itself  upon  the  determination  of  the 
level  of  the  nervous  system  involved,  for  the  pathogeny  is  extremely 
multiform.  Peripheral  nerve  lesions  (trapezius  palsy)  have  been  known 
to  be  followed  by  localized  scleroderma.  Spinal  cord  injury,  involving 
Jacobsohn's  sympathetic  nuclei,  occasions  other  cases.  Numerous 
cases  are  associated  with  other  spinal  injuries,  as  in  sjTingomyelia, 
poliomyelitis,  multiple  sclerosis,  etc. 

•  Jelliffe:  Psoriasis  as  a  Hysterical  Conversion  Mechanism,  New  York  Med.  Jour., 
December,  1916.  Reede,  E.  H.:  Role  of  Veg.  N.  Syst.  in  Diseases  of  the  Skin.  Derm. 
Sect.  Jour.  Am.  Med.  A.ssn.,  June  14,  1918.  Morris,  M.:  The  Internal  Secretions  in 
Relation  to  Dermatology,  B.  M.  J.,  May  17,  1913. 


192 


VEGETATIVE  OR   VISCERAL  NEUROLOGY 


As  a  polyglandular  endocrinopathic  syndrome,  scleroderma  has 
shown  a  many-sided  character.  Cases  are  known  with  associated 
h^'pophysis,  adrenal,  thyroid,  and  mesenteric  gland  disease.  The 
thjToid  (frequently  s^'philitic  thyroid  disease)  correlations  seem 
numerically  to  outnumber  the  other  in  the  cases  reported.^ 

No  psychogenic  cases  have  as  yet  been  analyzed,  but  inasmuch  as 
many  if  not  most  hyperthyroidisms  are  distinctly  psychogenic,  at 
least  in  the  initial  stages,  it  is  not  an  unreasonable  hypothesis  that 
psychogenic  factors  may  form  a  part  of  the  scleroderma  pathogeny. 


Fig.  71. — Scleroderma.     (Nammack.) 


The  chief  pathological  alteration,  increased  fibrosis,  is  largely  due  to 
an  imbalance  of  the  sjTnpathetic  branch  of  the  vegetative  system 
arcs.  This  increased  sympathicotrophic  action  may  result  from  a 
number  of  causes — diminished  thyroid  secretion,  diminished  adrenalin 
secretion  being  among  them.  .  The  more  exact  pharmacological 
dynamics  have  not  yet  been  definitely  worked  out.  Timme  has 
attempted  to  ascertain  the  resultant  pathology  of  oversyrapathetic 
stimuli  by  cutting  off  the  balancing  autonomic  stimuli  in  the  walls 
of  the  stomach.  His  experiments  throw  some  light  on  fibrosis  in  general 
and  should  be  consulted  in  any  study  of  a  lesion  resulting  in  a  chronic 
fibrosis  whether  of  the  skin,  the  stomach,  kidneys,  liver,  or  any  organ 
of  the  body." 

1  Marinesco  and  Goldstein:  Nouv.  iconog.  de  la  Salpetri^re,  Paris,  1913,  p.  272. 

2  Timme:  Loc.  cit.  Mosenthin:  Arch.  f.  Derm.,  cxviii,  p.  613.  Cassirer:  Loc.  cit. 
Lewandowsky:  Handbuch.  Laredde  et  Thomas:  Arch.  d.  med.  exp.,  1898.  Dinkier: 
D.  Arch.  f.  k.  med.,  1891,  48.  Roux,  Rev.  Neur,  1902,  p.  721.  Raymond  et  Alquier: 
Soc.  d.  m.  d.  Hop.  d.  Pari.s,  1904  (Spinal  cord  case). 


SWEAT  SECRETORY  MECHANISMS  193 

Treatment. — The  therapy  will  depend  upon  the  underlying  factors. 
The  endocrinous  and  psychogenic  cases  would  seem  to  offer  the  most 
opportunity  either  by  carefully  balanced  opotherapy  or  by  psycho- 
therapy. 


Fig.   72. — Scleroderma  with  selerodurryly.      (S.  P.  Goodhart.) 


Multiple  Neurotic  Gangrene  of  the  Skin.— The  condition  described  by 
this  title  may  be  mentioned.  It  is  a  dermal  process  in  which  the 
vasomotor  constriction  is  not  limited  solely  to  the  extremities,  but  to 
apparently  unrelated,  isolated  patches  of  the  skin. 

While  exaggerations  in  neuropaths  (hysteria),  or  in  psychoses 
(schizophrenia)  may  bring  these  changes  about  by  artefact,  malin- 
gering explains  only  a  small  number  of  them,  granting  that  the  term  is 
any  explanation  at  all,  for  why  people  malinger  still  remains  to  be 
understood. 

The  disorder  sets  in  with  a  burning  and  prickling  of  the  skin.  Then 
after  a  variable  length  of  time  (a  few  minutes  to  forty-eight  hours) 
swellings  occur,  blisters  form,  with  later  necrosis.  At  times  only  a 
circumscribed  edematous  bleb  forms. 

Sweat  Secretory  Mechanisms.— These  are  closely  related  to,  yet 
independent  of,  the  vasomotor  mechanisms.  They  probably  have 
independent  ganglion  cell  representation  in  the  lateral  horns.  In  general 
those  pharmacological  agents  which  increase  vasodilatation  (auto- 
nomic) increase  sweat  secretions,  yet  \asodilatation  may  occur  without 
sweating,  and  the  latter  may  occur  with  anemia  and  vasoconstriction 
(cold  sweat  of  fear,  sweating  of  face  in  migraine,  epileptic  aura,  etc.). 

(\'r\ical  sym])athetic  and  bulbar-autonomic  imiervation  is  jn'obable. 

and  cortical  connections,  though  still  topographically  unanalyzed  are 

certain  (unilateral  sweating  in  thalamic  lesions,  hemiplegia,  hysteria,- 

compulsion  neuroses,  schizophrenia).     Certain  anatomists  place  the 

13 


194  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

cortical  pathways  among  the  motor  tracts  in  the  internal  capsule. 
The  hypothalamus  is  made  a  midbrain  synaptic  station  by  some.^ 

Clinically,  sweating  is  increased  in  certain  hemiplegias,  and  in  herpes 
zoster.  Diminution  of  the  sweat  is  seen  in  certain  cases  of  polio- 
myelitis, multiple  sclerosis,  syringomyelia,  myelitis  and  tumor  of  the 
spinal  cord.  Great  variability  in  the  sweat  activity  is  seen  in  many 
psychopathic  individuals,  in  vagotonic  t^-pes  and  in  the  psychoneuroses, 
hysteria,  anxiety  states,  compulsive  states  and  in  hyperthjToidism. 

The  reactions  of  the  sweat  secretion  mechanisms  are  exquisitely 
sensitive,  as  is  seen  by  the  response  to  pain,  gastro-intestinal  cramp, 
carminatives,  nicotine,  anxiety  and  joy.  Veraguth's  pyschogalvanic 
reflex  experiments  show  that  a  close  relationship  exists  between  the 
skin  secretions  and  psjxhical  processes.  The  fineness  of  registration 
and  the  extreme  complexity  of  the  phenomena,  however,  militate 
against  the  practical  utility  of  the  galvanometer  tests. 

Pilomotor  System. — The  smooth  muscle  fibers  of  the  skin  are  under 
s.>Tnpathetic  innervation.  The  pilomotor  fibers  run  with  the  sensory 
fibers  (Higier),  each  sensory  nerve  carrying  fibers  from  about  five 
sjTiipathetic  ganglia  (Higier),  and  have  similar  topographical  (seg- 
mental) distributions.  Mechanical,  thermal,  and  electrical  stimuli 
cause  contractions.  The  erector  pilse  reflexes  (best  observed  by  side 
light)  are  particularly  responsive  to  cold.  The  frequently  felt  pares- 
thesise,  acroparesthesiae,  etc.,  of  psychoneurotics  depends  upon  these 
sjTnpathetic  reactions,  and  their  exact  observation  is  of  much  diagnostic 
importance.  Mackenzie's  observations  should  be  consulted  by  the 
interested  student  .^  The  feeling  of  cold  over  the  abdomen  after 
taking  cold  water  in  the  stomach  is  an  example  of  the  relationship 
of  the  sjTnpathetic  innervation  of  an  internal  organ  and  a  skin  area.^ 
A  large  number  of  analogous  phenomena  are  known.  Thus  among 
them,  mechanical  stimuli  of  the  plexus  pudendi,  as  in  cystitis,  rectal 
exploration,  prostatic  massage,  prostatitis,  causes  distinct  goose-flesh 
or  paresthesiae  in  the  region  of  the  motor  lumbar  plexus.  Erector 
pilse  crises  are  known  to  occur  in  tabes;  they  are  at  times  migraine 
equivalents.  Psychical  stimuli  may  lead  to  localized,  or  more  often 
to  generalized  reactions.  A  great  variety  of  extremely  important 
skin  hallucinatory  experiences  are  known  to  occur  in  dementia  precox, 
and  in  the  psychoneuroses,  hj^steria,  anxiety  states,  compulsive  states, 
(pathological  blushing,  etc.).  Their  correlation  is  only  just  being 
understood  through  the  results  of  psychoanalytic  research. 

Similarly  it  is  beginning  to  be  questioned,  if  not  affirmed,  whether  a 
whole  group  of  chronic  skin  disorders,  notably  certain  forms  of  eczema 
and  psoriasis,  and  which  are  stated  by  the  standard  dermatologists  to 
be  "nervous"  in  origin,  are  not  also  the  results  of  chronic  emotional 
conflicts  in  the  unconscious,  mediated  through  the  vegetative  nervous 
system,  with  or  without  related  endocrinopathies,  dysthyroidisms 
particularly     (endocrinosjuipathetic) .      If    this    be    so    appropriate 

'  Pee  Miiller  and  Glaser:  Deut.  Zeit.  f.  Nerven.,  vol.  xlvii  to  xlviii,  p.  365,  for  literature. 
^  Mackenzie:  The  Signs  of  Disease,  '  Head's  Zones,  Mackenzie'. 


PILOMOTOR  SYSTEM 


195 


psychotherapy  may  show  the  causal  relations  between  the  skin  dis- 
ease as  a  s\Tiibol  of  a  psychical  compromise  and  thus  the  scape-goat 
be  removed. 


Fig.  73. — Osteoporosis  copgenita  in  a  girl  aged  nine  years.     (A.  Josefson.i) 


Fh;.  74. — Osteoporosis  congenita  in  a  girl  aged  nine  years.      (.\.  Jo.sefson.) 

Bony  Syndromes. — A  host  of  bone  disorders  are  related  to  vegetative 
disturbances.    The  best  studied  are  the  atrophies  of  tabes  (Charcot 


'  Veihand,  a,  d.  Gebiet  u.  Roentg.  191(i. 


196  VEGETATIVE  OR  VISCERAL  NEUROLOGY 

joints)  due  to  interruption  of  autonomic  stimuli  because  of  the  syphilitic 
process/  and  the  changes  called  giantism  [thymus,  gonads,  pituitary] 
and  acromegaly  arising  from  increased  pituitary  function  are  a  few 
among  a  great  host  of  clinical  pictures  now  being  rapidly  analyzed. 
Mme.  Dejerine^  has  recently  described  a  whole  series  of  osteoarthrop- 
athies following  spinal  cord  injuries  in  war  accidents. 

Some  patients  with  chronic  rheumatoid  arthritis  recover  following 
polyglandular  therapy,  and  the  studies  of  Levi  and  Rothschild  seem 
to  implicate  the  th^Toid  more  particularly.     [See  Endocrinopathies.] 

Blood  Syndromes. — Knowledge  is  only  beginning  to  dawn  relative 
to  the  influence  of  the  vegetative  nervous  system  upon  the  blood. 
This  is  a  most  promising  field.  The  vegetative  nerve  supply  of  the 
bone  marrow  has  just  begun  to  be  investigated^  and  important  results 
relative  to  the  mechanism  of  the  formation  of  the  different  blood  con- 
stituents are  accumulating.  The  reaction  to  toxic  agents,  the  mechan- 
ism for  eosinophilia,^  of  various  anemias,  of  the  various  leukopenias  and 
leukoplasias  are  being  comprehended  through  the  vegetative  nervous 
system  supply  of  the  blood-making  organ  .^  The  best  studied  and 
apparently  most  widely  observed  blood  syndromes  of  vegetative  dis- 
order are  chlorosis  and  eosinophilia.^ 

Chlorosis. — From  the  time  of  the  early  h^-potheses  of  the  retention 
of  menstrual  blood  as  the  cause  of  chlorosis  to  the  present  this  prob- 
lem of  the  etiology  of  chlorosis  has  been  extremely  obscure.  Vege- 
tative nervous  disease  hypotheses  have  not  been  neglected,  however. 
Sydenham  (1705)  and  Grawitz,  among  modern  hematologists,  called  it 
hysteria.  What  they  understand  by  hysteria  is  hard  to  envisage. 
Copeland,  Hoefer,  Eisenmann,  Brinton  Hicks,  and  the  modern  Genoa 
school  (Giovanni)  have  conceived  it  thus.  The  general  formula  adopted 
by  them  is  that  chlorosis  is  an  endocrinosympathetic  dystrophy,  and 
exists  chiefly  in  two  forms  as  a  thyroid  and  a  suprarenal  chlorosis. 
It  is  originally  ovarian,  the  hormones  of  the  interstitial  ovarian  cells  (?) 
being  implicated.  What  the  real  dynamics  of  the  situation  is,  is  still 
obscure. 

Eosinophilia. — Many  forms  are  observed.  The  best  types  know  i  are 
those  due  to  various  parasites  (uncinaria,  taeniae,  trichinae)  and  to 
increased  activity  of  the  adrenal  glands.  Eosinophilia  is  a  frequent 
accompaniment  of  vagotonic  states  and  is  found  widely  in  the  agitated 
depressions  of  depressed  manics,  anxiety  hysterias,  and  anxious  com- 
pulsive states.  Here  fear,  conscious  or  unconscious,  is  the  etiological 
factor.    Fear  also  induces  the  increased  adrenalin  activity. 

'  Sterling,  W. :  Die  trophischen,  vegetativen  Erkrankungen  des  Knochensystems, 
Ztschr.  f.  d.  g.  Neur.  u.  Psych.  Referate,  vol.  ix. 

2  Arch,  dc  Med.,  January,  1919. 

'  Drinker:  Am.  Jour.  Physiol.,  June,  1916. 

*  Schwenker  u.  Schlecht:  Zeit.  f.  klin.  Med.,  1912,  Ixxvi,  p.  77. 

'  Skorazewski:  Zeit.  f.  exp.  Path.,  1912.  Borchardt:  Deut.  Arch.  f.  kUn.  Med.,  1912, 
cvi,  182.  Carpi:  Berl.  klin.  Wchnschr.,  1910,  2059.  Burr:  Jour.  Nervous  and  Mental 
Disease,  1899,  30.    Kaplan:  Am.  .Tour.  Med.  Sc,  1911,  cxlii,  702. 

"  Schwarz:  Eosinphilie,  Lubarsch  u.  Ostertag's  Ergebnisse. 


CHAPTER  III. 
THE  ENDOCRIXOPATHIES. 

INTERNAL  SECRETIONS. 

Medical  biology  has  not  yet  arrived  at  hj-potheses  sufficiently 
comprehensive  to  conceive  a  scheme  which  can  explain  all  of  the 
relations  of  the  vegetative  nervous  system  to  the  control  of  the  viscera. 
Some  suggestions  have  been  offered  concerning  the  action  of  the  vege- 
tative nervous  system  upon  the  gastro-intestinal  tract,  and  also  some 
ideas  relative  to  the  intricate  adjustments  of  internal  and  external 
organs  brought  about  through  the  bloodvessels  have  been  reviewed. 
Special  neurological  problems  in  which  direct  modification  of  the  vege- 
tative systems  in  the  eyes,  nose  and  throat,  lungs,  heart,  skin,  blood 
organs  and  bones  have  been  touched  upon,  all  too  hurriedly.  The 
problems  of  cellular  adjustment  now  demand  attention.  These 
concern  the  vital  phenomena  of  anabolism  and  katabolism,  and  the 
adjustment  of  all  of  the  organs  of  the  body  involved  in  the  elaboration 
of  special  substances,  which  are  of  importance  to  the  metabolism  of 
the  rest  of  the  organism. 

Thus,  what  part  is  played  by  the  nervous  system  in  the  carbohydrate 
oxidations  of  the  body,  acting  chiefly  through  the  lungs,  the  liver, 
the  pancreas,  and  suprarenals?  The  substances  of  the  hj-pophysis, 
its  hormones  or  active  principles,  what  are  they;  and  what  is  the 
interrelationship  between  them  and  growth  as  seen  in  the  clinical 
phenomena  of  infantile  dystrophies,  of  acromegaly,  and  a  number  of 
related  conditions?  Similar  questions  arise  for  discussion  concerning 
the  thjToid,  the  thymus,  and  the  adrenals.  The  pineal  gland  is  also 
a  glandular  formation,  likewise  the  parathjToids  ,  with  their  problem 
of  regulating  the  calcium  metabolism  not  yet  certainly  disposed  of. 
What  nervous  mechanisms  keep  the  other  constituents  of  the  body 
plasma  in  a  state  of  equilibrium,  so  that  all  tjpes  of  functioning  may 
go  on,  physicochemical,  sensorimotor,  and  psychical? 

This  entire  group  of  questions  cannot  even  be  asked  here.  It 
can  only  be  said  that  in  their  consideration,  one  sees  an  entirely  new 
country  opening  up  which  promises  to  greatly  modify  the  geography 
of  our  present  neurological  schemes,  since  Brown-Sequard  in  1889 
reached  out  for  immortality  by  his  use  of  testicular  substances.  It 
may  be  recalled  that  as  early  as  1775  Theophile  de  Bordeu  founded 
the  vitalistic  school  and  taught  that  each  organ  of  the  body  elaborated 
a  specific  substance.  So  far  as  space  permits  the  more  essential 
interrelationships  and  correlations  will  be  brought  out  in  the  dis- 
cussion of  the  various  diseases.  They  are  at  present  grouped  under 
their  respective  glands.  Varying  points  of  view  are  developing  rapidly. 

The  student  of  the  endocrinopathies  should  constantly  keep  in  mind 
the  fact  that  any  clinical  picture  seen  at  ony  one  time  represents  only 

(197) 


198  THE  ENDOCRINOPATHIES 

a  cross-section  of  a  dynamic  process  which  may  be  modifying  daily  or 
possibly  have  come  to  a  temporary  or  permanent  standstill.  Thus  a 
lymphatic  giant  picture  at  the  age  of  sixteen  or  seventeen  may  be  con- 
verted into  a  hyperpituitary  syndrome  a  few  years  later  from  failure 
of  thjTnus  involution ;  an  inferior  adrenal  system  failing  to  compensate 
throws  the  balance  to  its  chief  adjunct,  the  pituitary,  thus  the  mutual 
interactions  and  compensations  are  to  be  found  in  this  field  as  in  all 
other  fields  of  medicine  where  complex  dynamic  activities  are  con- 
stantly in  play  and  shifting  from  year  to  year.  As  in  manifest  bodily 
adjustments  the  expressed  symptom  may  not  be  due  directly  to  the 
disorder  of  the  organ  showing  the  most  evident  sign  of  disease.  Thus  a 
respiratory  difficulty  is  not  always  due  to  a  lung  disease.  It  may  arise 
from  distant  and  elusive  sources,  kidney,  heart,  emotional,  etc.  Thus 
an  endocrinopathy  is  not  necessarily  a  disease  of  the  gland  itself.  If 
this  principle  had  been  kept  in  mind  much  meddlesome  surgery  of  the 
thyroid  would  have  been  avoided,  as  so  frequently  a  hyperthyroid 
activity  may  be  a  functional  activity  trying  to  make  good  for  a  defi- 
ciency elsewhere.  To  cut  out  the  organ  apparently  diseased  only 
destroys  the  effort  nature  is  making  to  save  the  body. 

The  chief  available  literature  summaries  are  Biedl,^  Sajous,-  Falta,^ 
Lewandowsky,*  Parhon  and  Goldstein,  Levy  and  Rothschild,  Pende,* 
Laignel-Lavastine''  and  special  monographs  to  be  mentioned  under  the 
separate  diseases,  such  as  Gushing  on  the  Pituitary,  Klose  and  Vogt  on 
the  Thymus,  Morel  on  the  Parathyroid,  Sattler  on  Basedow's  Disease, 
Bell,  Sex  Complex,  Harms,  Keimdrusen,  etc. 

The  glands  in  question  may  be  grouped  in  general  according  to  their 
phylogeny  and  embryology  as  follows: 

1.  From  the  buccal  cavity: 

(a)  Thyroid  (phylogenetically  also  gonadal) . 
(6)  Pituitary  (posterior  lobe  of  hypophysis) . 

2.  From  the  nervous  tissues: 

(a)  Hypophysis  (anterior  lobe) . 
(6)  Ghromaffin  tissue  (suprarenal). 

3.  From  the  branchial  arches: 

(a)  Parathyroids. 
(6)  Thymus. 

4.  From  the  intestine: 

(a)  Parathyroids. 

(6)  Mucosa  of  small  intestine. 

5.  From  the  mesothelium  of  the  genital  ridge: 

(a)  Gonads  (sex  glands) . 

(6)  Interrenal  bodies. 
It  is  highly  important  to  remember  that  an  endocrinous  gland 
rarely  represents  in  itself  the  entire  material  which  may  function. 
Accessory  glands,  or  distributed  bits  of  specific  glandular  substances 

1  Internal  Secretions.  ^  The  Internal  Secretions. 

3  Die  Erkrankungen  der  Blutdriisen,  exhaustive  and  suggestive. 
■•  Handbuch  der   Neiirologie,  vol.  iv,   Special   Neurology,   a  collection  of  valuable 
monographs.  ^  Endocrinologia,  1916. 

6  Nervous  and  Mental  Disease  Monograph  Series,  1919,  No.  30. 


INTERNAL  SECRETIONS  199 

are  widely  distributed.  Failure  to  bear  this  in  mind  vitiates  many 
of  the  results  obtained  from  physiological  extirpation  experiments. 
Notably  is  this  true  for  many  studies  of  the  adrenals. 

Among  the  earlier  attempts  at  correlation  of  the  group  of  disorders 
of  the  blood  glands  or  internal  secretory  glands  were  those  of  Claude 
and  Gougerot,  and  Laignel-Lavastine. 

A  recent  systemization  of  this  latter  author  is  suggestive.  In  the 
first  place,  one  can  distinguish  (a)  neurological  symptoms  as  a  part 
of  an  endocrinopathy;  (h)  endocrinous  disturbances  in  neurological 
s^Tidromes,  and  (c)  double  forms  of  endocrino-neuro-endocrinopathies 
and  neuro-endocrino-neuropathies.  The  endocrinopathies,  as  outlined 
by  Laignel-Lavastine,  may  thus  be  rapidly  reviewed.  It  is  still  to  be 
proved  how  valid  certain  of  these  conclusions  are.  They  are  suggestive, 
how^ever,  and  entitled  to  be  tried  out. 

I.    Uniglandular  Endocrinopathies: 

1.  Th}Toid. 

(1)  Myxedema — ^hjTDof unction. 

(2)  Exophthalmic  goiter — hj^jerf unction. 

(3)  ThjToid    insufBciences. 

(4)  Th^Toid  instabilities. 

2.  Parath\Toids : 

(1)  Tetany. 

(2)  Paralysis  agitans  (?). 

3.  Thymus: 

(1)  Vagotonic  s^nnptoms  of  exophthalmic  goiter. 

(2)  Myasthenia  gravis. 

(3)  Thjinoprivous  idiocy. 

4.  Suprarenal: 

(1)  Addison's  disease — ^h\'pof unction. 

(2)  Genito-adrenal  syndrome. 

5.  S>Tnpathetic  paraganglia. 

6.  Pancreas: 

(1)  Diabetes  mellitus. 

7.  Hj-pophysis: 

(1)  Adiposogenitalis  of  Frohlich. 

(2)  Acromegaly  (hyperf unction) . 

(3)  Gigantism. 

(4)  H^'pophyseal  insufficiency. 

8.  Pineal: 

(I)  Macrogenitosomia. 

9.  Gonads  (ovary,  testicle): 

(1)  Infantilism. 

(2)  Acquired  ovarian  insufficiency. 

(3)  H}T3erovarianism. 

(a)  Infantilism. 

(b)  Acquired  testicular  insufficiency  (castration) 

(c)  Eunuchism. 
10.  Prostate: 

(1)  IIyi)o-  and  hyperprostatic  syndromes. 


200  THE  ENDOCRINOPATHIES 

II.  Polyglandnlar  Endocrinojjathies: 

1.  ThjToid  predominances. 

(1)  Exophthalmic  goiter  with  thymic  hypertrophy. 

(2)  ]\Iyxedema  with  thymic  h^'pertroph^^ 

(3)  Acromegahes  with  o^'a^ian  insufficiency,  at  times 

resembhng  exophthalmic  goiter,  at  times  myx- 
edema. 

2.  Ovarian  predominance : 

(1)  Thyroid  reactions  with  ovarian  insufficiency. 

(2)  Dyshyperovarianism  of  hypothyroidism. 
(.3)  Thyroovarianism. 

3.  With  hypophyseal  predominance: 

(1)  Infantile  giants.     Feminism,  eunuchism,   crypt- 

orchism,  pseudohermaphroditism. 

(2)  Acromegalics  with  defect  symptoms.  Infantilism, 

amenorrhea,  obesity,  asthenia. 

(3)  Acromegalics  with  hyperactivity  symptoms,  ex- 

ophthalmos, arterial  hypertension,  atheroma. 

4.  With  suprarenal  predominance: 

(1)  Addisonians  with  amenorrhea,  frilosity,  tetany,  or 

goiter. 

(2)  Goiter,  acromegalics,  giants  with  diabetes. 

5.  Without  marked  predominance. 

The  French  school  has  carried  these  deductions  to  great  lengths, 
and  have  developed  an  organotherapy  of  startling  complexity,  but  one 
which  should  be  carefully  reviewed  and  checked  up.  Here  we  purpose 
to  discuss  those  disorders  of  the  internal  secretions  with  pronounced 
disturbance  of  the  nervous  system,  beginning  with  those  best  known. 
The  student  is  reminded  that  a  text-book  can  only  deal  with  the 
most  pronounced  types,  and  those  concerning  which  there  is  a  certain 
unanimity  of  opinion,  hence  for  further  study  of  the  mass  of  material 
reference  should  be  made  to  the  literature  here  quoted."^ 

We  shall  therefore  take  up:  (1)  the  thyreopathies,  (2)  the  hypophy- 
seal disorders,  and  (3)  the  disorders  of  the  parathyroid,  thymus, 
adrenals,  and  sex  glands.  Finally,  some  suggestive  relationships 
between  disease  of  certain  viscera,  liver,  kidneys,  spleen,  etc.,  and  the 
nervous  system. 

Before  passing  to  the  detailed  study  of  these  forms  a  word  may  be 
said  concerning  the  interrelationship  of  these  various  endocrinous 
glands.  It  is  highly  probable  that  these  glands  work  in  u.iison  and 
that  such  regulatory  synchronism  is  mostly  brought  about  through 
the  vegetative  nervous  system.  Pure  chemical  regulation  may  take 
place,  but  it  is  becoming  more  and  more  evident  that  the  reactions 
w^hich  bring  more  or  less  hormone  to  the  blood  are  mediated  by  the 
sjTnpathetic  fibers  more  particularly.  These  hormones  in  turn  modify 
the  electrical  carrying  capacity  of  the  fibers  and  the  resistances  at  the 

'  Roseabloom:  Functional  Methods  for  Studying  Diseased  Conditions  of  Certain 
Endocrinopathies,  Interstate  Med.  Jour.,  1919,  vols,  xxv,  xxvi,  x,  xi,  xii. 


PLATE   V 


Objects  Removed  from  Stomach  of  Patient  Suffering  from 
Gastric  Tetany     (Warbasse.) 


INTERNAL  SECRETIONS  201 

synapse  and  thus  modify  function.  The  whole  series  of  processes  are 
highly  complex  and  the  student  is  referred  to  special  works^  with  the 
express  suggestion  that  science  is  dependent  upon  h^^Dotheses  for  its 
advance  but  these  should  be  constantly  checked  up  by  the  objective 
findings,  and  put  into  the  scrap  heap  so  soon  as  better  functional  tools, 
i.  e.,  hjTpotheses,  are  advanced. 

The  interrelationships  of  the  various  endocrinous  glands  have  been 
ably  illustrated  by  Noel  Paton,  a  reproduction  of  certain  of  his  charts 
or  diagrams  being  given  here. 

As  Paton  well  remarks,  these  may  well  be  a  grotesque  parody  of 
what  will  ultimately  be  found  to  be  the  relationship  of  the  activities 
of  these  organs.  "They  are  probably  as  near  the  truth  as  those  quaint 
ancient  maps  of  the  Indies  with  their  'here  be  much  gold'  scrawled 
across  them  which  served  as  the  charts  of  our  forefathers,  but  if,  like 
them  they  merely  indicate  the  direction  which  further  investigation 
should  take  and  suggest  lines  of  attack,  they  w^ill  have  served  their 
purpose." 


Fig.  75. — To  show  the  probable  influence  of  the  various  endocrinous  structures  on 
one  another.     The  following  explanations  apply  to  this  and  to  the  three  succeeding 

figures:  stimulation; inhibition.      The   arrow  indicates   the   direction 

of  action.  Hyp.,  hypophysis;  Par.,  parathyroid;  Ch.,  Chromaffin  system;  Art.,  artery; 
Pit.,  pituitary;  Thm.,  Thymus;  Th.,  thyroid;  G.,  gonads;  B.,  bone;  In.,  interrenal;  Pan., 
pancreas;  M.,  muscle.     (Paton.) 

The  direct  and  profound  action  of  the  secretions  of  the  sexual  glands 
(gonads)  upon  the  body  is  seen  in  every  tissue  of  the  body.  How  far 
their  action  is  facilitated  and  how  far  checked  by  other  endocrinous 
organs  is  not  yet  entirely  worked  out.  The  thymus  supplements  the 
action  of  the  testes  secretion.  Its  relations  to  the  ovaries  is  not  so 
certain.  It  exercises  a  checking  action  on  the  male  gonadal  activity 
which  in  its  turn  acts  reciprocally  on  the  th\7nus.    (See  Figs.  75  to  78.) 

The  removal  of  the  thjToid  checks  the  growth  of  the  gonads.  Cas- 
tration acts  less  on  the  th^Toid,  although  menstruation,  childbirth, 
and  the  menopause  cause  marked  th\Toid  activity.  (See  Th\Toid, 
Figs.  75  and  78.) 

The  destruction  of  the  pituitary  leads  to  gonad  atrophy  and  recip- 
rocally castration  causes  hypertrophy  of  the  pituitary.    The  secretion 

>  Noel  Paton:  Nervous  Regulators  of  Metabolism.  Higier:  Vegetative  Nervous 
System,  Jour.  Nerv.  and  Ment.  Dis.,  Monograph  Series,  1919. 


202 


THE  ENDOCRINOPATHIES 


of  both  stimulate  the  growth  of  the  long  bones,  the    uncontrolled 
activity  of  the  former  leading  to  gigantism  and    acromegaly.     The 


Fig.  76. — To  show  the  probable  mode  of  action  of  the  various  internal  secretions  on 
the  mobilization  of  sugar  in  the  liver.     (Paton.) 


Fig.  77. — To  show  the  probable  mode  of  action  of  certain  of  the  internal  secretions  upon 
the  spinal  reflex  arc.     (Paton.) 


Fig.  78. — To  show  the  probable  mode  of  action  of  the  internal  secretions  on  the 
growth  of  muscle  and  of  bone  and  of  the  connective  tissues.  The  possibility  of  this  being 
a  vasomotor  reflex  mechanism  is  indicated  by  lines  marked?     (Paton.) 


action  of  the  gonadal  secretions  is  to  check  the  pituitary  activity 
and  the  increase  in  size  of  the  eunuch  is  possibly  a  response  to  this 


INTERNAL  SECRETIONS  203 

unchecked  h^-pophyseal  activity.    The  gonads  are  not  alone  in  hinder- 
ing the  pituitary  action. 

Suprarenal  and  gonadal  activity  are  closely  related  and  suprarenal 
loss  is  usually  accompanied  by  genital  aplasias  or  anomalies.  Paton 
has  suggested  the  identity  of  certain  elements  of  these  tissues  and  that 
the  suprarenals  constitute  a  sort  of  bridge  or  intermediary  between 
the  bodily  and  the  sexual  cells. 

The  th^Toids  and  pituitary  are  closely  related.  Removal  of  one 
causes  h>-pertrophy  of  the  other.  (Fig.  67.)  They  thus  mutually 
check  each  other  in  part  and  are  also  cooperative,  the  pituitary  needing 
the  thyroid  to  complete  its  activities.  H^^Derthyroid  activity  does  not 
lead  to  hjN-perplasias  of  connective  or  bony  tissues  as  does  hj-perpituitary 
action;  the  reciprocal  autonomic  and  sjTnpathetic  nerve  activity  is 
not  exactly  similar;  although  diminished  activity  of  both  substances 
may  lead  to  diminished  bony  growth — atrophy.  The  vegetative 
mechanism  of  this,  however,  has  not  yet  been  elucidated. 

The  action  of  thj-mus  on  th}Toid  is  far  from  clear,  but  the  tendency 
is  to  show  a  reciprocal  checking  action  especially  on  the  neuromuscular 
apparatus.  The  problem  of  myasthenia  gravis  has  been  thought  to 
lie  behind  this  reaction.  Feeding  th\Tnus  to  salamander  larvae  induces 
parathjToid  atrophy  and  the  development  of  tetany,  probably  related 
to  the  diminished  calcium  regulation  of  the  parathjToid  substances. 
Dustin  sees  in  the  thjinus  a  glandular  reservoir  for  nuclein  metabolism 
mechanisms.  The  pituitary  undoubtedly  plays  a  large  part  in  phos- 
phorus distribution. 

The  thyroids  and  parathjToids  have  distinctly  different  and  even 
antagonistic  activities :  The  former  seems  to  be  related  more  distinctly 
to  the  iodine,  the  latter  to  the  calcium  metabolism  of  the  body.  Just 
how  they  are  regulated  through  the  vegetative  nervous  system  is 
unknown.  Calcium  is  of  pronounced  value  in  neuromuscular  activity 
as  the  phenomena  of  tetany  show.  Myasthenic  states  in  general  and 
myasthenia  gravis  in  particular  are  more  directly  related  to  disordered 
thyroid  and  thj-mus  activities.  Lundborg  has  suggested  that  the  para- 
thyroid function  plays  some  part  in  the  reaction. 

The  thjToid  acts  on  the  pancreas  chiefly  through  its  action  on  the 
liver  sympathetic  fibers.  Sugar  mobilization  and  release  are  brought 
about  through  modified  th^Toid  and  pancreatic  action,  which  latter 
prevents  the  mobilization  of  sugar  in  the  liver.  Thus  glycosuria  is 
frequent  in  hj-perthjToid  states.     (See  Fig.  66.) 

The  complicated  interrelationships  cannot  be  entered  into  more 
fully.  The  chief  available  literature  has  been  indicated.  One  point, 
however,  should  be  emphasized  and  that  is  that  the  activities  of  the 
internal  secretion  organs  are  all  under  vegetative  nervous  system 
control.  The  active  substances,  hormones,  if  one  wishes,  are  not 
entirely  independent  chemical  activators,  they  are  under  sympathetic 
and  parasympathetic  (autonomic)  control.  The  output  of  iodine,  of 
calcium,  of  adrenalin,  of  h.\T3ophysin  and  of  all  of  the  substances 
thus  far  known  or  named  is  controlled  almost  exclusively  by  the 


204  THE  ENDOCRINOPATHIES 

nervous  system.  The  internal  secretions  act  through  the  nervous 
system.  While  it  may  be  shown  that  within  an  organ  itself  primary 
chemical  regulators  may  be  effective — thus  one  must  explain  the  posi- 
tive and  negative  tropisnis  within  the  cells  of  an  organ  in  its  initial 
response  to  a  disturbance  of  cellular  adjustment — yet  the  chief  activ- 
ities of  the  internal  secretions  are  brought  about  by  neurochemical 
regulators,  as  Paton  terms  them. 

Just  as  the  complicated  sensorimotor  integrations  are  effective 
in  governing  the  muscular  activities  of  the  human  body,  so  the  integra- 
tion of  neurochemical  regulators,  taking  place  at  the  physicochemical 
level,  is  effective  in  adjusting  the  metabolism  of  the  body  cells.  Hor- 
mones are  not  the  activators  primarily;  they  are  the  servants  of  the 
vegetative  nervous  system.  All  of  the  endocrinopathies  are  really 
polyglandular  syndromes  and  markedly  under  emotional  influences. 
This  as  a  fact  has  been  known  for  centuries  but  is  now  being  demon- 
strated experimentally  in  the  physiological  laboratories.  (Pavlov, 
Cannon,  etc.). 

THE  THYREOPATHIES;  THYREOSES.i 

The  Thvroid. 

In  the  lowest  vertebrates  the  thyroid  was  intimately  connected  with 
the  genital  ducts,  they  were  uterine  glands,  but  from  Petromyzon 
upward  it  has  lost  that  connection,  although  it  is  known  that  an 
intimate  (polyglandular)  association  still  persists  (menstruation, 
pregnancy  and  swelling  of  gland,  genital  excitement  and  hyper- 
functioning and  other  relationships  to  be  discussed).  In  the  human 
embryo  it  seems  to  be  cut  off  from  the  foregut.  It  is  intensely  vascular 
and  its  chemistry  is  unique.  It  contains  a  comparatively  high  per- 
centage of  iodine,  also  phosphorus,  arsenic,  bromine  and  sulphur.  The 
chief  hormone  Kendall  names  the  alpha  iodine  compound.  It  is  usually 
associated  with  colloid  material,  the  presence  of  which  is  a  general 
though  not  a  certain  index  of  its  activity. 

The  nerve  supply,  thyroid  nerves,  arises  from  the  cervical  sympathetic, 
the  fibers,  mostly  non-medullated,  passing  from  the  middle  (inferior) 
cervical  ganglion  from  the  thyroid  plexuses  whose  fibers  penetrate 
the  gland.  Its  chief  visible  supply  is  autonomic  (vagus)  but  sym- 
pathetic fibers  also  are  present.  The  fibers  go  to  the  bloodvessels  and 
also  to  the  glandular  cells.  The  character  of  the  receptor  and  effector 
cells  of  the  glandular  cells  is  not  yet  known  but  there  is  some  evidence 
that  the  visceral  reflex  passes  by  means  of  the  sjTnpathetic  fibers  and 

'  Sattler:  Basedow's  Krankheit,  one  of  the  most  complete  monographs  on  the  subject. 
Collected  papers.  Mayo  Clinic,  1915.  McCarrison:  The  Thyroid  Gland,  1917.  Crotti: 
The  Thyroid  and  Thymus  Glands,  1917.  Richardson:  Thyroid  and  Parathyroid. 
A.  Josefson  of  Stockholm  has  contributed  many  important  papers.  These  are  the  chief 
large  monographs.  The  periodical  literature  will  be  best  consulted  in  Endocrinology, 
a  quarterly  devoted  to  this  branch  and  in  the  Current  Literature  department  of  the 
Journal  of  Nervous  and  Mental  Disease. 


THE  THYREOPATHIES  205 

not  by  the  cranial  autonomic  ones.^  S}Tnpatlietic  section  causes 
marked  atrophy  of  the  gland,  vagus  section  none. 

The  chief  endocrinopathies  produced  by  thyroid  disease  are  those 
due  to  lessened  function,  hypothyreoses,  and  those  due  to  an  excess  of 
function  h}-perth}Teoses.  While  the  iodine  is  the  most  striking  ion  in 
the  secretion  it  is  probably  not  the  only  one  in  producing  the  striking 
metabolic  disturbances  of  th}Toid  disorder. 

Certain  h^perfunction  cases,  even  with  toxemias,  are  not  due  essen- 
tially to  thyroid  disease.  They  are  not  primary  h\'perth\Teoses.  They 
are  usually  overcompensatory  reactions  chiefly  to  inferior  or  h}T)o- 
adrenal  states.  These  latter  are  frequently  conditioned  by  exhaustion 
(physical  or  fear  states)  and  thus  caused  the  great  amount  of  hyper- 
thjToid  states  seen  in  the  soldiers  in  the  World  War  of  1914-1918. 
Many  of  these  and  related  t^pes  are  often  quickly  relieved  by  pitui- 
tary extract  1  to  3  gr.  daily,  under  careful  scrutiny.  The  pituitary 
compensates  for  both  the  adrenal  and  thyroid.  A  large  thyroid  with 
defective  adrenals  and  tightly  bound  in  pituitary,  Timme  has  shown, 
is  the  type  which  reacts  better  to  pituitary  extract  than  to  surgical 
procedures.  The  .r-ray  of  the  sella  turcica  shows  the  small  restricted 
pituitary  which  cannot  compensatorily  h^-pertrophy  for  the  defective 
adrenals,  this  throws  the  job  over  to  the  thyroid,  which  enlarges. 
Surgery  here  is  worse  than  useless  unless  the  overcompensation  is 
threatening  life. 

Hypothyreoses;  Myxedema. — The  chief  pronounced  hypothyreoses 
are  grouped  under  the  symbols  myxedema  and  cretinism.  There  is 
an  important  group  of  cases  in  which  less  marked  insufficiencies  are 
found  and  which  need  close  study.  Three  main  types  of  the  former, 
congenital,  idiopathic,  and  operative,  are  described,  while  sporadic, 
endemic,  and  irregular  types  of  cretinism  are  distinguished.  The 
whole  group  may  be  considered  as  one,  i.  e.,  hypo-  and  athyreoses. 
The  various  subgroups  have  grown  up  clinically  since  Gull,  in  1873, 
first  called  attention  to  myxedema.  They  are  still  in  great  need  of 
clearer  differentiation  and  description,  especially  the  milder  and  the 
variable  types. 

Operative  m^-xedema  (cachexia  thyreopriva)  has  been  the  best 
studied  type  since  Kocher,  in  1883,  called  attention  to  it,  one  year 
after  Haddon  had  shown  the  relationship  between  myxedema  and  the 
thyroid . 

Symptoms. — The  chief  symptoms  are  present  in  the  skin,  nervous 
system,  thyroid,  circulatory  apparatus,  temperature,  digestive  tract, 
blood,  urine,  bones,  and  general  metabolism. 

Skin. — Plere  there  occurs  a  general  gradually  increasing  swelling, 
most  marked  in  the  hands  and  head.  It  seems  edematous  and  yet 
does  not  j)it  on  })ressure.  The  thickness  of  the  lips  causes  an  eversion 
or  hanging  down  of  the  same.  The  folds  of  the  skin,  especially  of  the 
forehead,  are  more  distinct  than  is  usual.     Irregular,  flattish,  fat- 

1  Cannon  and  Cattell;  Am,  Jour,  Physiol,,  1916. 


206 


THE  ENDOCRINOPATHIES 


like  deposits  are  preseit  in  different  parts  of  the  body,  often  being 
most  marked  in  the  supraclavicular  region. 


August  3,  1914.  August  17,  1914.  November  27,  1914. 

Fig.  79. — Infantile  myxedema.     Ten  years  old.     Treated  by  large  doses  of  thyroid 
tablets.     (A.  Josefson.) 

The  hands  are  apt  to  be  clumsy  and  thick,  the  skin  of  the  back  of  the 
hand  being  much  thickened.  The  feet  may  show  similar  changes. 
The  whole  skin  is  whitish  and  dry;  it  scales  readily  and  rarely  shows 
any  perspiration.  Diaphoretic  drugs  even  are  unable  to  bring  about 
any  marked  perspiration,  and  the  mucous  membranes  are  apt  to  be 


Fici.  80. — Hutchinson  teeth,  nonsyphilitic,  hypothyroid  stigma.     (A.  Josefson.') 

dry  and  not  easily  irritated  to  cause  exudates.    Yellowish  pigmenta- 
tion may  occur.    The  hair  breaks  easily  and  is  apt  to  be  badly  and 


1  Dermat.  Wochenschr,,  1914. 


THE   THYREOPATHIES  207 

sparsely  developed.  The  nails  are  brittle,  develop  slowly,  and  sKow 
irregular  markings.    The  teeth  also  develop  badly.     (See  Fig.  80.) 

There  is  a  sense  of  tension  in  the  skin,  and  coldness  of  the  extremities 
seems  universal.  This  is  made  much  worse  in  winter,  with  marked 
tendency  to  chapping  and  frost-bites. 

The  nervous  system  shows  a  number  of  defects  varying  with  the 
grade  of  h^-pothyreosis.  Any  of  the  cranial  nerves  may  show  defec- 
tive development.  The  cerebrospinal  nerves  may  be  deficieat.  The 
motility  as  well  as  the  sensibility  is  diminished  both  as  a  result  of 
peripheral  and  central  maldevelopment.  The  changes  in  sensibility 
are  furthermore  augmented  by  the  localized  skin  changes.  The  reflexes 
are  not  markedly  changed.  The  gait  is  usually  wide-based  and  clumsy 
and  due  in  part  to  the  mental  dulness.  Defect  in  thyroid  substance 
seems  to  hinder  the  regeneration  of  cut  or  injured  peripheral  nerves. 

Mentally  a  great  variety  of  changes  may  be  observed.  They 
are  usually  in  the  nature  of  defect.  There  is  defect  of  memory,  atten- 
tion is  diminished,  thinking  goes  on  more  slowly,  but  may  be  of  fair 
capacity.  There  is  usually  a  loss  of  initiative,  and  emotional  dulness 
goes  along  with  the  sensory  losses  and  motor  reluctance.  The  speech 
is  apt  to  be  slow,  as  are  other  motor  acts.  It  is  monotonous  and  the 
thickened  lips  further  contribute  to  make  it  at  times  unmtelligible. 
The  whole  appearance  of  the  patient  is  one  of  gradually  advancing 
stupidity  which,  if  there  is  no  relief,  goes  on  to  more  profound  defect 
states — dementia . 

The  thyroid  itself  is  usually  much  diminished  in  size,  or  not  at  all 
palpable.  Though  palpable,  its  active  secretory  substance  is  usually 
defective. 

The  circulatory  apparatus  shows  little  abnormality.  The  heart 
action  is  usually  normal — the  larger  vessels  may  be  felt.  Vaso- 
constrictor action  is  prominent  and  is  responsible  for  the  cold  extremi- 
ties and  possibly  some  of  the  dryness  of  the  skin. 

The  cold  sensations  are  not  subjective  alone,  as  there  seems  to  be  a 
fairly  constant  diminution  in  the  bodily  heat,  as  is  also  seen  in  hypo- 
pituitarism. Digestive  discomforts  from  dry  mouth  and  enlarged 
tongue  are  frequent.  There  is  not  infrequently  diminished  muscular 
tone  and  deficient  secretions  in  the  entire  digestive  apparatus  with 
obstinate  constipation. 

Genital  anomalies  are  frequent,  consisting  of  irregular  or  suppressed 
menstruation  or  diminished  potency.  The  organs  themselves — testes, 
ovaries — may  be  diminished  in  size  and  infantile;  there  is  defective 
hairy  development. 

The  blood  shows  fairly  constant  eosinophilia,  the  clotting  time  is 
increased,  and  the  fibrin  content  above  the  average. 

The  urine  is  not  characteristically  altered,  save  that  its  quantity  is 
usually  decreased. 

Metabolism  is  slowed  down  in  many  directions.  Oxygen  exchange 
is  reduced,  the  calories  consumed  being  markedly  diminished.  The 
nitrogen  output  is  less,  as  well  as  that  of  the  purin  derivatives.    The 


208 


THE  ENDOCRINOPATHIES 


Fig.  81.— March  5,  1913. 


Fig.  82, — January  5,  1915. 


Fig.  83.— March  5,  1913. 


Fici.   Si. — January  .5,  I'Jlo. 
Figs.  81,  82,  83  and  84. — Hypothyroidism.     Effects  of  thyroid  in  large  doses 
14  on  skeleton.      (A.  Josefson.) 


210 


THE  ENDOCRINOPATHIES 


calcium-magnesium  metabolism  is  not  modified  save  in  those  operated 
upon  and  in  whom  the  parath\Toids  are  also  disturbed.  Carbohydrate 
tolerance  is  high. 

The  bony  system  is  variously  altered.  Here  the  amount  of  change 
and  its  diversity  depends  largely  upon  the  age  of  the  patient  at  the 
onset  of  the  disorder.  The  long  bones  fail  to  grow  normally  and  those 
with  late  ossification  centers  fail  to  undergo  complete  development. 
The  skull  is  apt  to  be  macrocephalic,  but  the  thickness  of  the  bone 
may  diminish  the  interior  capacity  a  great  deal.  In  hypothyroid  cases 
fractures  are  apt  to  heal  slowly.  The  fontanelle  in  young  patients  is 
apt  to  remain  open.  The  changes  in  myxedema  are  not  those  seen-  in 
rachitis  or  chondrostrophia. 


Fig.  85  Fig.  86 

Figs.  85  and  86. — Myxedema  treated  with  thyroid  in  great  doses.      (Josefson.) 

Types. — The  idiopathic  form  usually  begins  with  changes  in  the 
skin,  and  is  often  accompanied  by  neuralgic  pains.  A  patient  recently 
seen  was  diagnosed  as  a  tabetic.  The  skin  of  the  face  is  often  first 
affected  and  usually  the  extremities  are  implicated  very  gradually. 
The  sj-mptoms  may  all  come  on  within  a  few  weeks,  but  usually  their 
development  occupies  months.  Women  are  much  more  frequently 
affected,  and  usually  about  the  menopause  period.  In  these  cases  the 
hypotliATeosis  may  be  diagnosed  as  a  "menopause  neurosis."  A  not 
unusual  result  of  the  cessation  of  the  menstrual  function  is  an  over- 
active thyroid,  but  in  other  cases  the  reciprocal  stimulation  which  is 
pronounced  between  ovary  and  thyroid  suffers  with  the  diminution  of 
the  ovarian  function. 


THE  THYREOPATHIES 


211 


Operative  myxedema  is  now  comparatively  rare,  since  the  essential 
relationships  have  been  pointed  out.  The  tetany  sjTnptoms  often 
seen  in  the  earlier  and  badly  operated  cases  were  due  to  the  para- 
thjToid  removal. 

Congenital  forms,  th^Teoaplasias,  occur  in  children  usually  of  normal 
birth  and  average  development  up  to  about  the  time  of  weaning — if 
not  breast-fed  usually  earlier  (thyroid  in  mother's  milk).  The  symp- 
toms then  develop  rapidly,  and,  as  a  rule,  are  very  extreme.  The  irreg- 
ular, imperfectly  developed,  cretinoid  pictures  are  not  the  usual  ones  in 
congenital  myxedema;  as  Eppinger  has  remarked,  there  are  few  "half- 
way" congenital  thyreoaplasias.  The  female  sex  preponderates  and 
there  are  no  geographical  limitations  as  in  endemic  cretinism.  Neither 
is  there,  as  a  rule,  any  goitrous 

family  history  as  is  often  found  ^S^ 

in  cretinisju. 

These  little  patients  forget  to 
suckle  and  to  swallow.  The  skin 
becomes  folded,  the  nose  broad, 
the  eyes  deeply  sunken,  the  nasal 
wings  widely  spread  apart.  They 
are  mouth-breathers,  with  swollen, 
not  infrequently  protruding,  cya- 
notic tongues.  Salivary  increase 
is  often  present .  The  hair  is  badh* 
developed,  the  face  that  of  an  old 
man.  The  head  grows  in  size, 
but  the  rest  of  the  body  stays 
behind  with  marked  dispropor- 
tion in  length  and  breadth 
throughout.  The  epiphyses  do 
not  ossify,  and  the  centers  of  ossi- 
fication, especially  in  the  hands, 
fail  to  develop.  The  fontanelles 
remain  open  perhaps  until  the 
twelfth  or  fifteenth  year,  and  the  teeth  are  slow  in  appearance.  The 
body  is  apt  to  be  fat  and  the  abdomen  especially  swollen,  in  part 
from  gas  and  obstinate  fecal  accumulations.  Umbilical  hernia  is 
frequent.  Other  defects  are  often  present  in  heart,  palate,  and  other 
structures,  and  they  die  early  with  the  general  mental  symptoms  in 
part  described. 

Diagnosis. — Nephritic  edema  and  other  skin  edemas  must  at  first 
be  ruled  out,  especially  ovarian  pseudoedema  of  the  menopause  and 
rare  forms  of  s;^'philitic  or  familial  neurotrophic  eflema.  Chondro- 
strophia  must  be  separated  from  the  cretinoid  complications.  The 
relations  to  cretinism  are  close.  There  are  differences  in  the  skin  and 
perspiration.     Deaf-mutism  is  rare  in  myxedema,  frequent  in  cretinism. 

Cretinism. — This  is  a  broad,  general  term  applied  to  a  combination 
of  physical  and  mental  changes  which,  in  the  young,  result  from  loss 


Fig.  87. — Myxedema  showing  failure  of 
ossification  in  epiphyses  of  the  bones  of  the 
hand.     (Siegert.) 


212 


THE  ENDOCRINOPATHIES 


or  diminution  of  the  thyroid  functions.  Such  a  loss  may  occur  sporad- 
ically, sporadic  cretinism,  from  causes  to  be  enumerated,  where  the 
picture  is  analogous  to  that  seen  in  the  adult  from  removal  of  the 
thyroid,  cachexia  thyreopriva  adultorum,  or  it  may  occur  as  a  localized 

or  endemic  degeneration,  affecting  the 
thyroids  of  a  large  number  of  indi- 
viduals, causing  a  hypothyreosis  which 
may  show  a  number  of  tendencies. 
When  these  are  pronounced  they  are 
spoken  of  as  goiter,  goitrous  heart,  and 
endemic  cretinism. 

These  three  fairly  well  separated 
conditions  may  be  discussed  to  ad- 
vantage under  the  head  of  cretinism. 
In  the  first  place  to  call  cretinism  a 
type  of  idiocy  is  misleading.  There 
are  numerous  very  intelligent  cretins. 
Cretinism,  as  here  used,  is  solely  a 
complex  of  different  conditions  due  to 
a  lack  of  development  of  one  or  more 
elements  in  the  body  and  due  to  defect 
or  loss  of  the  thyroid  hormones. 

The  historical  chapters  on  cretinism 
are  full  of  interest.  The  disorders 
were  known  in  early  days.  Pliny  has 
left  indubitable  evidence  of  their  pres- 
ence in  early  Roman  times.  Vogt,  in 
his  admirable  monograph  in  the 
Lewandowsky  Handhuch  der  Neuro- 
logie,  tells  of  Marco  Polo's  descriptions 
of  certain  types  he  had  seen  in  his 
Asiatic  travels.  During  the  past  two 
centuries  the  disorders  here  included 
under  this  term  have  been  observed 
throughout  the  world.  In  certain 
lands  the  disease  is  very  widely  dis- 
tributed, certain  mountainous  districts 
of  Switzerland,  Northern  Italy,  etc. — 
endemic  cretinism  —  while  in  other 
regions  it  occurs  rarely  —  sporadic 
cases.  In  the  United  States  it  is  not 
frequent .  It  has  been  observed  in  Cali- 
fornia, among  our  native  Indians  of  the 
Southwest,  in  ^>rmont,  and  such 
patients  have  been  seen  in  New  York  State  (Adirondacks) .  In  certain 
regions  it  has  been  a  veritable  plague.  Thus  in  Switzerland  between 
the  years  1S75-1S84  seven  per  cent,  of  the  army  recruits  showed 
some  form  of  cretinoid  degeneration.     In  ten  years  2500  men  were 


Fig.  88. — Cretinism.  Woman, 
aged  thirty-four  years;  mentally, 
seven  j'ears  by  Binet-Simon  test; 
height,  49  J  inches;  protuberant 
abdomen,  typical  facies,  supracla- 
vicular pads  of  fat. 


THE  THYREOPATHIES  213 

lost  to  the  Swiss  army  from  this  cause  alone.  Certain  valleys,  espe- 
cially those  of  Berne  and  Wallis,  are  overthickly  populated  with  indi- 
viduals showing  cretinoid  degeneration.  In  the  school  years  1899- 
1904,  of  336,000  children  fit  for  school,  15,000  had  one  or  another 
t>pe  of  cretinism.  Similar  conditions  existed  in  Styria,  Austria,  and 
in  certain  Italian  provinces.  F.  Bircher  has  contributed  an  important 
study  to  the  distribution  of  cretinoid  degeneration. 

So  far  as  the  etiology  is  concerned,  it  seems  certain  that  the  conditions 
are  fundamentally  due  to  a  defect  of  the  thyroid  substance — the  thy- 
roid hormones.  This  is  secondary  to  various  t^-pes  of  inflammation  or 
of  aplasias  of  the  thyroid.  There  are  certain  limitations  which  must  be 
discussed   in  their  respective  paragraphs. 

Sporadic  Cretinism. — Infantile  Myxedema  of  some  Aidhors. — ^The 
clinical  picture  in  an  extreme  case — i.  e.,  fully  developed — in  contrast 
to  the  manv  irregular  or  incomplete  forms — is  that  of  a  normally  born 
child  who  about  the  end  of  the  first  or  the  beginning  of  the  second  year 
begins  to  show  the  characteristic  changes  in  development.  The  little 
patient  falls  behind  in  his  normal  bony  development.  This  is  due  to  a 
defect  in  the  development  of  the  long  bones.  The  epiph}'ses  fail  to  lay 
down  bone  even  after  twenty  to  thirty-five  years,  and  in  twenty-year- 
old  cretins  the  anterior  fontanelle  may  still  remain  open.  There  is  a 
proportionate  loss  in  bony  substance  throughout;  thus  a  characteristic 
dwarfism  results  save  perhaps  in  the  development  of  the  skull,  which 
grows  larger  in  proportion  to  the  rest  of  the  body  giving  rise  to  the 
"full-moon"  face.  Other  cranial  bone  defects,  sphenoid,  nasal,  etc., 
give  rise  to  the  widely  separated  eyes,  the  pug-nose.  The  eyelids  are 
thickened,  the  ears  have  a  waxy  appearance.  The  bony  defect  is  in 
the  nature  of  a  sclerosis  and  the  bony  tissue  is  unusually  hard,  which 
is  the  reverse  of  that  seen  in  rachitis. 

Dental  deficiencies  (diminished  calcification)  go  hand  in  hand  with 
the  bony  defect.  In  severe  athyroid  cretins  the  teeth  do  no  develop 
for  a  number  of  years,  and  the  first  or  milk  teeth  may  persist  far 
beyond  the  normal  period.  Other  defects  appear  in  a  high  palatine 
arch,  which  with  large  adenoids  and  tonsils  and  a  chronic  hypertrophic 
rhinitis  cause  the  child  to  snore  and  sniffle,  often  with  copious  excre- 
tions from  the  nose. 

There  is  usually  a  short,  thick  neck.  A  fairly  constant  finding  is  that 
of  umbilical  hernia.  The  abdomen  is  usually  puffy,  the  navel  sunken 
(frog-belly).  The  skin  is  myxedematous  in  the  young,  but  becomes 
atrophic  in  later  years,  the  supraclavicular  and  facial  swellings  remain- 
ing for  many  years. 

The  facial  habitus  is  characteristic.  The  hair  line  begins  low.  The 
nose  is  sunken,  the  zygomatic  arches  prominent,  the  eyelids  swollen, 
the  face  puffy,  the  tongue  enlarged  and  often  protruding  between  the 
swollen  lijis,  in  the  mild  cases  giving  |^one|the  impression  of  a  child 
whose  whole  countenance  is  puffed  up  with  crying. 

There  is  usually  an  enlargement  of  the  liver.  Respiration  is 
unusually  slow  in  the  severe  athyreoses.     The  genital  organs  show 


214 


THE  ENDOCRINOPATHIES 


marked  changes.  The  labia  are  small,  the  external  not  covering  the 
internal  ones.  The  uterus  and  ovaries  are  usually  small,  and  the 
mammary  glands  are  atrophic  or  hypoplastic.  The  penis  is  apt  to 
be  small,  the  testicles  undescended  and  small.  Genital  and  axillary 
hair  is  absent  or  scanty.  In  boys  the  pubertal  changes  in  the  voice  are 
lacking. 

Blood  changes  are  present.  The  hemoglobin  is  reduced  and  is 
out  of  proportion  to  the  erythrocytes.  The  leukocytes  are  increased, 
the  polymorphic  neutrophiles  being  markedly  diminished  and  the 
lymphocytes  correspondingly  increased.  Large  numbers  of  granular 
cells  are  observed.  These  changes  appear  as  a  result  of  defective 
th^Toid  substance. 


Fig.  89. — Case  of  sporadic  cretinism,  aged 
twenty-one  years.    Before  treatment. 


Fig.  90. — Case  of  sporadic  cretinism. 
After  four  months'  treatment.  (H. 
Sanderson.') 


The  metabolism  of  calcium  is  markedly  diminished  (one-third  of 
its  normal  amount  in  the  studies  made  by  Haugardy  and  Langstein 
and  the  required  calories  are  far  below  that  of  the  normal  child's 
need.  The  assimilation  of  carbohydrates  seems  high,  and  adrenalin 
injections,  without  increased  sugar  intake,  do  not  result  in  a  glycosuria. 
There  is  a  definite  hiypothermia.  In  many  cretins  there  is  a  widening 
of  the  sella  turcica.    Hypoplasia  of  the  thymus  is  also  not  infrequent. 


1  Sanderson:  Sporadic  Cretinism,  with  Report  of  Three  Cases  in  One  Family,  Mich- 
igan State  Medical  Journal,  April,  1906. 


THE   THYREOPATHIES 


215 


Nervous  system  defects  are  present  Avith  the  others  and  apparently 
conditioned  by  the  endocrinous  gland  insufficiencies.  These  show 
at  sensorimotor  levels  in  defects  of  sensory  and  motor-nerve  structures, 
and  at  psychical  levels  in  various  grades  of  stupidity,  mental  weakness 
(moron),  imbecility  or  even  idiocy.  These  words  are  here  used  in 
accordance  with  the  arbitrary  scale  of  the  Binet-Simon  tests. 

Thus  smell  is  at  times  defective;  the  eyesight  poor;  hearing  is 
frequently  disturbed,  and  with  it  speech,  so  that  many  patients  are 
deaf  and*^  diraib.  The  vestibular  function  is  frequently  involved, 
so  that  these  patients  balance  badly,  often  showing  unsteady  gait, 
with  wobbling  of  the  head,  and  nystagmus. 

Some  sporadic  cretins  may  show  little  involvement  of  nervous 
structures. 


Fig.  91. — Case  of  sporadic  cretinism, 
aged  four  years.     Beginning  of  thyroid 
treatment. 


Fig.  92. — Case  of  sporadic  cretinism, 
five  years  old.  Thyroid  treatment  for 
one  year. 


Cretinoid  Degeneration. — IMention  has  been  made  of  the  widespread 
character  of  this  type  of  degeneration  related  to  defective  or  absent 
thyroid  secretions.  The  statistical  study  of  the  conditions,  particularly 
in  Switzerland,  in  France  and  in  Italy  (Bircher)  has  shown  that 
goiter,  goitrous  heart,  endemic  cretinism,  endemic  deaf-mutism,  and 
endemic  feeble-mindedness  are  closely  allied.  The  cretins  are  almost 
all  goitrous,  or  nearly  always  have  goitrous  parents.  Exophthalmic 
goiter  (h}T)erfunction)  is  rare  with  cretinism,  but  very  frequent  with 
goitrous  heart  conditions. 


216  THE  ENDOCRINOPATHIES 

The  causes  of  this  particular  type  of  hypothyroidism  are  not 
definitely  settled  but  there  seems  to  be  a  constant  relation  between  it 
and  certain  elements  in  the  water  supply,  and  goitrous  springs  are 
known.  Just  what  the  noxious  element  may  be  is  still  conjectural,  but 
it  apparently  is  related  to  mineral  constituents  found  in  certain  geo- 
logical formations,  notable  in  the  trias  and  tertiary.  The  disease  is 
absent  in  regions  fed  from  waters  flowing  through  crystalline  formations. 
As  a  result  of  Bircher's  suggestion  of  supplying  a  goitrous  region  in  Rup- 
perwill  from  Jura  water  coming  from  an  adjacent  valley,  the  disease 
disappeared.  Similar  results  followed  in  the  town  of  Asp.  Animals 
may  be  made  goitrous  from  drinking  water  from  certain  springs.  They 
also  develop  goitrous  hearts,  and  are  delayed  in  their  development. 
The  thyroid  shows  degenerative  changes.  The  agent  passes  through  a 
Berkefeld  filter,  but  is  modified  and  made  non-active  by  being  heated 
to  70°  C.  It  does  not  dialyze  and  is  thought  not  to  be  an  organized 
plant  or  animal  substance,  but  to  be  of  colloid  nature.  Bircher  held 
that  the  disease  was  of  an  infectious  nature.  Our  own  inclination  is  to 
ascribe  it  to  the  iodine  ions  held  in  peculiar  colloidal  states. 

Goiter. — Here  Falta  describes  those  enlarged  h3T)erplastic,  non- 
inflammatory thyroid  formations,  with  degenerative  changes  in  the 
struma.  The  hyperplasia  invades  the  parench\Tna  and  the  vessels. 
Notwithstanding  histological  hyperplasia  there  is  physiological  dim- 
inution in  function.  Functionating  parenchyma,  however,  does  not 
exist.  Histologically  one  finds  parench^Tnatous,  vascular,  or  fibrous 
hyperplasias,  with  circumscribed  or  diffuse  goiters.  A  relationship 
between  goiter  and  uterine  myomata  exists,  and  with  a  diminution 
of  one  there  is  a  decrease  of  the  other  at  the  menopause. 

The  etiology  of  some  goiters  is  ascribed  by  McCarrison  to  chronic 
fecal  infection  from  contaminated  water  supplies. 

Goiter  Heart.^ — See  Exophthalmic  Goiter. 

Endemic  Cretinism. — Here  there  is  a  richer  and  much  more  variable 
picture  than  obtains  for  sporadic  cretinism.  Whereas  a  typical 
habitus  is  described,  there  are  many  anomalies  and  variations.  The 
head  is  usually  broad,  but  may  be  small  and  flat  instead  of  large 
and  broad,  at  times  very  large.  The  nose  is  usually  wide-spreading 
and  flat,  the  eyes  wide  apart.  The  neck  is  short  and  thick,  the  features 
swollen,  the  early  impression,  especially  due  to  the  prognathism,  one  of 
moroseness  or  stolidity.  The  bones  are  shortened,  various  anomalies 
as  scoliosis,  ankyloses,  etc.,  being  present.  Great  variation  in  dwarfism 
is  observed.  Certain  cretins  are  under  three  feet  six  inches,  but  full 
cretins  have  been  observed  seven  feet  in  height.  As  a  rule  they  die 
young,  but  Kocher  reports  cretins  seventy  and  even  one  hundred 
years  of  age. 

The  general  coordination  of  these  patients  is  poor.  They  are 
usually  short,  clumsy,  inelastic  with  badly  developed  musculature. 
The  skin  is  loose,  lax,  anemic,  marked  with  folds  and  wrinkles,  giving 
a  peculiar  appearance  of  old  age.  The  lips  are  swollen,  the  tongue 
enlarged,   and   not  infrequently  protruding.     The   breasts   are  flat 


THJ<:  THYREOPATHIBS 


217 


or  badly  developed,  the  abdomen  flat  or  pendulous.  Short,  stumpy 
fingers  and  toes  give  an  ugly  appearance  to  the  extremities  and  con- 
tribute to  clumsiness.  The  entire  acti^'ity  is  apt  to  be  hea^•y  and 
awkward,  although  a  few  athletes  and  acrobats  may  be  found  among 
them. 

The  changes  in  the  bones  have  been  mentioned  in  the  paragraphs 
on  sporadic  cretinism.  Here,  however,  the  variations  are  more 
marked  and  Weygandt's  study  of  Virchow's  material  shows  that  many 
bony  anomalies  exist  among  cretins  not  mentioned  in  Virchow's 
classic  which  has  remained  a  standard  for  writers  for  many  years. 

The  skin  has  a  peculiar  cachexia.  It  is  swollen  and  flabby,  whitish 
or  yellowish,  folded  and  soggy.  The  general  appearance  of  old  age 
is  striking.  The  hair  and  nails  are 
badly  developed,  both  breaking 
easily.  Thick,  underlying,  fatty 
masses  are  unevenly  distributed, 
usually  in  the  neck,  back,  upper 
chest  regions,  occasionally  over  the 
hands.  Variable  states  of  tension 
occur  in  these  fatty  masses;  at 
times  they  are  hard,  again  like 
empty  sacks.  The  mucous  mem- 
branes are  also  pale  and  gray,  often 
folded  but  look  different  from  a 
typical  anemia. 

The  sexual  organ  changes  have 
been  touched  upon  in  the  description 
of  sporadic  cretinism.  They  are 
characteristically  infantile.  Men- 
struation is  scanty,  wanting,  or 
develops  very  late.  Fecundation 
maj'  take  place,  but  the  results 
are  miscarriages,  dead  children 
monsters,  etc.  The  secondary 
sexual  characters  are  all  delayed  in 
their  development.    - 

The  majority  (63  per  cent.,  Ewald)  of  cretins  show  a  swollen  thyroid, 
but  it  is  not  an  overfunctioning  one,  nor  do  they  all  show  athyreosis, 
or  hypoth^Tcosis.  Schonemann  has  reported  the  findings  of  strumous 
changes  in  the  glandular  portion  of  the  hjT^ophysis.  In  112  autopsies 
on  endemic  cretins  he  found  a  normal  hxpophysis  in  only  twenty-seven 
instances.  These  individuals  had  no  goiter.  He  states  that  in  individ- 
uals with  struma  of  the  thyroid  almost  invariably  there  was  an  increase 
in  the  connective  tissue,  also  the  chromaffin  cells,  struma  of  the  vessels, 
hyaline  degeneration  and  swelling  of  the  cell  strands  and  finally  goiters 
with  colloid  formation.  It  is  highly  probable  therefore  that  the  goiter 
poison  works  deleteriously  upon  the  hypophysis  (Falta).  The  para- 
thyroids show  no  changes. 


Fig.  93. — Fetal  udciunia  of  thyroid 
with  enhirgement  of  the  pituitary 
body.     (Josef  son.) 


218 


THE  ENDOCRINOPATHTES 


Most  of  the  internal  organs  show  reduction  in  activity.  Digestion 
is  usually  slow,  constipation  is  marked.  The  metabolism  is  modified 
as  already  indicated.  The  urinary  secretions  are  apt  to  be  diminished, 
and  of  high  specific  gravity.  * 

Mentally  cretins  show  marked  variability.  A  few  are  practically 
normal,  but  most  show  a  characteristic  combination  of  mental  traits, 
which  is  in  marked  contrast  with  many  other  defective  mental  states. 
(See  chapters  on  Feeble-mindedness.) 


Fig.   94. — Two  cases  of  hypothyroidism.     Elsa,  aged  fourteen  years;  Lisa,  aged  sixteen 
years.     (A.  Josefson,  Deut.  Arch.  f.  klin.  Med.,  1914.) 

As  noted,  the  great  majority  suffer  from  impairment  of  the  chief 
sensory  tracts.  Hearing  seems  to  suffer  most.  The  defect  in  hearing 
is  associated  with  speech  defects.  Taste  and  smell  are  also  defective. 
They  take  little  interest  in  their  food  or  drink.  The  feeble-mindedness 
is  accompanied  by  great  slowness  of  all  reactions,  with  marked  retar- 
dation of  motion,  with  apathy,  and  indolence.  This  indolence  is  a 
marked  feature.  Many  cretins  will  lie  in  the  sun  all  day  long,  and  in 
the  hospital  or  other  institution  will  sit  around  and  do  nothing  for 
weeks  or  months.  In  the  milder  grades  there  is  often  great  shyness 
which  makes  them  unapproachable  and  serves  to  make  them  appear 
more  feeble-minded  than  they  really  are.  It  is  with  the  greatest 
difficulty  that  they  can  be  trained  to  the  simplest  of  performances. 
With  many,  in  spite  of  the  marked  general  stolidity  of  their  average 
mood,  they  may  show  great  excitement  and  emotional  outbreaks. 


THE  THYREOPATHIES  219 

The  sense  of  sight  is  frequently  diminished.  It  is  highly  probable 
that  the  receptors  and  conduction  paths  are  less  involved  than  the 
perception  areas  in  this  diminution  in  sensory  intake.  The  hearing 
seems  to  be  affected  both  as  to  its  receptors  and  the  conduction 
paths.  Pain,  touch  and  thermal  sensibilities  are  all  dulled.  INIotilit}^ 
is  extremely  retarded.  The  reflexes  are  active  (60  per  cent.).  The 
field  of  vision  is  reduced  in  many,  although  the  fundus  is  usually 
normal  (Hitschmann) . 

Aberrant  and  abortive  t}^es  are  to  be  expected.  In  the  former 
one  may  find  patients  with  striking  development  of  one  or  more 
features,  in  the  latter  a  very  great  shading  off  to  almost  normal  states, 
i.  e.,  endemic  goiter  with  mild  mental  signs. 

Endemic  Deaf-mutism. — This  combination  is  extremely  frequent 
where  endemic  cretinism  is  present  (29  per  cent.,  Scholz).  It  may 
constitute  one  of  the  aberrant  tj^es  just  mentioned  with  striking 
development  of  single  features,  or  it  may  be  associated  with  all  the 
grades  of  a  complete  cretin  picture.  According  to  the  studies  of 
Kocher,  the  loss  of  hearing  is  due  to  a  bony  defect  which  has  destroyed 
the  possibility  of  normal  cochlear  development.  Bad  hearing  is 
reported  at  32  per  cent,  among  cretins  in  Scholz 's  investigations. 

The  changes  found  in  the  brain  which  may  account  for  the  feeble- 
mindedness have  been  variable.  Meningeal  inflammation  and  mild 
grades  of  hydrocephalus  have  been  found  by  Scholz  and  Zingerle. 
The  brain  is  often  asymmetrical,  small,  or  single  lobes  are  diminished 
in  size.  Often  the  brain's  development  is  arrested  at  an  infantile 
stage,  the  pallium  or  the  ganglia  being  involved  alone  or  together. 
The  cerebellum  is  often  imperfectly  developed,  which  fact  stands  in 
correlation  with  the  marked  incoordination  and  possibly  in  relation 
with   defective  labyrinthine  development. 

The  ear  difficulties  are  numerous.  Peripheral,  conducting  and  central 
mechanisms  are  found  to  be  at  fault,  but  at  all  events  seem  second- 
ary to  the  developmental  anomalies  induced  by  the  action  of  the 
poisonous  substance  on  the  thjToid.  The  speech  defects  usually  go 
hand  in  hand  with  those  of  hearing,  but  this  is  not  universah  The 
cortical  developmental  defect  is  sufficiently  explanatory  for  most  of 
the  cases. 

Mild  and  Mixed  Hypothyroid  States. — Under  the  general  title  of 
abortive  or  mild  hypothjToidism  may  be  grouped  a  very  large  number 
of  individuals,  rarely  considered  sick,  but  who  nevertheless  are  not  up 
to  concert  pitch.  They  show  one  or  more  symptoms  which  are  due  in 
part  solely  to  a  mild  thyroid  deficiency,  or  to  a  polyglandular  syndrome 
with  hypothyroid  predominance.  These  show  themselves  at  various 
ages.  Thus  in  the  nursing  child  the  absence  of  appetite,  constipation, 
obesity,  and  somnolence  have  been  referred  to.  In  the  older,  the 
premature  loss  of  the  hair,  irregularities  of  dentition,  wide  spacing, 
non-emergence,  etc.,  precocious  graying,  somnolence,  constipation. 
Anorexia  as  a  syndrome  is  often  conditioned  by  mild  thyroid  defect. 
Certain  studies  of  families  have  shown  in  the    dift'erent  members 


220 


THE  ENDOCRINOPATHIES 


jjraded  series  of  hypothyroidisms  from  myxedema  to  the  mildest 
involvement,  and  again  in  others  the  gamut  from  the  most  severe 
types  of  myxedema  on  the  one  hand  to  the  most  severe  types  of 
exophthalmic  goiter  on  the  other  may  be  observed. 

The  hypothyroid  iyye  is  usually  smaller  than  he  should  be,  with  a 
tendency  to  obesity.  There  is  a  trend  toward  facial  puffiness,  the 
eyelids,  particularly  in  the  morning,  being  swollen.  The  complexion 
has  a  tendency  to  be  sallow  and  varicosities  are  frequent.  The  hair  is 
apt  to  be  dry,  and  the  hair  line  high.  The  hair  over  the  brows  is 
scanty,  particularly  at  their  outer  edges.  The  moustache  may  be 
scanty.     The  eyeball  is  deep  set,  often  lacking  luster  and  without 


Fig.  95. — Lanugo  hair.    Hypothyroid  disturbance.     (A.  .Tusefson.) 

expression  or  listless.  The  palpebral  fissures  are  narrowed,  frequently 
unequally  so;  the  teeth  irregularly  developed.  Napoleon  was  a  classical 
illustration. 

Gingivitis  is  not  rare.  The  nails  are  brittle  or  frequently  very  hard. 
The  extremities  have  a  tendency  to  be  cold,  the  hands  cold,  bluish, 
slightly  swollen  and  moist.    Chilblains  are  not  infrequent  in  winter. 

This  is  a  general  schematic  summary  of  the  chief  minor  hypothyroid 
signs.  These  symptoms  may  be  found  entire  or  in  groups.  Those  most 
likely  to  be  constant  are  the  defects  in  development,  the  hairy  anomalies 
and  the  coldness  of  the  extremities,  which  latter  is  frequently  associated 
with  a  h;)^othermia,  with  frilosity  and  tendency  to  shivering,  goose- 


THE   THYREOPATHIES 


221 


flesh,  chattering  of  the  teeth,  etc.  A  marked  hyperesthesia  to  cold  is 
often  found  in  these  types  which  leads  readily  to  coryzas,  to  bron- 
chitidis  and  to  neuralgic  pains. 

Hj^Tpothyroid  constipation  is  probably  demonstrated  to  be  a  fact. 
It  is  a  marked  featiu-e  of  the  mjotedematous  and  its  opposite,  diarrhea, 
is  recognized  in  exophthalmic  goiter.  The  explanation  probably  lies 
in  the  lowered  tone  of  the  unstriped  muscle  fibers,  as  an  altered 
autonomic  response  to  the  diminished  thyroid.  In  some  mixed  tj^jes 
where  thyroid  lability  is  marked  (chiefly  secondary  to  marked  emotional 
lability — the  so-called  nervous,  neurotic  or  hysterical  types)  alterna- 


FiG.  96  Fig.  97 

Figs.  96  and  97. — Hypothyroidi.-5m  before  and  after  treatment.     (A.  .Josefson.) 


tions  of  diarrhea  and  constipation  are  frequent.  This  is  related  to  an 
especially  significant  type  of  intestinal  movement  syknokenosis,  i.  e., 
increased  frequency  of  movements,  not  diarrheal,  but  soft  and  frequent. 
From  the  vegetative  side  these  are  related,  particularly  by  Levi  and 
Rothschild,  to  thyroid  instability,  from  the  psychical  side  they  are 
related  to  the  symbols  of  impatience,  haste,  tenseness,  unconscious 
rather  than  conscious.  They  frequently  subside  on  thyroid  therapy 
but  are  equally  amenable  to  rest  and  quiet.  A  more  fundamental 
])sy('h()thera])y  is  called  for  in  others. 

A  number  of  other  syndromes,   much   emphasized    by  Levi  and 


222 


THE  ENDOCRINOPATHIES 


Rothschild  may  be  mentioned.  Personal  experience  has  not  yet  con- 
firmed these  completely.  They  are  hemorrhoids,  mucomembranous 
enteritis  (undoubtedly  thyroidal  secondarily,  but  primarily  emotional) . 
Bradycardia  is  frequent.  Transitory  edemas,  tendency  to  colds,  nasal 
asthma,  respiratory  oppression  are  among  the  minor  signs. 

Certain  skin  syndromes  are  closely  related  to  mild  hypothyroid 
states.  The  skin  is  usually  dry,  rough,  apt  to  be  thick.  It  is  frequently 
scaly,  even  advancing  to  ichthyosis.  The  vegetative  instability  leads 
to  acrocyanosis,  to  transitory  edemas,  at  times  to  eczemas  on  the 


Fig.  98. — Pseudoepiphysis.     An  endocrinous  (hypothyroid)  product.     (Josefson.) 


flexor  surfaces,  to  psoriasis  on  the  extensor  surfaces.  Urticaria;  are 
not  infrequent]th}Tpothyroid  signs  and  are  closely  related  to  gastro- 
intestinal inferiorities  (vegetative)  usually  spoken  of  as  anaphylactic 
reactions  to  certain  (usually)  proteids.  Acne,  herpes,  eczema,  psoriasis, 
scleroderma  should  always  be  studied  with  the  thyroid  in  view. 

The  bony  or  joint  inferiority  which  may  result  from  defective 
development  on  a  hypothyroid  basis  is  frequently  responded  to  by 
chronic  arthritides.  Sometimes  the  specific  overthrowing  lesion  is  an 
infection,  again  metabolic  inferiority  is  registered  by  a  chronic  arthritis. 


THE  THYREOPATHIES  223 

Cases  of  chronic  rheumatoid  arthritis  then  should  be  carefully  analyzed 
with  the  possibility  of  hj'poth^Toid  states  in  view. 

Pseudoepiphvsis,  Josefson  has  shown  to  be  a  h^-pothyroid  stigma. 
(See  Fig.  99.) 

In  the  paragraphs  on  ni\'xedeina  attention  has  been  directed  to  the 
sense-organ  deficits.  These  may  show  in  benign  hypothjToid  states 
as  corneal  opacities,  opacities  in  the  vitreous,  interstitial  keratitis, 
iritis    (so-called   rheumatoid   iritis). 

Pathology  of  Hypothyroid  States. — The  study  of  the  changes  in  the 
thjToid  lies  outside  of  the  purposes  of  this  work.  The  most  important 
defect  states  result  from  infections,  causing  acute  and  chronic  thy- 
roiditis, from  s^-philis  and  from  various  aplasias.  The  changes  in  the 
nervous  system  related  to  or  possibly  due  to  h\'poth}Toid  states  have 
been  extensively  studied.  Kojima's  work  in  relation  to  that  carried 
out  by  Mott  is  noteworthy.^ 

The  nerve-cell  changes  are  most  strikingly  seen  in  certain  cases  of 
mj-xedema  and  in  experimental  h\'pothyroidism.  Chromatolysis,  par- 
ticularly within  the  vegetative  nuclei  groups,  is  marked.  Vagus  and 
glossopharjTigeal  nucleus  chromatolysis  was  extreme  in  certain  of 
Mott's  cases.  In  those  patients  with  marked  mental  s;vTnptoms — 
myxedematous  psychoses  or  in  certain  manic-depressive  states  of  the 
menopause,  possibly  related  to  dysthjToid  activities,  either  due  to  age 
atrophies  or  emotionally  induced  unbalances,  there  are  found  extensive 
cortical  and  bulbar  changes.  Precise  correlations  between  the  destruc- 
tive lesions  found  and  the  symptomatology  are  still  in  too  crude  a  stage 
to  be  didactically  formulated. 

Therapy  for  Hypothyroidism. — ^]\Jany  contradictions  may  be  found  in 
the  literature  concerning  the  use  of  th^Toid  substance  in  various  tj'pes 
of  myxedema  and  cretinoid  degeneration.  This  is  to  be  expected  since 
so  man}'  observers  use  their  diagnostic  terms  so  lightly.  Age  differences 
are  not  recorded — stage  and  intensity  of  the  disease — and  grade  of  defect 
are  overlooked,  and  hence  no  uniform  basis  for  comparison  exists. 

Among  the  best  reported  results  are  those  of  v.  Wagner  who  obtained 
the  following  results:  a  diminution  in  the  myxedematous  swelling  of 
the  skin,  the  genitals  developed  rapidly,  the  tongue  diminished  m  size, 
there  was  loss  of  the  umbilical  hernia,  development  of  new  hair, 
dentition  was  hastened,  closure  of  the  fontanelles  occurred,  and  there 
was  an  increase  in  bony  development.  The  psyche  was  less  hopefully 
modified,  but  there  was  a  diminution  in  the  apathy,  and  slight  increase 
in  the  intellectual  capacity  was  noted. 

Early  therapy  is  naturally  the  main  feature.  According  to  v.  Wagner 
small  doses  of  iodine  in  addition  seem  to  stimulate  the  thyroid 
acti\ities  still  further.  Magnus  Levy,  v.  Eysselt,  and  others  also 
report  excellent  results,  complete  cure  resulting  in  some  patients 
still  in  their  teens. 

A  widespread  state  experiment  was  carried  out  by  v.  Kutscheras  in 

'  Proceedings  Royal  Society  of  Medicine,  Psychiatric  Section,  vol.  viii;  Mott,  ibid., 
Pathological  Section,  February  13,  1917. 


224  THE  ENDOCRINOPATHIES 

Styria  by  treating  1011  cretins.  A  large  number  were  neglected  by  the 
parents,  i.  e.,  treatment  was  not  kept  up.  In  2.4  per  cent,  the  thyroid 
tablets  could  not  be  well  borne.  All  idiots  and  severe  grades  of  deaf- 
ness and  dumbness  were  left  alone.  Of  440  of  the  cases  10.2  per  cent, 
showed  slight  increase  in  bony  development,  4  per  cent,  showed  definite 
change,  85  per  cent,  showed  an  increase  well  above  the  average.  The 
increase  in  bony  gro"v\i:h  was  marked  with  the  younger  individuals, 
but  also  persisted  into  the  third  decade. 

A  careful  revision  of  677  cases  showed  42.8  per  cent,  marked  im- 
provement, 48  per  cent,  some  definite  improvement,  8.6  per  cent,  no 
improvement. 

Scholz's  experiences  with  100  cretins  in  an  institution  were  dis- 
appointing. He  used  as  many  as  eight  tablets  a  day.  Von  Wagner 
claims  the  doses  were  too  extreme,  hence  the  bad  results.  Emaciation, 
weakness,  loss  of  appetite,  vomiting  and  diarrhea,  and  other  symptoms 
of  h;s'perth\Toidism  developed.  Von  Wagner  recommends  the  use  of 
only  one-half  or  one  tablet  (thjToidinum  siccum,  Merck  =  gram  0.1); 
0.4  gram  corresponds  to  the  activity  of  an  entire  gland.  (Burroughs 
Welcome  Co.,  0.1-0..3  gram  =  gr.  iss-v.) 

lodothjTine  has  also  been  utilized.  One  gram  contains  three  milli- 
grammes of  active  substance  representing  the  iodin  content  of  one 
gram  of  fresh  sheep's  th;vToid.  It  would  seem  that  the  iodine  content 
is  not  the  only  factor  in  the  activity  of  the  thyroid  substance  and  it  is 
not  as  yet  definitely  demonstrated  what  the  combination  is  that  is 
effective. 

Surgical  implanting  of  the  thyroid  gland  itself  would  seem  to  be 
the  ideal  therapy,  and  experiments  directed  to  this  end  have  been 
carried  out  since  1889,  when  Bircher  was  one  of  the  first  to  attempt 
it.  The  gland  has  been  implanted  in  different  portions  of  the  body— 
the  neck,  under  the  breast,  in  the  spleen,  even  in  the  bony  substance. 
As  a  rule,  however,  the  implantation  has  not  been  as  successful  as 
was  hoped,  the  gland  itself  undergoing  retrograde  changes.  Further- 
more, it  would  appear  from  the  studies  bj^  Enderlen  and  Borst  that 
th\Toids  from  other  animals  possibly  are  not  the  best  things  to  use,  as 
the  biochemical  composition  of  the  human  and  animal  types  varies 
so  widely  as  to  render  degeneration  of  the  implanted  gland  likely. 
Implantation  of  human  glands  has  not  been  successfully  brought  about 
as  yet,  but  with  the  newer  work  on  organ  transplantation  as  inaugu- 
rated through  the  researches  of  Carrel  and  the  work  of  Hess  and 
Strauss,^  it  would  seem  that  this  technical  difficulty  might  be  overcome 
in  the  very  near  future. 

One  is  compelled,  therefore,  to  resort  in  most  cases  to  the  dried 
or  liquid  preparations  of  the  thyroid  itself  or  to  such  biochemical 
products  as  laboratory  research  has  provided  through  the  utilization 
of  the  glandular  substance  itself  or  that  portion  of  it  which  presents 
its  chief  hormone  activity,  Kendall's  Thyroxin  for  example. 

'  Autotransplantation  and  Homotransplantation  of  the  Thyroid  Gland,  Archives  of 
Internal  Medicine,  1917. 


THE   THYREOPATHIES  225 

The  general  results  of  th^Toid  medication  in  t\'pical  cases  are  fairly 
constant.  Especially  is  it  of  value  in  the  aberrant  and  minor  forms 
of  the  disease  of  which  one  of  the  most  chronic  of  symptoms  is  the 
persistent  anemia.  This  may  be  in  part  overcome  by  the  simultaneous 
use  of  small  doses  of  arsenic,  which  have  been  recommended  by  a 
number  of  investigators.  Alcohol  and  morphin  work  disadvantage- 
ously,  and  should  be  carefully  avoided.  The  use  of  small  doses  of  sodi- 
um bicarbonate  and  bismuth  work  advantageously  in  diarrheal  states. 

Thyroid  medication  for  the  sporadic  cases  varies  somewhat  from 
its  use  in  the  endemic  cases.  In  the  sporadic  cases  of  the  light  or 
mild  t^Tpe  the  action  is  quite  similar  to  that  seen  in  the  endemic  ones, 
but  as  a  rule  sporadic  cases  by  reason  of  their  longer  involvement 
and  the  less  rapid  development  of  the  s\'mptoms,  their  more  hidden 
or  obscure  nature  with  their  great  mixture  of  syndromes  make  them 
less  responsive  to  the  therapy.  Nevertheless,  many  of  them  respond 
very  kindly  to  it,  the  same  dosage  being  utilized. 

In  the  benign  h}-poth\Toid  states  carefully  selected  thyroid  therapy 
has  been  of  excellent  service.  ^Massive  doses  are  at  times  required 
to  bring  about  the  desired  effects;  again  very  minute  dosage  is 
sufficient.  The  use  of  the  endocrinous  glands  at  the  present  time 
seems  to  be  going  through  the  characteristic  cycle  of  all  enthu- 
siasms. Th\Toid  is  now  a  universal  panacea.  Out  of  this  hj^er- 
therapeutic  activity  careful  discriminations  will  come  and  proper 
means  adapted  to  help  the  s\mdromes  which  have  been  here  rather 
hastily  summarized.  Hormone  therapy  has  its  place,  the  limits  of 
which  are  being  outlined  by  a  careful  scrutiny  of  the  vegetative 
reactions  and  their  relations  to  the  endocrinous  hormones. 

Hyperthyrebses :  Exophthalmic  Goiter. — Graves  described  the  con- 
dition in  1835,  Basedow  m  1840.  ]Mobius  in  1886  insisted  on  the 
relation  of  the  disorder  to  changes  in  the  thjToid  gland. 

Exophthalmic  goiter  is  a  disorder  conditioned  by  a  modification  of 
the  activity  of  thyroid  gland  substance  which  in  turn  leads  to  an 
increased  acti^•ity  of  the  vegetative  nervous  system  with  a  series  of 
cardiovascular  signs,  tachycardia,  exophthalmos,  tremor  and  increased 
metabolic  activity.  Under  some  circumstances  the  increase  in  the 
glandular  activity  is  primary,  the  vegetative  symptomatology,  second- 
ary, under  others  the  reverse.  In  every  case  the  cause  for  the  hj^jer- 
activity  should  be  ascertained  before  therapy  is  inaugurated.  To 
operate  for  an  acutely  disturbed  thjToid  due  to  a  severe  mental  shock 
is  hasty  and  mostly  meddlesome  therapy.  To  attempt  a  psychoanalysis 
for  a  septic  thyroiditis  is  equally  farcical.  As  has  been  pointed  out 
certain  forms  of  hyperthyroid  functioning  are  healthy  compensations 
for  defective  adrenal  capacity.  ]Many  of  the  h\'i)erthyroid  states 
observed  among  soldiers  were  due  to  this  interrelationship.  A  lessening 
of  the  adrenal  load  (from  exertion,  fear  (fight  and  flight),  infections), 
with  pituitary  therapy,  1  to  3  grains  daily,  often  very  shortly  restores 
the  impaired  balance  and  the  thjToid  swelling  and  thjToid  s\Tnptoms 
subside. 


226 


THE  ENDOCRINOPATHIES 


The  disturbance  is  widespread  and  presents  many  variations.  Indeed 
incomplete  and  irregular  forms  are  among  the  commonest  of  the  mani- 
festations of  hyperthjToidism.  Women  much  more  often  than  men 
show  this  particular  type  of  disturbance.  In  Sattler's  great  monograph 
3200  of  3800  cases  reported  were  females.    Hereditary  types  are  known. 

Symptoms. — The  chief  symptoms  are  found  in  the  thyroid,  eye,  heart 
and  bloodvessels,  skin,  and  muscles.  Psychical,  gastro-intestinal, 
respiratory,  genital,  and  metabolic  changes  occur  as  well. 

It  must  be  emphasized  that  the  symptom  groups  which  are  here 
described  apply  to  the  more  evident  h^-perthyroid  states.  It  should 
constantly  be  borne  in  mind  that  a  great  many  persons,  women 
particularly,  as  a  result  of  emotional  disturbance,  often  unconscious, 
suffer  from  mild  toxic  thyroid  states.  The  literature  is  enormous. 
Sattler's  monograph  is  the  most  complete  to  date. 


Fig.  99. — Exophthalmic  goiter,  show- 
ing marked  exophthalmos  and  enlarged 
thyroid.  (Courtesy  of  Dr.  George  W. 
Crile.) 


Fig.  100. — Same  patient  tour  months 
after  operation  (extirpation.)  Greatly 
diminished  exophthalmos  and  change  of 
facial  expression.  (Courtesy  of  Dr. 
George  W.  Crile.) 


An  enlarged  thjToid  is  fairly  constant,  although  occasionally  absent. 
It  is  soft  and  elastic,  rich  in  new  bloodvessels,  pulsates,  and  varies  in 
volume  (often  very  rapidly) .  Its  ^^ariations  in  volume  are  fairly  com- 
mensurate with  the  intensity  of  the  symptoms.  Auscultation  of  the 
enlarged  thyroid  often  gives  a  marked  bruit. 

The  heart  action  is  rapid  (tachycardia),  and  the  pulse  is  very 
^'ariable,  reacting  excessively,  particularly  to  psychical  influences.  The 
heart  sounds  are  increased  in  force,  the  beating  being  felt  in  the  neck, 
and  the  whole  chest  wall  is  at  times  moved  by  the   cardiac  tumult. 


THE  THYREOPATHIES  227 

The  blood-pressure  is  rarely  raised  and  the  radial  and  other  vessels 
show  marked  hypotonus  with  reddening  of  the  face,  ears,  and  finger 
extremities. 

The  eye  sjniptoms  consist  of  a  marked  and  variable  protrusion  of 
the  ej^eball,  with  widened  orbital  fissure,  soroetimes  greater  on  one 
side  than  the  other,  and  felt  as  a  disagreeable  pressure  and  tension  by 
the  patient.  The  eyelids  are  at  times  swollen,  and  the  upper,  and 
possibly  the  lower  lids  largely  retracted  (DalrjTuple,  Stellwag) ^inde- 
pendently of  and  often  preceding  the  protrusion  of  the  eyeballs.  The 
upper  lid  also  does  not  move  synchronously  with  the  lowering  or 
the  raising  of  the  eyeball  (von  Graefe's  sign),  following  more  slowly 
or  receding  more  rapidly  (spasticity)  than  the  moving  eyeball,  in  the 
presence  or  absence  of  protrusion.  Both  signs  may  be  unequally 
present.  The  relative  infrequency  of  winking  (Stellwag)  is  a  frequent 
sign.  Eppinger  shows  in  tabular  form  the  relative  frequency  of  these 
ocular  phenomena. 

Wilbrand  and 

Sanger.  Sattler.  Eppinger. 

Symptom.  Per  cent.  Per  cent.  Per  cent. 

Protrusion,  wide,  V.  Graefe 23.0  37.3  35.6 

Protrusion,  V.  Graefe 36.0  19.8  16.4 

Protrusion 10.4  17.6  19.2 

No  eye  signs 15.3  13.3  4.8 

Wide,  protrusion 2.2  6.7 

V.  Graefe,  wide 2.6  5.4  9.6 

Wide 5.1  1.1  3.8 

V.  Graefe 7.6  3.3  3.8 

Niunber  of  cases 39.0  91.0  104.0 

Lowi's  sign  (dilatation  from  adrenalin)  is  frequent.  Occasion- 
ally mydriasis  is  present,  less  frequently  miosis.  Irregular  or  stiff 
pupils  may  be  observed  as  well  as  loss  of  the  accommodation  reflexes. 
Optic  nerve  atrophy  is  infrequent,  Increased  tear  secretion  is  often 
observed  early,  dryness  late  in  the  disease.  Mechanical  complications 
— pus,  ulcerations — are  met  with. 

Insufficiency  of  convergence,  without  double  vision  (Mobius's  sign), 
is  frequent  and  is  independent  of  protrusion. 

Cardiovascular  symptoms  are  among  the  most  constant  and  early 
signs  of  exophthalmic  goiter,  and  are  due  to  the  actioa  of  the  thyroid 
secretion.  Tachycardia  (occasionally  paroxysmal)  is  the  most  promi- 
nent single  sign.  The  pulse  may  beat  from  100  to  160  times  a  minute 
— even  200  has  been  recorded.  Undoubted  cases  may  show  no  rise 
above  100,  and  great  variability  is  the  rule,  especially  in  response  to 
psychical  stimuli.  During  sleep,  and  also  on  lying  down,  the  pulse 
frequency  slows  down. 

Angina-like  attacks,  with  hyperalgesia  in  the  left  ulnaris  region 
are  not  infrequent,  and  in  most  patients  the  feeling  of  distress  and 
anxiety  over  an  increased  sense  of  heart  oppression  (apart  from  the 
tachycardia)  is  one  of  the  most  annoying  symptoms.  Cardiac  dilata- 
tion, with  later  hypertrophy,  without  valvular  defect,  may  or  may 
not  (50  per  cent.)  accompany  the  disease  and  disappear  at  its  termina- 


228  THE  ENDOCRINOPATHIES 

tion.  On  auscultation  the  first  sound  is  usually  accentuated,  and 
systolic  murmurs  at  the  base  are  frequent.  Valvular  insufficiencies 
occur  under  special  circumstances,  and  are  often  of  serious  moment  in 
operative  cases. 

Strong  pulsation  of  the  carotids  is  frequent,  and  though  the  large 
vessels  are  often  prominent,  and  apparently  arteriosclerotic,  the 
walls  are  usually  soft  and  yielding.  Vasomotor  instability  is  frequent. 
Marked  reddening  alternates  with  paleness.  Irregular  erythemata 
also  are  not  infrequent  and  many  patients  complain  of  surface  heat, 
seek  cold  places  and  light  clothing,  even  in  winter,  and  yet  show  no 
temperature  anomalies.  Dermographism  is  also  a  frequent  vasomotor 
phenomenon,  and  epistaxis  is  not  infrequent.  Rarer  lu-ticarise,  irregular 
circumscribed  edemas,  pruritus,  etc.,  are  to  be  expected. 

Skill  s\Tnptoms  are  frec^uent.  Increased  perspiration  is  not  rare 
and  the  skin  is  always  moist  with  some  patients,  possibly  only  on 
one  side,  or  in  isolated  (head)  areas  or  smaller  spots. 

The  electrical  resistance  of  the  skin  (Veraguth-Vigoureux)  is  dimin- 
ished as  a  result  of  this  increased  secretory  activity.  Goose-flesh 
develops  readily  with  these  patients,  and  changing  pigmentar}'-  anom- 
alies, chiefly  chloasma  spots,  are  present  on  the  skin,  not  on  mucous 
membranes,  more  especially  on  the  eyelids,  neck,  nipples,  armpits, 
and  genitals.  In  many  patients  (23  per  cent.,  Sattler),  there  is  a 
tendency  for  the  hair  to  fall  out  (sometimes  unilaterally)  with  the 
development  of  the  disease,  and  usually  there  is  renewed  growth 
of  the  hair  with  improvement. 

The  nails  frequently  show  deformities  and  dystrophies  (marbling). 

Tremor  is  an  early  and  frequent  symptom,  and  may  involve  the 
entire  body.  It  is  usually  fine,  varies  from  seven  to  forty  vibra- 
tions per  second,  and  occasionally  is  intermingled  with  wider,  irregu- 
lar choreic-like  movements.  Psychical  rather  than  physical  stimuli 
increase  the  tremor  greatly;  lying  down  tends  to  diminish  it.  It 
is  more  apparent  in  the  upper  than  in  the  lower  extremities  and 
is  marked  in  the  eyelids  (Rosenbach),  in  the  tongue,  and  when  in  the 
vocal  cords  produces  a  peculiar  staccato  breathing  (]\Iinor.) 

Digestive  disturbances  are  frequent.  Dry  mouth  may  alternate 
with  excessive  salivation.  There  is  a  marked  tendency  to  chronic 
alvine  discharges  (30  per  cent.)  and  to  vomiting  without  anorexia 
(15  per  cent.).  Both  occur  in  paroxysms,  somewhat  resembling 
tabetic  crises.  Hour-glass  contraction  of  the  stomach  may  be  demon- 
strated by  the  .r-rays.  Both  the  vomiting  and  diarrhea  are  obstinate, 
are  accompanied  by  mucus  or  colloid,  at  times  bloody  material  and 
the  movements  may  occur  as  often  as  four  or  five  times  a  day.  Fatty 
stools  without  diarrhea  may  occur.  With  both  diarrhea  and  vomiting 
the  patients  are  in  grave  danger.  Obstipation  of  spastic  type  ma}^  also 
occur.  In  many  patients  there  are  enlarged  hmphatics,  tonsils, 
tongue  follicles,  thAinus,  and  lymphatics  of  the  intestines. 

liesplratory  sNinptoms,  dyspneic  in  character  are  usual.  Normal 
breathing  is  frequently  irregular  in  depth  and  rhythm,  and  seems 


THE  THYREOPATHIES 


229 


strained.  The  swollen  gland  may  cause  relative  stenosis.  Asthmatic 
tendencies  are  present,  and  the  general  sense  of  air  hunger  is  striking 
with   nervous   pseudohysterical   coughing. 

Menstrual  irregularities  are  common.  The  flow  is  usually  small 
in  amount  and  infrequent  in  occmrence,  with  occasionally  the  direct 
reverse  condition.  Thinning  of  the  breasts,  and  other  atrophies 
(testicles)  have  been  recorded,  and  seem  to  be  coordinated  with 
thjTQus  anomalies. 

Metabolic  anomalies  are  characteristic.  The  patients  become 
markedly  emaciated  and  get  very  weak.  This  is  related  to  a  definite 
nitrogen  loss,  and  also  to  a  marked  overoxidation  of  carbohydrates 
and  fats  (see  fatty  stools).    This  sudden  loss  of  flesh  and  strength 


Fig  .   101 . — Exophthalmic  goiter. 
(Hammond.) 


Fig.   102. — Exophthalmic  goiter. 
(Hammond.) 


may  come  on  in  attacks,  and  then  a  distinct  improvement  takes  place. 
Eppinger  speaks  of  these  patients  as  indi\iduals  who,  not  doing 
any  work,  need  the  carbohydrates  of  a  hard-working  individual.  In 
mild  cases  an  increase  in  carbohydrates  keeps  the  patient  at  a  normal 
weight.  The  increased  oxidation  also  shows  in  a  mild  hjTDerthermia. 
Alimentary  glycosuria  is  frequent,  and  gradually  disappears  on 
recovery;  hjTJerglycemia,  0.1  per  cent,  and  over,  is  a  common  accom- 
paniment, and  not  infrequently  the  blood-sugar  findings  may  be  utilized 
as  a  test  for  the  severity  of  the  disorder.  True  diabetes  is  an  infrequent 
complication.  Polyuria  is  frequent  (13  per  cent.,  Sattler),  less  so 
albuminuria  (11  per  cent.,  Sattler.) 
The  blood  shows  anemic  changes  frequently  at  the    \ery  outset. 


230  THE  ENDOCRINOPATHIES 

The  number  of  red  cells  is  not  markedly  lowered,  as  a  rule,  save  in 
those  patients  with  marked  cachexia.  The  leukocyte  count  is  usually 
low;  the  percentage  of  cells  is  altered.  L\7nphocytosis  is  marked 
(60  per  cent.).  The  polynuclear  neutrophiles  are  markedly  diminished. 
The  eosinophile  cells  are  increased  (8  to  20  per  cent.).  The  large  mono- 
nuclears are  normal.  Thj^roidectomy  changes  the  entire  blood  picture 
more  toward  normal,  as  does  also  ligation  of  the  thyroid  arteries 
and  according  to  Kocher  the  blood  picture  is  a  valuable  prognostic 
index.  The  coagulation  time  is  increased.  In  the  young,  hj^per- 
thweosis  leads  to  increased  growth  of  the  bones,  and  young  exophthal- 
mic patients  are  apt  to  be  very  large  and  very  fat. 

The  mental  symptomatology  of  hjTDerthyreosis  is  of  great  importance, 
since  from  the  studies  of  Parhon  and  others  it  seems  possible  that  like 
others  of  the  symptoms  the  mental  signs  may  develop  almost  exclu- 
sively. In  many  cases  the  psychical  signs  are  mild.  The  tendency  is  to 
both  psychomotor  and  emotional  irritability.  Moodiness  and  sudden 
changes  are  frequent.  In  marked  cases  distinctly  manic  phases  may 
develop;  again  acute  and  deep  depressions  (often  suicidal)  take  their 
place.  Thus  the  picture  approaches  very  closely  at  times  to  the  type 
of  Kraepelin's  mixed  manic-depressives,  or  the  more  tj^^ical  circular 
forms  of  this  psychosis.  Toxic  epiphenomena  may  take  place  with 
ideas  of  reference,  of  persecution,  even  hallucinations,  principally  of 
sight.   The  general  picture  of  an  acute  delirium  is  a  grave  sign. 

The  analysis  of  the  psychical  pictures  in  exophthalmic  goiter  is  far 
from  complete.  Sattler  advocates  a  catholic  attitude,  saying  there 
is  no  one  tj^ical  h3T)erth\Toid  psychosis.  The  present  tendency  is 
to  ally  the  mental  phenomena  of  the  hj^Derthyreoses  with  the  manic- 
depressive  group,  and  to  separate  certain  manic-depressive  cases  as 
largely  conditioned  by  hyperthyroid  activity  on  a  polyglandular  basis. 

General  Etiology  and  Pathology.^ — General  considerations  relative  to 
the  athyreoses,  dysthyreoses,  and  h^'perthyreoses  as  seen  in  the  clinical 
pictures  of  congenital  and  acquired  hj^pofunctioning  as  in  cretinism 
and  myxedema,  and  in  M'perfunctioning  as  seen  in  exophthalmic 
goiter  point  to  paralytic  and  irritative  phenomena  of  the  sympathetic 
and  parasympathetic  (autonomic)  nervous  systems. 

The  blood  changes  in  exophthalmic  goiter  and  in  myxedema  are 
similar,  i.  e.,  there  is  a  relative  lymphocytosis  with  diminution  of  the 
neutrophile  leukocytes.  In  exophthalmic  goiter  the  coagulation  time 
in  increased,  in  myxedema  diminished.  In  exophthalmic  goiter  the 
sympathetic  irritation  explains  the  exophthalmos,  tachycardia,  loss  of 
weight,  and  the  alimentary  glycosuria.  Autonomic  irritability  causes 
the  von  Graefe,  the  lymphocytosis,  the  diarrhea,  the  increased  secre- 
tions. The  influence  of  the  thyroids  on  the  carbohydrate  metabolism, 
as  seen  in  the  rapid  emaciation  and  alimentary  glycosuria,  possibly 
acts  through  the  pancreatic  retardation  or  through  a  relative  increase 
in  adrenalin  action.  That  the  thymus  is  involved  in  the  blood  picture 
formation  seems  certain. 

Thus  one  comes  to  a  combined  neurochemical  theory  in  that  exoph- 


THE  THYREOFATHIES  23 1 

thalmic  goiter  is  dependent  upon  h\'peractivity  of  the  thyroid  secre- 
tions, which  increased  secretions  act  through  the  visceral  or  vegetative 
nervous  system.  Both  autonomic  and  sympathetic  systems  are  thus 
in  a  state  of  hyperexcitabihty — a  condition  the  anatomical  foundations 
for  which  are  found  in  a  certain  type  of  individual  termed  vagotonic  by 
Eppinger. 

The  detoxication  hypothesis  of  Blum,  Ostwald,  Kocher,  Klose  and 
others,  in  which  a  dysthyreosis  is  assumed  with  a  t\"pe  of  iodine 
poisoning  from  insufficient  detoxication  is  ingenious,  but  not  yet 
satisfactory. 

The .  full  etiology  is  still  very  dark.  Many  apparently  healthj^ 
individuals  suddenly  develop  the  disorder  following  a  shock.  This 
shock  frequently  involves  the  complex  of  the  fear  of  death  or  the  loss 
of  money.  There  is  a  characteristic  fear  reaction.  Minute  analyses 
from  the  psychoanalytic  school  are  not  yet  available  to  permit  generali- 
zation, but  the  psychical  import  of  shock  is  undoubted.  In  certain 
personal  experiences  chronically  increased  thyroid  activity  and  certain 
definite  unconscious  trends  have  been  uniformly  associated. 

Infectious  and  toxic  tj'pes  are  also  recognized — acute  thyroiditis,  etc., 
and  also  a  form  of  iodine  intoxication  in  individuals  who  have  taken 
potassium  or  other  iodides. 

The  changes  in  the  gland  itself  are  of  little  moment  for  the  inter- 
pretation of  the  disorders.  A  great  variety  of  variations  from  the 
average  have  been  described,  chiefly  of  hyperplastic  tj'pe.  Apparently 
perfectly  normal  glands  are  at  times  associated  with  severe  t}T)es 
of  the  disease.  The  gland  is  usually  enlarged,  elastic,  the  vessels 
dilated,  and  new  proliferating  bloodvessels  are  found.  Kocher  has 
described  the  goitrous  type  as  parenchymatous  hyperplastic  struma, 
poor  in  colloid  and  in  iodine;  other  changes  are  largely  due  to  compli- 
cations. 

Forms  and  Diagnosis. — If  the  classical  triad,  exophthalmos,  tachy- 
cardia, and  goiter  be  present,  there  is  little  question  as  to  diagnosis, 
but  still  all  may  be  absent  and  yet  the  patient  be  suffering  from  severe 
hjTDerthyroidism.  Hence  great  variability  may  be  expected.  Eppinger 
and  Hess  distinguish  two  chief  groups  according  to  the  predominance 
of  the  sympathetic  or  autonomic  irritative  phenomena.  These  deserve 
more  detailed  study.  The  autonomic  group,  in  particular,  is  often 
overlooked.  These  show  von  Graefe's  sign,  diarrhea,  lymphocytosis 
and  increased  perspiration  and  marked  anxiety.  They  are  not  infre- 
quently taken  for  cases  of  anxiety  neurosis  or  other  neurasthenoid 
hybrids.  Severe  gastric  or  enteric  crises  have  led  to  a  mistaken 
diagnosis  of  tabes.  Undeveloped  forms  may  be  readily  overlooked, 
especially  when  the  more  classical  triad  just  noted  is  not  present. 

Particular  attention  should  be  focuss.ed  on  the  thyroid  itself.  Its 
rich  and  increased  vascularity  tends  to  give  it  a  peculiar  consistency, 
even  when  not  markedly  enlarged,  which  is  very  characteristic.  Kocher 
has  compared  it  to  the  general  fulness  of  the  breast  of  a  pregnant  or 
nursing  woman. 


232  THE  ENDOCRINOPATHIES 

In  a  very  large  number  of  patients,  especially  those  showing  the 
parasympathetic  irritation  (vagotonic)  signs  discussed,  hypertrophy 
of  other  lymphatics  is  to  be  observed.  These  are  chiefly  to  be  sought 
in  the  thymus,  tonsils,  tongue  and  rectal  lymphatics.  There  is  a 
tendency  to  elongated  extremities,  scanty  beards  in  men  and*l)adly 
developed  genitals  in  women.  Marked  lymphocytosis  is  also  present. 
This  relative  lymphatism  possibly  plays  a  very  important  compensa- 
tory role  in  the  disease. 

The  patients  with  marked  psychical  signs  are  apt  to  show  both  auto- 
nomic and  sympathetic  symptoms.  Certain  patients  show  only  cardio- 
vascular signs.  Such  are  those  described  as  goitrous  heart.  They 
show  tachycardia,  dilated  heart,  some  respiratory  arrhythmia.  The 
eyes  are  often  shiny,  pupils  dilated,  and  striking  even  if  not  protruded. 
Dermographia  is  frequent  and  dizziness  is  often  complained  of.  Other 
closely  related  forms  suffer  from  dyspnea  and  bronchial  catarrh,  bleed- 
ing from  the  nose  and  congestion  of  the  upper  air  passages.  Neurotic 
goitrous  heart  from  pressure  is  another  special  type  often  overlooked. 
There  is  also  unilateral  mydriasis,  at  times  tachycardia,  and  the  eye 
on  the  pressure  side  protrudes  and  recedes.  Rarer  cases  are  disguised 
under  mild  diabetics,  and  F.  Miiller  had  described  a  group  of  pseudo- 
sclerosis cases  of  hyperthyroidism. 

The  iodine  toxic  cases  form  another  group.  Running  from  the 
nose,  bronchial  catarrh,  salivation,  stomachic  distress,  nausea,  diarrhea, 
sleeplessness,  headache,  and  skin  eruptions  are  the  more  frequently 
found  symptoms  which  may  develop  with  but  small  doses  of  iodides. 

That  the  blood  of  patients  with  exophthalmic  goiter  contains  an 
increased  adrenalin  content  which  in  some  cases  may  be  experimentally 
demonstrated  in  animals,  is  a  point  of  hypothetical  diagnostic  value. 
It  also  points  to  the  possible  relation  between  this  disorder  and  uncon- 
scious "  fight  or  flight." 

Occurrence  and  Course. — The  disease  is  comparatively  rare.  The 
proportion  of  men  to  women  is  1  to  6  on  the  average.  Between  fifteen 
and  thirty  are  the  most  frequent  years  of  incidence. 

The  course  is  usually  chronic  wdth  ups  and  downs  and  many  varia- 
tions. Emotional  shocks,  behind  which  simple  term  there  may  lie  the 
profoundest  and  most  tragic  events  of  life,  almost  invariably  bring  on 
an  attack  or  increase  an  existing  one.  Acute  infections  frequently 
bring  on  exacerbations,  while  pregnancy  frequently  acts  advantage- 
ously. The  length  of  time  that  the  disease  persists  is  extremely 
variable — from  three  months  to  thirty  years.  The  prognosis  also 
varies  with  the  severity  of  the  hyperthyroidism.  With  healthy 
individuals  the  prognosis  is  relatively  good,  with  distinctly  nervous 
(especially  vagotonic)  individuals  it  is  less  hopeful. 

Lymphocytosis,  wdth  normal  numbers  of  white  cells,  is  a  better 
prognostic  sign  than  lymphocytosis  with  leukopenia. 

Treatment. — This  may  be  surgical,  by  internal  remedies  or  by  psycho- 
therapy. Surgical  treatment  is  the  most  radical  and  should  be  the 
last  to  be  considered.    The  statistics  of  various  operators  have  shown 


THE  THYREOPATHIES 


233 


improvement  in  from  6  to  76  per  cent.,  death  in  from  2  to  22  per  cent.^ 
Kocher  has  reported  76  per  cent,  good  results.  These  figures  are  prob- 
ably high,  if  ultimate  results  are  meant.  The  operation  of  choice  is  the 
successive  elimination  of  tliAToid  substance,  with  minimal  handling 
of  the  gland.  This  is  advocated  particularly  by  Kocher,  whose  lethal 
results  have  been  from  3  to  7  per  cent.  Death  frequently  is  preceded  by 
narcosis,  with  a  very  characteristic  symptom-complex.  The  face  gets 
red,  the  whole  body  becomes  tremulous  and  breaks  out  in  perspiration, 
diarrhea  supervenes,  the  temperature  rises  and  the  heart  action  becomes 
excessively  rapid,  and  death  with  cyanosis  and  dyspnea  takes  place. 
Status  th^inoh-mphaticus  is  possibly  responsible.  Inferior  adrenals 
are  also  to  be  considered. 


Fig.   103. — Before  treatment. 


Fig.   104.— After    five    wee 
with  great  do^e.- 


ri-itiuent 


The  operations  on  the  cervical  sympathetic  are  not  to  be  recom- 
mended. They  help  the  eye  sjTnptoms  possibly,  but  the  disease  is 
not  one  of  the  cervical  sympathetics  alone. 

Internal  therapy  is  still  unsatisfactory.  Rest  in  bed  is  primary 
and  essential.  Any  remedy  increasing  the  th\Toid  secretion  is  bad, 
hence  thyoids  and  iodine  are  to  be  avoided.  Thymus  has  been  tried 
with  best  results  in  the  sympathetic  tj'pes.  The  fresh  gland  is  given 
by  mouth.  Heliotherapies,  a'-rays,  violet  rays,  as  at  present  developed 
are  justified  in  a  limited  number  of  carefully  chosen  cases.  The 
chronic  infectious,  hyperplastic  goiters  do  best  with  light  therapy. 

Pharmacological  agents  which  act  to  diminish  the  thyroid  secretion 
have  been  used.    Chief  of  these  is  belladonna.     It  is  the  most  reliable 


*  Eppinger,  loc.  cit.,  p.  70. 

2  A.  Josefson:  Hygiea,  1915,  p.  517. 


234  THE  ENDOCRlNOPATHIES 

of  the  internal  remedies  and  can  be  given  in  fairly  large  doses.  Adren- 
alin in  solution  by  rectum  is  of  service  at  times.  Vagotonic  cases 
react  best  to  its  influence,  the  tachycardia  and  diarrhea  bemg  well 
influenced  by  it.  The  digitalis  group  of  glucosides  are  not  to  be  recom- 
mended, neither  is  iron  of  any  service.  Arsenic  and  bromides  may  be  of 
passing  service,  especially  the  latter,  in  aiding  sleep.  Certain  cases 
react  very  advantageously  to  extremely  minute  doses  of  thyroid. 
(Psychotherapy?)  Pituitary  is  useful  in  thyroid  overcompensations 
for  adrenal  deficiency.  A  combined  thymus  and  pituitary  therapy 
helps  a  great  many  neurotic  unsatisfied  women  (sex  repressions  usually 
with  an  unconscious  masturbatory  basis)  whose  dissatisfactions  and 
childish  impatience  with  reality  keeps  them  in  a  constant  state 
of  adrenal  exhaustion  in  which  an  attempted  thyroid  compensation 
causes  chronic  hyperthyroid  signs  of  a  minor  grade. 

Psychotherapy  is  above  all  of  great  value,  especially  as  applied 
toward  "an  education  of  the  patient  concerning  his  fears,  in  the  sense  of 
Dubois.  This  is  a  combined  rest  and  reeducation  therapy  which  Dubois 
claims  has  been  successful  in  the  vast  majority  of  cases.  Psycho- 
analj^sis  is  of  the  most  signal  service  in  a  large  group  of  cases,  especially 
in  readjusting  the  patient  to  his  unconscious  wishes,  revolts  and  rebel- 
lions. It  is  particularly  valuable  as  a  follow-up  of  a  surgical  operation 
which  has  been  performed  to  save  life. 

Serum  treatments  aiming  to  exert  a  lytic  action  upon  the  secretory 
cells  of  the  thyroid  have  been  devised.  As  they  do  nothing  but  destroy 
structure  they  are  not  particularly  useful. 

PARATHYROID  SYNDROMES.^ 

The  parathjToid  glands  arise  in  man  from  epithelial  outgrowi;hs 
on  the  third  and  fourth  branchial  clefts.  That  from  the  third  cleft 
usually  lies  free  from  the  thyroid  in  most  animals  save  in  man.  Acces- 
sory parathyroids  are  present  in  difi^erent  neck  structures  and  para- 
thjToid  tissue  is  frequently  found  in  the  thymus  gland.  In  man  the 
chief  parathyroid  masses  are  imbedded  in  and  blended  with  the  thyroid 
tissue,  although  distinct  from  it.  In  general  the  removal  of  two  or 
more  of  the  parathyroids  gives  rise  to  the  disorder  termed  tetany. 

Tetany. — Corvisart  first  used  this  term  "tetanic"  in  1852  Frankl- 
Hochwart,  in  1887,  clarified  the  conception,  and  since  his  striking 
description  the  term  tetany  has  had  universal  recognition. 

As  early  as  1815  J.  Clarke  described  the  symptoms,  Cheyne  in  1819 
and  HaU  m  1841  also  gave  sufficiently  detailed  histories  of  convulsive 
states  to  permit  the  recognition  of  tetany. 

Tetany  is  most  satisfactorily  interpreted  as  a  disorder  of  the  vegeta- 
tive control  of  the  calcium  metabolism  of  the  body,  alterations  of  which 
modify  the  electrical  permeability  of  the  neuron  membrane  of  the 
synapse,  causing  marked  hyperfunctioning  of  the  sensory  and  motor 

1  Richardson:  Thyroid  and  Parathyroid.  Pepere:  Paratiroidee,  and  papers  by  Dustin 
Uhlenhuth  and  others  on  the  reciprocal  relations  of  thymus  and  thyroid  and  parathyroid. 
See  Noel  Paton  and  L.  Findlay:  Quart.  Jour.  Exp.  Physiol.,  10,  1916-1917  for  exten- 
sive study. 


PARATHYROID  SYNDROMES  .         235 

spinal  mechanisms.  As  the  parathjToids  are  possibl}^  the  chief  regu- 
lators of  the  calcium  ions  in  the  bodily  fluids  tetany  is  preeminently 
a  result  of  parathyroid  h\pofunctioning.^ 

Symptoms. — Tetany  has,  as  its  main  symptoms,  tonic,  intermittent, 
bilateral,  often  painful  cramps,  which,  without,  for  the  most  part,  any 
loss  of  consciousness,  involve  the  muscles  of  the  upper  extremities, 
particularly  the  hand,  which  is  held  in  the  obstetrical  position.  The 
muscles  of  the  lower  extremities  may  also  be  involved,  those  of  the 
larynx,  of  the  face,  and  of  the  jaw,  seldom  those  of  the  chest,  abdomen, 
neck,  diaphragm,  or  tongue.  In  rarer  cases  the  eyeball  muscles  are 
implicated,  as  is  also  the  bladder.  In  the  sensory  sphere  paresthesise 
and  pains  are  present,  while  hj-peresthesise  occur  now  and  then.  Pres- 
sure upon  the  brachial  plexus  may  give  rise  to  an  attack  (Trousseau) ; 
hj'perexcitability  to  electrical  currents  is  present  (Erb);  mechanical 
hyperexcitability  of  the  muscles  and  motor  nerves  is  observed 
(Chvostek),  while  the  sensory  hj^peractivity  to  mechanical  and  elec- 
trical stimuli  is  also  present  (Hoffmann) .  The  psyche  is  rarely  unin- 
volved,  and  following  operative  removal  there  has  developed  extreme 
anxiety  with  the  sense  of  impending  dissolution. 

In  chronic  and  repeating  forms  secretory  and  trophic  disturbances 
occur,  such  as  increased  perspiration,  reddening  of  the  skin,  swellings 
of  the  joints,  mild  edema,  falling  out  of  the  hair  (alopecia)  and  nails, 
discoloration  of  the  skin,  urticaria,  and  herpes.  Dyspnea  may  inter- 
vene; poljoiria  and  glycosuria  are  rare  accompanying  symptoms. 
Incomplete  forms  have  been  designated  "tetanoid"  by  Frankl- 
Hochwart. 

For  didactic  purposes  Frankl-Hochwart  divides  tetany  into  simple 
and  acute  forms  and  chronic  recurring  forms.  A  further  division 
of  forms  occurring  in  children  and  in  adults  is  made.  Tetany  of  the 
adult  he  groups  into  seven  classes:  (1)  Tetany  idiopathica — tetany  of 
otherwise  healthy  individuals — workman's  tetany.  This  is  the  form 
which  seems  to  occur  epidemically  as  an  acute,  or  acutely  recurring 
affection  in  certain  cities,  notably  Vienna,  Heidelberg,  etc.,  principally 
in  the  early  spring  months,  and  among  certain  handworkers — tailors, 
shoemakers,  etc,  (2)  The  tetany  of  gastric  and  intestinal  affections. 
(3)  The  tetanies  of  acute  infectious  diseases,  typhoid  fever,  cholera, 
measles,  scarlet  fever,  etc.  (4)  The  tetanies  of  acute  poisoning,  chloro- 
form,  morphin,    ergot,   phosphorus,   renal,  and  gonadal    substances. 

(5)  The  tetanies  of  maternity  (pregnancy,  parturition  and  lactation). 

(6)  The  tetanies  of  parathyroid  involvement.  (7)  The  tetanies  accom- 
panying other  nerv^ous  diseases,  exophthalmic  goiter,  brain  tumors, 
cysticerci,  syringomyelia,  etc. 

Incidence. — Tetany  in  its  different  manifestations  is  very  rarely 
seen,  and  is  even  less  frequently  reported.  In  undeveloped  phases 
the  tetanoid  reaction  is  comparatively  frequent  in  children.  Gas- 
tric tetanies  are  probably  the  most  frequent,  while  the  pure  epidemic 

*  See  Osterhout:  Botanical  Gazette,  1915,  et  seq.,  for  valuable  studies  on  the  altera- 
tions of  electrical  permeability  of  cell  membranes,  due  to  bivalent  kations. 


236 


THE  ENDOCRINOPATHIES 


form  has  not  been  encountered  in  the  United  States.  In  Griffith's 
study  only  77  cases  were  found  recorded,  while  Howard's  later  collec- 
tion brings  the  American  cases  to  154  in  1907. 

Etiology. — Whether  the  work  of  MacCallum  and  A  oegtlin^  has  solved 
this  problem  is  to  be  determined,  but  it  would  appear  that  an  essential 
factor  has  been  found  in  the  relation  of  the  parathyroid  to  the  calcium 
metabolism  of  the  body.  The  hyperexcitability  of  the  neuromuscular 
apparatus  is  primarily  due  to  a  change  (chiefly  a  deficiency)  in  the 
amount  of  the  calcium  in  the  blood,  and  this  is  thought  to  be  due  to  a 
relative  or  absolute  insufficiency  of  the  parathyroid  glands.  Changes 
in  the  calcium  content  (bivalent  kations)  alter  the  permeability  of  cell 
membranes  to  electrical  stimuli,  and  the  tetany  reaction  may  be  due  to 
a  lowering  of  the  synaptic  threshold  to  sensory  stimuli  (analogous  to 
strychnine).  An  increase  in  stimuli  summation  takes  place  with  the 
overreaction  response.- 


FiG.   105. — Adenoma  in  the  parathjToid.     (Josefson.) 


Uhlenhuth  has  shown  in  the  tadpoles  of  certain  salamanders  that 
thymus  feeding  brings  on  tetany.  He  argues  that  the  thymus  contains 
a  tetany  toxin.  We  believe  the  thymus  feeding  causes  regression  in 
the  thyroid  and  parathyroid  glands  (proved  by  Dustin  and  others); 
this  causes  a  defective  parathyroid  functioning  and  thus  disarranges 
the  bivalent  kations  of  the  body  fluids  (Ca.  Mg.).  Paton  and  others 
argue  that  the  presence  of  guanidin  bases  is  responsible  for  the  tetany 
reaction.    They  do  not  work  out  the  mechanism  in  kinetic  terms. 

1  American  Journal  of  Insanity,  1909. 

2  Jelliffe,  Transactions,  Am.  Neur.  Soc,  1919. 


PARATHYROID  SYNDROMES  237 

Pathology. — Concerning  the  histological  changes,  the  present  view 
excludes  a  specific  pathology.  The  insufficiency  of  the  parath\Toids, 
be  it  relative  or  complete,  may  be  brought  about  by  a  great  variety  of 
lesions.  These  in  reality  offer  evidence  in  favor  of  the  parathyroid 
insufficiency  hj'pothesis,  but  go  no  further.  In  the  minor  grades  of 
tetany  in  children,  particularly  in  so-called  spasmophiles  which  Frankl- 
Hochwart  regards  as  tetany,  the  findings  of  Yanase,  of  Escherisch's 
clinic,  are  illuminating.  Here  hemorrhages  in  the  parathyroid  seemed 
fairly  constant  findings,  and  offer  an  explanation  of  the  galvanic  h^-per- 
excitability.  At  the  other  extreme  one  finds  the  absolute  insufficiency 
tetanies  in  experimental  parathyreopriva.  In  acute  epidemic  forms 
thyroid  (and  probably  parathyroid)  involvements  are  known.  Tumors, 
tuberculosis  and  a  host  of  other  changes  in  the  thyroids  have  been 
described.  It  will  probably  be  found  that  in  most  of  these  the  para- 
thyroids are  likewise  implicated.  Thus,  in  exophthalmic  goiter  a 
combination  of  thyroid  and  parathyroid  symptoms  is  often  present. 
In  many  tetanies  pure  thyroid  symptoms  appear.  Tetany  with 
healthy  (somatically)  glands  has  been  observed. 

Symptoms. — Considerable  variation  is  to  be  found,  but  in  general 
four  types  of  symptoms  are  observable  in  the  fully  developed  attack. 
These  are  the  muscular  spasms,  which  may  go  on  to  an  exhaustion 
paralysis,  or  paresis;  the  Trousseau  phenomenon;  increased  electrical 
excitability,  or  the  Erb  symptom;  and  mechanical  hyperexcitability 
of  the  muscles — Chvostek's  sign.  In  some  patients  one  or  more  of 
these  may  be  missing.  Incomplete  forms,  so-called,  may  present  even 
fewer  signs.  On  the  other  hand,  a  richer  combination  of  symptoms 
apparently  closely  related  to  the  general  disorder,  may  be  encountered. 
Sensory  disturbances,  anomalies  of  circulation  with  edema,  of  respira- 
ston  with  cyanosis,  and  of  temperature  are  sometimes  found.  True 
piychoses,  perhaps  indistinguishable  from  the  hysterical  confusions, 
are  found.  Trophic  disorders  of  the  skin,  hair  (alopecia  areata),  and 
nails  occur.  In  some  rare  instances,  widely  diffused  convulsive  phe- 
nomena resembling  epileptic  seizures  occur. 

Course. — Clinicians  have  recognized  arbitrarily  three  groups  of  cases 
in  adults,  and  most  modern  authors  are  inclined  to  follow  Trousseau 
in  liis  classical  description.  In  the  benign  form  the  sensory  phenomena, 
such  as  formication  or  a  simple  sensation  of  heat,  may  precede  the 
spasms.  These  are  confined  for  the  most  part  to  the  hands  or  occasion- 
ally to  the  feet.  The  contractions  may  be  fleeting,  persisting  from 
five  to  fifteen  minutes,  or  they  may  persist  for  an  hour  or  more.  Often 
the  attack  terminates  by  a  recurrence  of  the  sensory  symptoms.  A 
period  of  repose  lasting  for  a  quarter  of  an  hour  to  two  or  three  hours 
supervenes,  and  the  spasms  recur.  In  some  instances  two  or  three 
attacks  a  day  may  persist  for  several  months.  Again,  more  severe 
attacks  are  observed.  These  occur  more  often  in  young  adults,  the 
benign  forms  having  been  mostly  observed  among  children.  Here  the 
contractions  are  more  violent  and  more  painful.  The  preceding  sensory 
phenomena  are  usually  more  pronounced,  and  with  the  increase  in  the 


238 


THE  ENDOCRINOPATHIES 


severity  of  the  condition  other  symptoms  may  be  noted.    Headache, 
malaise,  and  a  rise  in  temperature  of  1°  to  3°  F.  may  be  noted.    The 


Fig.   106. — -Tetanic  spa.sm,  showing  flexion  of  wrist.     (Pool.) 

affected  muscles  may  show  signs  of  congestion,  and  localized  edema 
of  the  hands  and  feet  may  be  observed.  Other  muscles  than  those  of 
the  extremities  mav  be  involved. 


Fig.   107. — Tetanic  spasm,  showing  plantar  fle.xion  of  foot  and  toes.     (Pool.) 

These  severe  attacks  are  rarer  than  the  benign  ones.     Frankl- 
Hochwart  has  shown  that  there  isa  distinct  tendency  for  the  well 


PARATHYROID  SYNDROMES 


239 


marked  lighter  cases  in  many  instances  to  become  graver,  and  the 
good  prognosis  which  most  writers  have  given  is  seriously  doubted  by 
this  observer. 

In  the  grave  form  there  is  no  addition  of  symptoms.  The  attacks 
occur  with  greater  and  greater  frequency,  and  become  more  and  more 
intense,  and  the  patients  die  as  a  direct  result. 


Fig.   108. — Method  of  producing  tetanic  spasm  of  hand  by  stretching  the  brachial  plexus 
by  forcible  abduction  of  the  arm.     Note  "obstetrical"  hand.     (Pool.) 

Diagnosis. — The  diagnosis  of  a  classical  case  offers  few  difficulties. 
In  English-speaking  countries  it  is  apt  to  be  overlooked,  although  the 
more  frequent  reports  of  recent  years  point  to  the  fact  that  it  is  being 
recognized  more  often,  especially  in  its  milder  forms.  The  presence 
of  cramps  in  the  upper  extremities,  alone  or  in  conjunction  with  the 
upper  limbs,  with  the  classical  obstetrical  hand  and  the  additional 
evidence  supplied  by  the  Chvostek,  Trousseau,  and  Erb  signs,  is 
usually  sufficient  to  determine  a  diagnosis. 

Tetany  stnunipriva,  or,  better,  fmrathyreopriva,  as  suggested  by 
Erdheim,^  offers  the  most  classical  manifestations  of  the  disorder, 
throwing,  as  well,  considerable  light  upon  some  of  the  possible  under- 
lying and  fundamental  features  of  this  peculiar  reaction  type.  Insuf- 
ficiency of  the  parathyroids  results  in  convulsive  phenomena  of  the 
tetany  type. 

Prognosis. — The  point  of  view  here  maintained  precludes  the  possi- 
bility of  the  statement  of  a  general  prognosis.  Very  little  is  known 
definitely  of  the  prognosis  in  infants  and  children.  Most  authors 
agree  in  giving  a  fairly  good  prognosis,  although  Frankl-I  loch  wart 
says  that  healthy  children  rarely  acquire  convulsions,  and  that  the 
prognosis  is  not  good.  In  many  of  these  children  only  one  tetany- 
like  spasm  has  been  noted.    In  others  the  spasms  may  persist  for  weeks 

»  Mitt.  a.  d.  Grenzgcb.  d.  Med.  u.  Chir.,  1900,  vol.  xvi. 


240  THE  ENDOCRINOPATHIES 

and  even  months.  In  simple  cases  the  prognosis  is  much  better  than 
in  those  complicated  especially  with  gastric  or  intestinal  affections. 
Bronchitis,  pneumonia,  and  occasionally  an  ascaris  infection  also 
determined  a  less  favorable  prognosis.  Dangerous  signs  appear  with 
glossal  cramps,  which  may  cause  death.  Recurrences  are  frequent 
in  those  who  recover. 

Tetany  coming  on  during  pregnancy  and  childbirth  usually  has 
a  good  prognosis.  The  hyperexcitability  of  the  nervous  system  may 
persist  for  weeks  after  delivery.  In  succeeding  pregnancies  the  recur- 
rence of  the  phenomenon  may  be  looked  for. 

In  the  cases  apparently  due  to  disturbances  of  the  stomach  surgical 
interference  has  brought  about  distinct  amelioration.  Sudden  death 
may  occur,  and  apart  from  surgical  intervention  the  prognosis  is 
admittedly  bad  (70  to  80  per  cent.).  The  cases  are  comparatively 
rare,  however.  In  severe  cases  associated  with  marked  gastric  dila- 
tation, operation,  if  only  exploratory,  is  advisable.  The  mortality 
after  operation  in  some  dozen  or  more  cases  now  reported  is  as  low  as 
30  per  cent.  The  subsequent  history  of  these  patients  remains  to  be 
reported. 

Tetany  following  infectious  diseases  and  acute  or  chronic  poisoning 
seems  to  present  a  favorable  prognosis,  perhaps  the  best  of  the  various 
forms. 

The  prognosis  of  tetany  thyreopriva  depends  upon  the  amount 
of  thyroid  gland  removed  and  whether  the  parathyroids  are  included. 
Total  extirpation  of  the  entire  thyroid  and  parathyroid  tissue  is 
recognized  to  have  a  fatal  outcome.  Tetany  appears  after  total 
removal  of  the  parathyroids,  not  only  in  man,  but  in  lower  animals. 

Treatment.^ — From  the  standpoint  here  outlined  it  may  be  readily 
deduced  that  the  treatment  must  be  carefully  worked  out  for  each 
individual  case.  The  parathyroidectomized  individual  would  not  be 
benefited  by  a  gastric  operation. 

With  a  positive  diagnosis  established,  the  organ  involved  should 
come  into  review.  Inasmuch  as  parathyroid  insufficiency  is  the  most 
general  cause,  it  is  rational  to  treat  these  cases,  many  in  children,  the 
whole  group  of  so-called  idiopathic  tetanies,  many  tetanies  of  preg- 
nancy and  of  the  thyroid  disease  with  the  thyroid  and  parathyroid 
preparations.  Parathyroid  preparations  seem  to  fulfil  most  of  the  con- 
ditions, yet  occasionally  the  combined  thyroid  and  parathyroid  involve- 
ment renders  the  giving  of  the  combined  products  of  more  service. 

The  use  of  foodstuffs  rich  in  calcium  and  of  calcium  salts  follows  as 
a  natural  corollary  from  the  studies  enumerated.  Such  medication 
may  entirely  replace  the  use  of  the  glandular  substances  themselves. 
In  experimental  tetanies  the  success  of  the  calcium  salts  has  been 
very  striking,  and  in  tetanies  in  children  calcium  therapy  has  given 
almost  uniformly  good  results.^     Such  therapy   apparently  renders 

1  See  Howland  and  Mtirriott:  Quart.  Jour.  Med.,  July,  1918,  for  good  discussion. 
The  dynamic  concept  of  the  organism  as  a  transmitter  of  energy  is  not  utilized  in  this 
otherwi.sQ  Qxcell^nt  study.    Also  consult  J^Uiffe:  Trans,  Am,  NQur,  Soc,  1919, 


DISEASES  OF   THE  HYPOPHYSIS  241 

the  older  means  unnecessary,  such  as  curare,  opium,  hyoscyamus,  the 
bromides,  chloral,  belladonna,  chloroform,  galvanism,  sweat  baths, 
etc.  Up  to  the  present  time  therapeutic  experience  is  not  sufficient 
to  definitely  prove  the  durability  of  calcium  medication  in  the  cases 
in  which  it  seems  to  be  indicated.  Light  therapy  to  enlarged  and  per- 
sistent thymic  glands  has  been  of  service  in  some  cases. 

The  surgical  expedient  of  transplanting  parath\Toid  tissue  has  proved 
successful  in  animal  work;^  its  successful  application  in  persistent 
chronic  tetanies  in  man  is  clearly  foreshadowed  by  the  experimental 
work  on  dogs.  The  technical  difficulties  do  not  seem  insuperable  in 
view  of  the  ready  transplantation  of  these  structures  in  different  parts 
of  the  body. 

DISEASES  OF  THE  HYPOPHYSIS— PITUITARY. 

The  terms  h^-pophysis  and  pituitary  have  been  employed  sjTiony- 
mously  but  the  present  usage  is  to  reserve  the  term  hypophysis  to 
designate  the  collective  structure  made  up  of  two  distinct  parts  with 
very  different  functions.  An  anterior  part  (pars  anterior) ,  the  pituitary 
which  is  epithelial  and  derived  from  the  gastro-enteron,  and  a  posterior 
part  (pars  nervosa) ,  which  is  nervous  in  origin  and  termed  the  infundib- 
ular process.  A  pars  intermedia  separates  the  two  but  in  reality  is  a 
part  of  the  pituitary.  Falta  is  in  doubt  about  this.  It  is  derived  from 
the  oral  cavity  and  contributes  its  secretion  to  the  cerebrospinal  fluid. 
The  whole  structure  is  innervated  by  the  vegetative  nervous  system. 
By  reason  of  its  special  relations  to  the  optic  chiasm,  third,  fourth  and 
sixth  nerves  and  to  the  infundibulum  alterations  in  the  gland  produce 
not  only  syndromes  correlated  with  the  internal  secretions — ^pituitrin 
(anterior  lobe)  and  infundibulin  (posterior  lobe),  but  also  may  give 
rise  to  profound  neurological  disturbances  of  these  contiguous  struc- 
tures. Accessory  pituitary  structures  are  known:  Parahypophysis, 
hypophysis  pharyngea.  The  physiology  of  the  gland  cannot  be 
discussed  here;  the  student  is  referred  to  the  works  given  in  the 
introduction  to  the  endocrinopathies :  the  monographs  of  Gushing, 
Hinsdale,  Falta,  Xoel  Paton,  Schaefer,  Lewandowsky,  Pende,  and 
Bell  (1919)  are  the  most  available  and  reliable.  Tilney's  important 
mcmograph  (1911)  gives  the  chief  anatomical  features. 

As  yet  the  complex  s^-ndromy  of  the  varying  grades  of  involvement 
of  anterior  and  posterior  lobes  is  not  completely  analyzed.  Robertson 
has  isolated  a  substance  called  tethalein  from  the  anterior  lobes  which 
are  stated  to  have  marked  bony  growth  functions.  It  is  possible  that 
phosphorus  metabolism  factors  are  here  partially  concentrated. 

A  large  number  of  substances  have  been  thought  to  liave  been  iso- 
lated from  the  ])ituitary  proper.  Some  of  these  (pituitrin)  have  a 
definite  action  on  unstriped  muscular  fiber,  hence,  cause  uterine  con- 
tractions, raise  blood-pressure,  induce  peristalsis,  cause  bladder  con- 

1  Leischner:  Arch.  f.  kliii.  Chir.,  1907,  Ixxxiv,  1,  208. 


242  THE  ENDOCRINOPATHIES 

tractiire  (polyuria).  Others  cause  increased  milk  secretion,  increase 
the  sugar  mobilization  and  modify  metabolism  in  general;  thus  dim- 
inished action  causes  increase  in  weight,  in  fatty  deposits  of  the  lipo- 
matoses types,  differing  from  the  fatty  states  of  hypothyroid  types. 
Changes  in  the  cerebrospinal  fluid  flow  and  modification  of  gonadal 
activities  are  frequent.  An  interaction  with  the  pineal  seems  to 
underlie  hypergenital  developments  and  vice  versa. 

Hj'perfunction  of  the  anterior  lobe  may  take  place  at  any  time, 
either  primarily  or  as  compensatory  to  other  defect  states.  The  chief 
impulse  in  bony  development  seems  to  be  exerted  on  the  long  bones 
— hence  giants,  acromegaly,  disproportionately  long  arms  or  legs, 
prognathism,  stumpy  fingers.  A  closed  sella  may  limit  this  capacity 
for  growth.  This,  if  the  clinoids  do  not  erode  as  they  frequently 
do,  often  gives  rise  to  a  definite  headache  syndromic^  which  is  relieved 
as  the  clinoids  erode  or  the  gland  can  enlarge,  permanently  or  period- 
ically. There  may  be  daily,  monthly  or  yearly  functional  changes 
in  the  size  of  the  h}T>ophysis.  These  are  seen  in  hibernating 
animals  and  at  menstrual  epochs  in  women  and  may  be  related  to 
uterine  contractions,  metrorrhagias,  etc.  Diminished  h^^^ophyseal 
development  may  show  as  various  dwarfings,  small  hands,  micro- 
miria,  small  close  teeth,  high  palatal  arches,  faulty  hair  development, 
adiposity,  with  or  without  mental  (social)  defects.  Such  defect  states 
of  minimal  grade  are  very  frequent  and  various  psychosexual  retar- 
dations— narcissism,  overt  and  latent  homosexual  trends,  etc.^ — are 
among  the  possibilities. 

Clinically  three  gross  trends  may  be  recognized  which  are  due  to 
increased,  diminished  or  irregular  functioning  of  different  parts  of  the 
pituitary  structures.  The  analyses  of  these  syndromes  has  only  just 
begun,  but  in  view  of  Cushing's,^  Tilney's^  and  Timme's^  fundamental 
studies  on  the  hypophyseal  structures  syndromy  groupings  will  develop : 

(1)  Hyperintuitarism  is  associated  with  gigantism  and  with  acromegaly; 

(2)  hypoyituiiarism  with  various  grades  of  infantilism,  physical  and 
mental,  with  adiposity  and  genital  dystrophies;  (3)  dysjyitiiitarism 
shows  many  mixed  syndromes.  Absolute  loss  of  the  pituitary  occurs 
very  rarely. 

Hyperpituitarism:  Acromegaly  and  Gigantism. — These  conditions 
are  closely  related,  showing  overgrowth  in  the  skeleton  and  particu- 
larly in  the  long  bones  in  gigantism;  changes  in  the  toes,  fingers  and 
bones  of  the  face,  more  prominent  in  the  acromegalic  tendency.  In 
general  gigantism  occurs  when  the  disorder  begins  prior  to  epiphyseal 
union,  acromegaly  when  the  changes  occiu"  after  the  union  of  the 
epiph^^ses.  Prodromata  such  as  fatigue,  muscular  pains,  apathy  and 
sleepiness  are  frequent. 

'  See  Timme,  Endocrinology,  1919,  for  careful  outline  of  a  polyglandular  syndromy 
of  this  type. 

2  The  Pituitary  Body,  Philadelphia,  1912. 

'  Comparative  Histology  of  the  Hypophysis,  Memoirs  Wistar  Inst.,  1911. 

*  Loc.  cit. 


DISEASES  OF   THE  HYPOPHYSIS 


243 


Acromegaly  is  characterized  by  the  gradual  enlargement  of  the  bones 
of  the  nose,  jaw,  hands  and  feet  and  a  hyperplasia  of  all  of  the  bony 
structures  due  to  an  overactivity  of  the  vegetative  nervous  system. 
This  overexcitability  of  the  nervous  regulators  of  metabolism,  from 
excessive  glandular  secretion,  also  induces  hyperplasia  of  other  endo- 
crinous structures,  notably  the  thyroids,  interstitial  gonadal  cells  and 
the  suprarenal  cortex.  From  these  contributory  factors  arise  a  medley 
(often  contradictory)  of  th\Toid,  genital  and  vascular  anomalies,  some 
in  the  nature  of  hyperplasias  (see  Thyroid),  some  of  a  degenerative  or 
inhibitive  character  (see  Gonads),  such  as  hairy  and  genital  defects. 
The  change  in  the  pituitary  itself  is  most  frequently  of  an  adenomatous 
or  adenosarcomatous  type,  although  this  is  not  invariable.  In  pure 
adenomata  of  the  pituitary  the  symptoms  tend  to  be  more  clean-cut 


Fig.   109. — Acromegaly.     (Josefson,  Hygiea,  1904.) 

and  classical.  As  a  rule  the  whole  hypophysis  is  implicated  which 
brings  the  posterior  lobe  (infundibulin)  into  increased  or  diminished 
activity  with  contradictory  and  mixed  syndromes,  the  minute  details 
of  which  must  be  looked  for  in  the  rich  and  growing  periodical  literature. 
Symptoms. — The  growth  in  acromegaly  is  very  gradual,  usually 
occurring  between  the  ages  of  twenty  and  forty.  It  includes  changes 
in  the  skin  and  hair  as  well  as  in  the  bones.  These  latter  are  all  hyper- 
trophied,  causing  striking  peculiarities  in  appearance,  particularly 
of  the  face.  The  nose  is  greatly  thickened,  as  are  also  the  superciliary 
ridges  and  the  malar  bones.  The  eyebrows  are  heavy,  overhanging 
and  coarse;  the  lips  are  thickened  and  protrude,  with  marked  pro- 
jection of  the  often  enormously  hypertrophied  lower  jaw.  This 
hypertrophy  causes  the  spreading  of  the  teeth,     "^rhe  mucous  mem- 


244 


THE  ENDOCRINOPATHIES 


Fig.   110. — Acromegaly.     (Josefson,  Hygiea,  1904.) 


Fig.   111. — Sella  turcica  in  patient  with  acromegaly.     (Josefson.) 


DISEASES  OF   THE  HYPOPHYSIS 


245 


branes  share  in  the  hypertrophy.  This  marked  cranial  bone  alteration, 
with  the  presence  of  the  tumor  causes  a  greatly  enlarged  sella  turcica. 
The  hands  and  feet  are  notably  widened,  the  fingers  and  toes  stumpy 
and  thick.  The  skin  and  hair  throughout,  inclusive  of  the  genitals, 
show  the  same  hypertrophies,  as  do  practically  all  of  the  bones  of 
the  skeleton.  Amenorrhea  is  frequent  in  women  and  loss  of  potency 
in  men,  usually  associated  with  atrophy  of  the  gonads.  Glycosuria  is 
frequent.  Carbohydrate  tolerance  may  be  high,  however,  and  an 
increased  fondness  for  sw^eets  is  frequent.  The  muscular  tissues  have 
a  tendency  to  atrophy  early  in  the  disorder  following  hypertrophy. 


Fig.  112. — Characteristic  hand  of  acromegaly.  Note  heaping  of  tissues  about  nails,  "type 
en  large"  of  Marie.     Compare  vdth  Fig.  113.     (From  Cushing's  "Pituitary  Body.") 

Anomalies  dependent  upon  thyroid  alterations  are  frequent.  These 
consist,  for  the  most  part,  of  increased  sweating,  tachycardia,  diar- 
rheas, exophthalmos  at  times,  Stelwag's  symptom,  irregular  palpebral 
fissures,  variations  in  pupillary  equality,  tremor,  thermal  alterations 
and  marked  irritability.  Suprarenal  cortex  alteration  is  apparently 
related  to  the  arteriosclerosis  frequently  seen,  diabetes  mellitus  or 
glycosuria,  and  other  signs  of  altered  adrenalin  (q.  v.)  activity. 

In  addition  to  the  essential  metabolic  disturbances,  symptoms  due 
to  the  nature  of  the  producing  lesions — tumor,  hyperplasia,  i.  e., 
neighborhood  symptoms,  are  frequently  found,  but  these  are  not 
invariable. 

Severe  bitemporal  headaches  are  frequent.  This  is  an  intracranial 
pressure  sign.  The  sella  turcica  is  usually  enlarged  from  tumor 
formation,  as  disclosed  by  the  a:-ray  examination. 


246 


THE  ENDOCRINOPATHIES 


Pressure  upon  the  optic  nerves  at  the  chiasm  is  usual,  leading  to 
various  types  of  hemianopsia  or  even  blindness.  Distorted  fields  are 
the  rule. 

Mental  symptoms  ranging  from  sluggishness  to  severe  deteriora- 
tion occur,  but  are  not  invariable.  Epileptic  attacks  may  accompany 
hypophyseal  deficiency. 

Prognosis. — This  is  always  grave.  The  disorder  is  progressive, 
usually  very  gradual,  five  to  twenty  years,  but  the  advance  in  symp- 
toms may  be  arrested  spontaneously.  As  yet  no  positive  mode  of 
influencing  slight  grades  of  hyperpituitarism  is  known.  Polyglandular 
experimentation  is  widely  employed.  Thyroid  preparations  are  useful 
in  those  cases  with  accompanying  hypothyroidism.  Careful  analysis 
of  the  symptoms  will  afford  other  suggestive  clues.  Light  therapy 
has  been  useful  in  some  cases.  When  pressure  symptoms,  enlarged 
sella,  visual  defects  develop,  operation  is  advisable.  The  results 
have  at  times  been  brilliant. 


Fig.  113.- 


-Typical  tapering  hand  of  adolescent  hypopituitarism.    Compare  with  Fig.  1 12 
(From  Cushing's  "Pituitary  Body.") 


Hypopituitarism. — Deficiency  of  the  pituitary  substance  gives  rise 
to  a  group  of  syndromes  the  most  classical  of  which  is  Frohlich's  dps- 
trophia  adiposogenitalis,  probably  a  dyspituitary  whole  gland  disorder. 

Definition. — The  clinical  picture  is  characterized  by  a  progressive 
accumulation  of  fat,  often  localized,  chiefly  about  the  buttocks  and 
breasts,  as  is  seen  in  the  gonadal  disturbances  of  eunuchs.  This  is 
possibly  correlated  with  defective  activity  of  the  interstitial  glands — 
from  autonomic  and  sympathetic  action  of  the  deficient  pituitrin,  with 
the  consequent  failure  of  development  of  the  secondary  sexual  charac- 


DISEASES  OF  THE  HYPOPHYSIS 


247 


ters,  involving  the  genitals.  The  psychosexual  development  even  is 
hindered,  showing  as  various  grades  of  conscious  and  unconscious 
homosexuality.  Polyuria  is  frequent  and  additional  pressure  signs 
(tumor)  may  be  found  (optic  nerve  changes). 

Etiology  and  Pathogenesis. — A  not  infrequent  cause  for  hypopitui- 
tarism is  hydrocephalus.  This  occurring  in  the  young  from  numerous 
causes — acute  inflammation,  hereditary  syphilis,  tuberculosis,  polio- 
encephalomyelitis,  etc. — tends  to  bring  about  a  compression  of   the 


Hoger 


Fig.   114. — Visual  fields  in  case  of  acromegaly.     (Kl.  Monatsbl.  f.  Auglik,  1915,  55. 
Om  akromegalia  och  hypofystumorer,  1903.)     (A.  Josefson.) 

pituitary  with  atrophy.  Tumors  which  in  the  early  stages  may  cause 
acromegalic  symptoms,  may  later  lead  to  destruction  with  defect 
symptoms.  Tumors  may  also  bring  about  the  syndrome  without 
acromegalic  features.  Frankl-Hochwart^  has  made  a  collection  of  a 
large  number  of  these. 

The  pathogenesis  is  not  clear.     Loss  of  pituitary  substance  itself 
is  a  sine  qua  non,  but  whether  this  loss  acts  purely  chemically  or  is 

1  XVIth  International  Congress,  Budapest,  1909. 


248 


THE  ENDOCRINOPATHIES 


mediated  by  the  vegetath'e  nervous  system  is  still  not   understood. 
The  latter  hypothesis  is  the  more  fav'ored. 

Symptoms. — The  disorder  is  chiefly  developed  in  youth.  The 
obesity  is  the  most  striking  feature  in  the  youthful  cases.^  The  hips, 
buttocks,  mons  veneris  and  mammary  glands  are  the  chief  localizations. 
The  lower  abdomen  is  involved  in  both  young  and  older  cases.  Fatty 
cuffs  on  the  malleoli;  clavicular  collar-like  thickening  are  other  local 
sites  for  the  accumulation.     Cases  without  obesity  are  known. 


Fig.  115. — Case  of  post-traumatic  hypopituitarism  in  a  child,  with  extreme  adiposity, 
high  sugar  tolerance,  and  epilepsy.  Marked  improvement  with  whole  gland  feeding 
(pituitary).     (From  Cushing's  "Pituitary  Body.") 

The  skin  is  alabaster- like,  and  in  the  adult  types  is  cold,  hard,  and 
dry  and  exfoliates  readily.  At  times  it  is  myxedematous.  The  hairy 
parts  are  much  smoother  or  all  hair  is  absent. 

The  genitals  are  underdeveloped,  penis  small,  and  buried  in  cushions 


1  Consult  Falta's  discussion,  p.  320,  Philadelphia. 


DISEASES  OF  THE  HYPOPHYSIS 


249 


of  fat.  The  scrotum  is  small  and  the  testicles  may  not  descend.  The 
labiffi  remain  infantile,  the  o^'aries  small  and  the  breast  glands  defec- 
tive.    Menstruation  is  irregular. 

The  voice  may  remain  thin  and  child-like  and  the  type  of  object 
fixation  remain  infantile  (asexual  or  homosexual).^ 

As  a  rule  there  is  a  fairly  persistent  though  slight  subnormal  tem- 
perature, a  marked  degree  of  sugar  tolerance,  marked  reduction  in 
respiratory  exchange,  slowed  pulse  and  a  tendency  to  sluggishness  or 
even  sleepiness.  The  blood  picture  tends  to  show  a  slightly  reduced 
red  cell  count,  reduction  in  hemoglobin,  the  neutrophiles  are  distinctly 
reduced,  the  mononuclears,  lymphocji;es  and  eosinophiles  increased. 
Taper  fingers  are  a  contrasting  picture  to  the  pudgy  ones  of  acromegaly. 

The  patients  often  remain  childish  in  their  stature  (the  lower 
extremities  usually  being  much  larger  proportionately  to  the  upper  in 
direct  contrast  with  gonadal  infantilism),  and  in  their  psyche.  Lilli- 
putian dwarfism  is  associated  with  hypophyseal  defect  (teratoma).^ 


Fig.   116. — Spreading  of  the  teeth  in  hj'perpituitary  disorder.     (Josefson.) 

Neighborhood  symptoms  may  also  be  observed,  as  with  the  acro- 
megalic patients.  The  optic  nerve  changes,  bitemporal  hemianopsia, 
are  among  the  most  important.  Other  symptoms  of  a  general  nature 
due  to  pressure,  as  headache,  nausea,  vomiting,  changes  in  the  sella 
turcica,  etc.,  often  occur,  especially  from  tumors  which  destroy  the 
hypophysis.     Trigeminal  neuralgia  has  been  observed. 

Dyspituitarism. — Under  this  head,  a  great  number  of  constitutional 
anomalies  due  to  disturbed  pituitary  secretions  may  be  gathered. 
These  are  incomplete  forms  of  acromegaly  and  gigantism,  cases  of 
adiposity,  alone  or  with  genital  atrophies,  or  genital  anomalies,  showing 
hyperfunction  or  hypofunction.  Various  epilepsies,  probably  condi- 
tioned by  hydrocephalic  changes  occur  with  dyspituitarism  and  at 
times  are  helped  by  pituitary  therapy.'  Variations  in  mental  capacity 
are  frequent,  as  well  as  a  variety  of  anomalies  such  as  increased  sugar 
tolerance  or  glycosuria;  slightly  sub-  or  supranormal  temperatures, 
polyuria,  wakefulness,  irritability  and  a  group  of  character  anomalies 
as  well.    The  skin  is  usually  smooth  and  soft  and  free  from  moisture, 


'  See  an  important  study  by  O'Malley,  Jour.  Nervous  and  Mental  Disease,  July,  1918, 
for  various  hypophyseal  defect  states,  mental  syndromes  and  psychosexual  anomalies. 
'  Konne:  Deutsch.  med.  Wchnschr.,  1915. 
»  Munson,  J.  F.:   Pituitary  Gland  in  Epileptics  II,  Arch.  Int.  Med.,  April,  1918. 


250 


THE  E N DOC RINOPAT HIES 


and  the  hair  is  apt  to  be  thin,  fine  and  scanty.  A  great  variation  in 
cases  will  be  found  and,  in  fact,  nearly  all  the  dyspituitary  syndromes 
are  polyglandular  in  their  manifestations.' 

The  total  absence  of  pituitary  substance  brings  about  conditions  of 
lethargy  and  narcolepsy,  with  marked  slowing  of  the  pulse  and  of  the 
respiration.  There  is  insensibility  to  pain,  marked  reduction  in  tem- 
perature and  in  blood-pressure  and  slow  death. 


Fig.    117. — Hypopituitarism  in  boy. 
(A.  Josefson.) 


Fig.  118. — Hypopituitarism  in  man. 
Tumor  hypophysis;  twenty-nine  years. 
(A.  Josefson,  Kl.  Mon.  f.  Augenh.,  1915.) 


Syndrome  of  Benoii-DeliUe}  ^ — ^These  authors  have  described  a 
syndrome  of  hypophyseal  insufficiency  characterized  by  lowering  of 
the  arterial  tension,  tachycardia,  diminution  in  the  amount  of  urine, 
insomnia,  increase  in  perspiration,  and  inability  to  stand  heat.     These 


1  Beck:  Am.  Jour.  Med.  Sc,  November,  1918. 

2  Congress  de  Med.,  Paris,  1907. 

3  Th^se  de  Paris,  Steinheil,  1909. 


DISEASES  OF  THE  HYPOPHYSIS 


251 


symptoms,  often  confused  with  a  so-called  functional  myocarditis, 
clear  up  under  hypophyseal  medication. 

Treatment. — Acromegalic  patients,  or  those  showing  pituitary  syn- 
dromes due  to  evident  tumor  of  the  hypophyseal  region,  need  surgical 
intervention,  whether  the  signs  of  hypo-  or  hyperpituitarism  be  present. 
Hypopituitary  and  dyspituitary  cases  without  neighborhood  symp- 
toms of  tumor  may  be  given  pituitary  extract  (0.1  gm.  of  combined 
extracts)  sometimes  to  advantage.  In  certain  refractory  cases  com- 
bined opotherapy,  pituitrin  and  th\Toid  may  be  employed  to  advan- 
tage. Certain  stationary  acromegalic  cases  are  benefited  by  this 
treatment  and  others  apparently  are  rendered  stationary. 

Radiotherapy  is  in  general  inefficient. 


Fig.   119. — Adipose  genital  dystrojjhy.    Hypopituitarism.    Tumor  of  pineal. 
(Bailey  and  Jelliffe.) 

The  chief  surgical  modes  of  relief  are:  (1)  sellar  decompression,  for 
hypophyseal  headaches,  or  to  permit  a  tumor  mass  to  expand  outside 
of  the  cranial  cavity;  (2)  partial  removal  of  a  hyperplastic  and  over- 
acting gland;  (.3)  partial  removal  for  the  sake  of  saving  eyesight;  (4) 
subtemporal  decompression  to  relieve  general  brain  pressure  symptoms; 
(5)  Anton's  callosal  puncture;  (6)  combined  operations;^  (7)  operations 
for  glandular  transplantation. 


'  See  Gushing,  Pituitarj'  Body,  Lippincott,  1912. 


252  THE  EN  DOC  RI  NOP  AT  HIES 

Infundibular  Syndromes. — Little  positive  concerning  a  pure  syn- 
drome of  posterior  lobe  disease  is  established.  Its  exclusive  removal 
seems  to  be  well  borne  by  animals  if  the  anterior  lobe  is  left  intact. 
Its  active  principle,  infundibulin  (hypophysin),  acts  much  like 
adrenalin  but  apparently  through  other  pans  of  the  vegetative  reflex 
arc  than  does  adrenalin,  the  chemical  structure  of  which  it  does  not 
resemble.  In  animals  (rats)  feeding  experiments  have  shown  that  it 
has  a  retarding  eflFect  on  the  development  of  the  sex  glands,  in  contrast 
with  a  marked  stimulation  from  feeding  with  the  anterior  lobe  extract.^ 

Polyuria,  arrested  growth  and  low  temperature  are  often  combined 
with  tumor  of  the  infundibular  portion  of  the  hypoplysis.  As  the 
different  parts  of  that  structure  are  so  closely  related  a  definite 
syndromy  for  purely  posterior  disorder  is  still  in  the  making.^ 

DISEASES   OF   THE   PINEAL   ORGAN. 

Pineal  Syndrome.^ — By  Gaskell  the  pineal  gland  (epiphysis)  is  said 
to  appear  as  a  vestigial  remnant  of  the  paired  median  eyes  of  the 
paleostracean  ancestor  of  the  vertebrate  stock.  As  low  down  in  the 
animal  phylum  as  Ammocoetes  "only  one  of  these  structures,  right, 
persists  and  it  is  rudimentary.  Through  Meynert's  bundle  connection 
is  made  with  the  ganglion  habenulse,  traces  of  which  still  persist  in  the 
human  brain.  Gaskell  believes  this  ganglion  habenulse  to  be  the 
primitive  optic  ganglion  of  the  median  eye.  Researches  by  Tilney 
seem  to  disprove  this  general  assertion  and  show  a  separate  embryo- 
logical  and  phyletic  origin  for  the  pineal  gland  and  the  pineal  eye. 
He  also  shows  fairly  definitely  the  endocrinous  nature  of  the  pineal 
body." 

The  varying  syndromes  are  still  only  hazily  outlined.  Timme's 
researches  point  to  muscular  defect  syndromes  associated  with  pineal 
gland  defect  states,  much  relieved  by  pineal  therapy. 

Tumors  of  the  pineal,  chiefly  teratomata,  when  they  become  about 
one- half  inch  in  diameter,  cause  a  striking  group  of  symptoms  due 
chiefly  to  (1)  a  hydrocephalus,  which  causes  the  adiposogenitalis 
syndrome  of  Frohlich,  in  part;  a  sexual  precocity,  in  part,  and  (2)  by 
pressure  upon  the  quadrigemina,  certain  ocular  palsies.  A  combination 
of  these  is  the  pineal  syndrome.  Partial  pineal  syndromes  in  which 
fatty  and  muscular  anomalies  are  present  seem  to  show  a  relationship 
between  the  hormone  action  of  this  structure  and  muscular  dystrophic 
states.     (See  Muscular  Dystrophies.^) 

An  interaction  between  the  pituitary  and  the  pineal  seems  to  be 
established  and  a  giant  type,  a  small  genital  type  and  a  large  genital 
type  may  result  from  reciprocal  pituitary  and  pineal  balances.    The 

*  Weber  and  Schmidt:  Am.  Jour.  Med.  Sc,  December,  1916. 
2  Newmark:  Infundibular  Tumor,  Arch.  Int.  Med.,  1917. 
'  Bailey  and  Jelliffe:  Arch.  f.  Int.  Med.,  1912. 

'  Tilney:  The  Pineal  Body,  Memoirs  Wi.star  Institute,  1918.  Timme:  Progressive 
Muscular  Dystrophy,  Archives  of  Internal  Medicine,  1917. 


DISEASES  OF   THE  SUPRARENAL  BODY 


253 


large  genital  type  seems  to  come  on  as  the  pineal  involutes  and  the 
pituitary  enlarges.     This  is  still  conjectural. 

Operative  interference  may  save  a  patient  with  pineal  tumor  but 
the  technical  surgical  difficulties  are  extremely  great.  Pineal  gland 
therapy  is  still  in  an  experimental  stage. 


r 

^^^^B 

^^^H 

Mk 

iiJl 

^^^^H 

1 

P>Z- 

I^^H 

i 

tl^ 

'^^^^^^m 

^^^^^^^^^^^^K  -.•- 

1 

^^^^^V   ^^^^^H 

pH 

B 

^^j^^^m 



—  '•^^^^^^^^^^^^^^^ 

U^^^^^^^^^^^^^^HB 

Fig.  120. — Progressive  muscular  dystrophy  supposedly  related  to  pineal  gland  defect. 
The  roentgen-ray  picture  of  the  brain  showed  a  pineal  shadow.     (Timme.) 

DISEASES   OF   THE   SUPRARENAL  BODY.i 

Suprarenal  Sjnidromes. — From  the  standpoint  of  comparative 
anatomy^  the  adrenal  glands,  the  s^Tnpathetic  part  of  the  vegetative 
nervous  system  and  the  chromaffin  tissue  must  be  considered  as  one 
in  relation  to  the  blood  vascular  system.  From  the  lowest  vertebrate 
forms  there  is  a  close  relation  between  the  adrenal  bodies  and  the 
sympathetic  system.  In  Petromyzon  two  distinct  sets  of  bodies  are 
known.  One  situated  in  the  walls  of  the  cardinal  veins,  the  renal 
arteries  which  project  into  the  lumen  of  these  vessels.  They  are  the 
homologues  of  the  cortical  or  interrenal  bodies.  Another  body,  the 
chromaffin  series,  extends  from  the  region  of  the  second  gill  slit  to  the 
tail,  running  along  the  arteries  and  their  branches.  These  correspond 
to  the  medulla.  In  Reptilia  the  relationship  is  closer;  in  birds,  one 
body  occupies  the  meshes  of  the  other  and  in  mammals  the  cortex 


'  Lucien  et  Parisot:  Glandes  surrcnales  et  organes  chroniaffines,  Paris,  1913. 
^  Orr  and  Rows:    The  Interdependence   of   the   Sympathetic  and  Central  Nervous 
Sj-stems,  Brain,  1918,  xli,  p.  1. 


254  THE  ENDOCRINOPATHIES 

surrounds  the  medulla.  True  chromaffin  tissue  is  widely  distributed, 
and  is  not  confined  to  the  adrenals.  It  is  found  also  in  the  sympathetic 
paraganglia  of  the  solar  plexus,  Zuckerkandl's  aortic  ganglia,  the 
cardiac  paraganglia,  the  coccygeal  and  carotid  ganglia  of  Luschka, 
the  tympanic  paraganglia  and  chromaffin  cells  are  found  in  the 
central  gray  substance  from  the  cauda  to  the  brain. ^  The  tissues 
themselves  are  richly  supplied  with  sympathetic  nerve  fibers. 

All  chromaffin  tissues  yield  a  true  internal  secretion,  adrenalin, 
whose  chemical  composition  is  known:  ortho-dioxy-phenyl-ethanol- 
methylamine. 

H 

c  H         H    H    H 

/  \  I  III 

HO— C  C        C—    — C— N— C— H 

HO— C  C— HOH        H  H 

\   / 
C 

H 

Its  nearest  relative  is  ty rosin,  a  well-known  product  of  protein  decom- 
position. The  chief  action  of  adrenalin  is  upon  the  sympathetic 
nervous  fibers  increasing  their  reactive  capacity,  or  sensitizing  them, 
as  it  were.  The  routine  function  of  the  chromaffine  tissue  is  to  react 
to  metabolic  stimuli  largely  in  response  to  desire  and  fear.  Their 
emergency  function,  as  Cannon  has  termed  them,  is  to  provide  the 
necessary  overresponse  to  emotional  hyperactivity — i.  e.,  to  increased 
or  diminished  desire  and  fear — which,  as  their  correlates  love  and  hate, 
are  the  ultimate  expressions  in  the  symbolic  sphere  of  what  are  instinc- 
tively known  as  useful  or  harmful  agencies  to  the  organism  and  to  the 
race.  This  over-  or  underresponse  brings  about,  through  widespread 
vegetative  nervous  system  activities,  including  those  upon  other  endo- 
crinous glands,  the  approximately  necessary  metabolic  adjustment. 
This  takes  place  chiefly  through  the  regulation  of  the  blood  volume 
and  of  the  organic  and  inorganic  constituents  of  its  plasma.  Adrenalin 
itself  is  present  in  the  plasma  in  proportions  of  1  to  20,000,000.  Not- 
withstanding this  extreme  dilution  it  acts  upon  unstriped  muscle  fiber 
and  on  sympathetic  receptors.  Adrenalin  then  is  a  typical  product 
which  demonstrates  the  metabolic  regulation  mechanisms  of  the 
vegetative  nervous  system.  In  addition  to  this  broad  function  of 
keeping  the  sympathetic  nerve  fibers  in  adjustment  it  has  many 
specific  functions.  One  of  the  mechanisms  more  clearly  established, 
but  not  entirely  worked  out,  is  the  interaction  with  the  pancreatic 
internal  secretions,  whereby  muscle  sugar  utilization  is  partly  regu- 
lated. Asthenia  is  the  chief  symptom  which  comes  out  when  this 
function  is  disturbed.^    The  adrenals  are  profoundly  under  the  influ- 

•  Bruni:    Archivio  per  le  Scienza  Medichi,  1917,  xl.  p.  146. 

2  Mackenzie,  G.  M.:  Suprarenal  System  and  Carbohydrate  Metabolism,  Arch.  Int. 
Med.,  1917. 


DISEASES  OF   THE  SUPRAREXAL  BODY  255 

ence  of  emotional  states.  One  hardly  needs  to  be  reminded  of  the 
herculean  efforts  that  the  lover  can  put  forth,  or  the  collapse  states  of 
fear,  despondency  and  chagrin.^ 

The  numerous  polyglandular  syndromes  which  have  been  described 
cannot  be  discussed  here  in  detail.  Their  name  is  legion  and  most 
are  still  so  incompletely  thought  out  that  were  it  not  for  the  many 
suggestive  possibilities  they  could  be  dismissed  with  few  words. 
Only  the  more  pronounced  forms  can  be  taken  up  but  the  student  is 
reminded  that  a  great  many  constitutional  anomalies,  eccentricities 
and  incapacities  are  related  to  excess  and  defective  suprarenal  activities, 
in  which  the  medullary  and  cortical  portions  must  be  separately 
indicated  in  explaining  the  symptomatology  of  the  endocrinopathic 
disturbances. 

The  general  pathological  anatomy  of  the  suprarenals  comprises 
congenital  aplasia  usually  associated  with  varying  tA'pes  of  monsters, 
unilateral  absence  of  the  suprarenal  has  been  observed  frequently 
(Winslow,  Legg,  Bramwell),  simple  atrophy,  inflammatory  and  toxic 
atrophy,  neurotic  atrophy  (Laignel-Lavastine)  related  to  disease  of 
the  abdominal  sympathetic  plexuses,  simple  h^-pertrophy  and  hyper- 
plasia, compensators'  hApertrophy  chiefly  of  the  cortex  from  disease 
of  one  of  the  glands  or  from  causes  as  yet  midetermined,  h^-per- 
plastic  reaction  to  chronic  sclerosing  disorders  and  differentiated  as 
nodular  and  miliary  hyperplasias  adenomata.  This  hyperplasia  may 
involve  both  the  cortex  and  the  medulla  or  either  alone,  the  adenoma- 
tous hAperplasise  involving  chiefly  the  cortex.  Suprarenal  degenera- 
tion, parenchymatous,  fatty,  hyaline,  amyloid,  calcareous.  Among 
the  vascular, alterations  of  the  adrenal  active  and  passive  congestion, 
thrombosis  and  infarcts  and  hemorrhage  must  be  held  in  mind,  and 
a  group  of  inflammatory  and  toxic  changes  are  known.  Thus,  an 
acute  adrenalitis  with  a  fairly  definite  pathology  has  been  described, 
whereas  a  large  group  of  chronic  adrenalitides  have  been  studied, 
particularly  by  Delamare,  Husnot  and  Sezary.  Syphilitic,  tubercu- 
lous, mycotic  and  parasitic  lesions  are  also  known.  The  tumors  of  the 
suprarenal  make  up  a  final  and  large  group.  All  kinds  of  tumors  of 
the  adrenals  have  been  found. 

Hypoadrenalemia. — The  most  acute  form  which  is  present  in  com- 
plete or  great  loss  of  the  suprarenals  is  rare.  Pende^  has  described  six 
t\pes,  to  which  he  gives  the  names  impromptu  death  of  suprarenal 
origin,  pseudoperitoneal  type,  cholera-like  or  gastro-intestinal  adrena- 
lemia,  apoplectiform  type,  meningo-encephalitic  type  and  myocardial 
type.  In  the  first  form  individuals  suddenly  die  without  warning, 
without  symptoms  save  perhaps  an  epileptiform  cry,  or  acute  dyspnea 
or  angina.  Caseous  degeneration  of  the  suprarenals  has  been  observed. 
The  pseudoperitoneal  forms  resemble  an  inexplicable  attack  of  acute 
peritonitis  with  death.     The  gastro-intestinal  form  behaves  like  an 

'  See  Cannon:  Loc.  cit. 

2  Patologia  dell  apparato  surrenale.     Milano.l'JO'J. 


256  THE  ENDOCRINOPATHIES 

acute  poisoning.  The  apoplectiform  resembles  a  cerebral  hemorrhage, 
but  autopsy  has  shown  no  cerebral  defect  but  suprarenal  hemorrhage. 

The  cases  are  extremely  difficult  of  diagnosis  and  are  rarities.  Less 
severe  types  may  be  met  with,  among  which  the  incorrigible  attacks 
of  vomiting  of  pregnancy  may  be  considered. 

Addison's  Disease. — This  is  a  more  chronic  type  due  to  more  or 
less  total  involvement. 

As  early  as  1855  this  disorder  was  first  described  by  Thomas  Addison 
whose  outline  practically  covered  the  essential  symptomatology.  It 
is  a  disorder  of  adult  life — thirty  to  forty  years.  Its  chief  features  are 
a  gradually  developing  asthenia,  with  arterial  hypotension.  There  is 
morning  nausea  or  vomiting,  lumbar  pains,  an  advancing  yellowish 
pigmentation  of  the  skin  and  mucous  membranes,  amyatrophy,  depres- 
sion, unwillingness  to  do  anything,  with  episodic  occurrence  of  myo- 
clonic, tetanoid  or  epileptiform  convulsions,  with  periodic  palsies, 
confusional  states,  delirium,  chronic  paranoid  ideas,  coma,  death. 
The  chief  lesion  found  is  tuberculosis  of  the  medulla  of  the  suprarenal 
glands.  The  more  complete  symptom  picture  may  be  consulted  in 
works  on  general  medicine. 

Partial  Hypoadrenalemias. — These  have  been  termed  the  abortive 
or  latent  types  of  Addison's  disease.  The  melanoderma  is  absent,  but 
the  other  symptoms  noted  are  observed.  Constitutional  hypoadrena- 
lemic  states  no  doubt  are  very  numerous  and  show  themselves  as 
rare  and  difficult  forms  of  lowered  vascular  tonus,  cardiac  instability, 
muscular  asthenia,  visceral  and  ligamentous  ptoses.  Sergent's  white 
line  is  a  very  frequent  symptom  in  many  hypoadrenalemias.  It  is 
not  invariable,  however,  and  severe  adrenal  defect  states  may  be  present 
wdthout  the  presence  of  the  Sergent  white  line.     (See  Examination.) 

Hyperadrenalemia. — A  group  of  genital-adrenal  syndromes  has  been 
isolated  by  a  number  of  observers."^  In  these  the  cortex  chiefly  of 
the  gland  has  been  involved,  either  as  a  simple  hypertrophy,  an  ade- 
nomatous hyperplasia,  benign  and  malignant  adenomata,  or  carcino- 
matous hypernephromata.  In  nearly  all  of  these  cases  there  has  been 
an  associated  series  of  atrophic  lesions  of  the  internal  genital  organs 
of  the  female.  The  uterus,  tubes  and  vagina  are  apt  to  be  small,  the 
ovaries  small,  usually  cystic,  the  Graafian  follicles  regressive  or  absent, 
with  atresia  of  the  primitive  follicles.  The  male  analogues  are  not 
yet  as  sharply  delimited. 

The  chief  clinical  forms  which  this  interrelationship  brings  about 
may  be  formulated  under  a  few  pronounced  trends,  with  a  medley 
of  intermediaries.  The  chief  trends  are  (1)  a  pseudohermaphroditism, 
(2)  suprarenal  virilism,  (3)  menstrual  and  (4)  obstetrical  forms. 

1.  A  Suprarenal  Pseudohermaphroditism. — ^These  are  feminine  her- 
maphroditic forms  externally  with  virile  secondary  male  sexual  char- 
acters. The  earliest  case  reported  was  by  Crecchio  in  1865,  of  a 
woman,  aged  fifty-two  years,  taken  to  be  a  man.    She  had  a  large  penis- 

'  Gallais:  Le  syndrome  geuito-surr ena) ,  1913. 


DISEASES  OF  THE  SUPRARENAL  BODY  257 

like  clitoris  with  iiA'pospadias,  no  scrotum  nor  testicles,  a  uterus  with 
two  tubes,  two  ovaries  without  a  trace  of  corpora  lutei,  and  an  enlarged 
and  voluminous  suprarenal.  She  had  been  markedly  asthenic,  dying 
in  a  syncopal  attack  with  vomiting  and  persistent  diarrhea.  This  is  a 
type  case  from  which  many  variations  in  the  individual  primary  and 
secondary  sex  character  developments  may  be  seen  in  other  patients 
with  this  syndrome.  The  usual  glandular  defect  is  a  simple  cortical 
hj'pertrophy  of  the  adrenal  and  the  anomaly  is  usually  embryonic  and 
the  sjTnptoms  develop  slowly  throughout  childhood  and  adolescence. 

2.  Suprarenal  Virilism. — In  this  trend  the  suprarenal  tumor  develops 
without  congenital  anomalies  of  the  genital  organs.  Here  the  second- 
ary sex  character  changes  are  predominant.  The  primary  sex  modi- 
fications are  more  functional,  sterility,  menstrual  irregularities,  than 
microscopically  anatomical.  The  vegetative  mechanism  of  the  ovary 
is  nevertheless  interfered  with.  Females  are  chiefly  involved.  The 
phases  of  development  vary,  but  there  is  usually  early  menstrual 
irregularity  or  amenorrhea.  Anorexia,  nausea  and  vomiting  are  not 
infrequent.  The  adolescent  girls  are  usually  very  fat,  and  there  is 
much  muscular  h^'pertension  and  restlessness.  The  girls  are  thought 
to  be  fine  athletic  specimens,  although  irritable,  nervous  and  at  times 
flighty.  They  tend  to  be  aggressive,  domineering  and  often  play  the 
man  role  with  their  schoolmates,  or  "crushes."  Vasomotor  crises  are 
frequent.  A  moustache  is  apt  to  develop  and  pigmentation  of  the 
Addisonian  type  may  show  itself.  This  stage  gradually  changes 
over  after  several  years  to  a  distinct  thinnmg  of  the  body  and  marked 
muscular  weakness.  Thoracic  pains  then  develop  and  in  the  more 
marked  cases  an  abdominal  tumor.  The  patients  are  apt  to  be 
depressed,  and  at  times  even  suicidal.  The  disease  may  stop  at  any 
stage  before  a  terminal  cachexia  with  asphyxia  and  con\Tilsions.  In 
young  girls  the  tumor  is  apt  to  develop  to  a  pronounced  degree  by 
eleven  or  fourteen  years  of  age. 

The  usual  tumor  is  a  benign  h}T)ernephroma,  sometimes  malignant. 

Another  trend  consists  of  the  "  infant  hercules'"  anomalies,  Avho  at 
the  ages  of  from  four  to  eleven  years  develop  genital  hair,  beards, 
general  h\7jertrichosis  and  markedly  older  skeletons.  Sometimes  the 
intelligence  is  precocious,  again  they  are  imbeciles. 

3.  A  menstrual  form  is  characterized  in  older  women,  twenty  to 
fifty  years,  by  persistent  and  inveterate  dysmenorrhea.  Adiposity 
is  apt  to  be  marked,  and  often  persistent,  sometimes  the  patients 
become  thin.  Pigmentation  may  develop,  naevi  are  frequent  and 
h^-pertrichosis  generalized.  These  women  often  pull  out  the  hair  or 
peroxide  their  moustaclies.  Hypertlnroid-like  sym])toms  are  fre- 
quent, ]>robal)ly  a  true  compensatory  eflort  to  make  good  the  adrenal 
loss.  Tumor  is  less  frequent,  but  cortical  iiyperplasia  usual.  Psycho- 
genic components  play  a  large  role  in  this  type. 

4.  The  obstetrical  for  III  is  a  minor  variant  of  the  former  trenfl.  The 
chief  anomaly'  here  is  the  false  pregnancy  syndrome  which  sometimes 
develops,  or  an  ectopic  pregnancy  with  its  grave  complications. 

17 


258  THE  ENDOCRINOPATHIES 

The  chief  differential  diagnostic  syndromes  are  hypophyseal  giantism, 
FrohHch's  s;sTidrome,  pineal  genital  anomalies,  thymic  hj^Dertrophies, 
miyxedema,  hereditary  trophedema,  adiposis  dolorosa  and  genito- 
dystrophic  gerodermia. 

Therapy. — The  therapeutic  indications  in  adrenal  disorders  are  still 
much  in  the  dark.  Many  tj^es  of  diversion,  of  interest-provoking 
stimuli,  of  pain-reducing  and  worry-removing  agents,  physical, 
pharmacodynamic  or  psychogenic,  are  all  more  or  less  empii'ically 
employed  for  the  transitory  asthenias  or  collapse  states  of  hypo- 
adrenalemias  of  mild  grade  chiefly  induced  by  emotional  disturbances. 

Chronic  asthenic  states  in  unconscious  phobic  cases  may  be  reached 
by  thoroughgoing  psychotherapy  along  psychoanalytic  lines. 

The  opotherapeutic  indications  are  also  crystallizing  out.  Adre- 
nalin itself,  seems  as  yet  to  be  a  disappointing  substance.  Far  better 
results  seem  to  be  obtained  by  the  use  of  pituitary  substance,  by 
thyroid  and  other  sympathetic  stimuli  than  by  the  use  of  adrenalin 
in  the  asthenic.  The  hypertensive  states  are  rendered  worse  by 
pituitary  and  thyroid.  Ovarian  therapy  is  indicated  in  certain  of 
the  surrenal-genital  syndromes,  especially  in  those  with  pronounced 
menstrual  disturbances.  Thymic  hj^erplasiae  and  adrenal  defect 
often  seem  to  be  related  and  suggest  that  thymic  sudden  death  is 
rather  a  suprarenal  defect  and  certain  surgical  shock  deaths  have 
a  related  pathology. 

Certain  chronic  Addisonian-like  types  are  much  benefited  by 
suprarenal  in  small  doses,  or  by  adrenalin  in  minute  doses  (0.004  to 
0.005  gm.).  In  the  chronic  capsular  cases  the  fresh  gland  itself 
seems  to  work  better  than  adrenalin.  In  the  present  state  of  opo- 
therapy each  patient  must  be  worked  out  separately  starting  with 
minute  doses  and  noting  the  effects  chiefly  on  the  pulse-rate,  blood- 
pressure,  Sergent's  white  line  and  the  asthenia. 

In  certain  hypertensive  states  with  high  blood-pressure,  due  to 
unconscious  factors  of  pathological  extroversion  (impatient,  busy 
people,  always  trying  to  do  twice  as  much  as  is  possible — chronic 
unconscious  sadism)  psychoanalysis  is  of  inestimable  service.  In  the 
exhaustive  states  of  acute  septic  processes,  acute  toxemias  (gas  gan- 
grene of  War  1914-1918)^  the  cortex  undergoes  marked  changes,  the 
medulla  less  so.  The  whole  gland  extract  should  be  used  as  a  stimu- 
lant in  such  prostrations  from  infections  with  other  signs  of  adrenal 
insufficiency. 

DISEASE  OF  THE  GONADAL  SYSTEMS. 

Genital  Syndromes. — Agenitalism,  Hypergenitalism  and  Hypogenital- 
ism.-— Genital  sjTidromes  arise  from  disorders  in  (1)  the  gonads  and 
(2)  the  interrenal  tissues  of  the  adrenal  cortex. 

1  Goormaghitigh :  Suprarenal  Capsule  and  Infectious  States,  Archives  de  med.  exp., 
1919,    xxviii,    p.    277. 

2  Consult  Harms:  Innere  Sekretion  der  Keimdrusen,  Fischer,  1914.  Tandler  u. 
Grosz:  Biologische  Grundlagen  d.  sekundjiren  Geschlechtscharaktere,  Berlin,  1913,  and 
the  monographs  mentioned,  particularly  Biedl. 


DISEASE  OF  THE  GONADAL  SYSTEMS  259 

Of  all  the  glands  of  internal  secretion  the  gonads  have  best  and 
earliest  been  known  to  possess  definite  control  of  metabolism.  The 
ancient  practice  of  castration  called  attention  early  to  this  intimate 
relationship. 

In  the  male  the  testis  is  formed  of  (1)  true  gametic  cells,  which 
develop  spermatozoa,  and  are  not  kno^^^l  to  possess  any  hormone 
activity,  (2)  the  interstitial  cells  of  Leydig  which  are  probably  the 
true  cells  of  internal  secretion. 

In  the  female  one  finds  (1)  the  Graafian  follicle  containing  the  o^ama 
and  (2)  interstitial  cells,  between  the  follicles,  which  closely  resemble 
those  of  the  testis.  Both  interstitial  cell  t^-pes  appear  to  be  modified 
gamete  cells  and  both  are  the  hormone  producers. 

This  interstitial  hormone  acts  as  a  connectmg  link  between  the  soma 
and  the  gonads  and  through  this  specific  action,  particularly  marked 
in  the  male,  exercises  a  direct  and  specific  stimulus  upon  the  somatic 
structure  of  the  body,  thus  increasing  growth  activity,  causing  definite 
lines  of  development,  varv'ing  in  the  sexes,  and  so  affectmg  the  whole 
muscle  and  nerve  metabolism  as  to  produce  profound  and  far-reaching 
alterations. 

The  gonads  are  supplied  both  by  autonomic  and  sympathetic  fibers. 
The  chief  genital  or  gonadal  sjTidromes  result  from  aplasias  or  mal- 
formations, giving  rise  to  various  hermaphroditic  syndromes;  from 
agenitalism  or  h}'pogenitalism  with  eunuchs,  eunuchoids,  and  their  many 
transitional  forms  and,  from  hj^Dergenitalism  with  the  syndromes  of 
dyshyperovarianism,  dyshyperdiastematy  and  chlorosis{l) .  The  genital 
like  the  other  sjudromes  are  usually  polyglandular. 

Hermaphroditism. — ^True  hermaphrodites  are  probably  non-existant 
so  far  as  the  male  sex  is  concerned.  True  cases  of  ovotestis  are 
extremely  rare.  Pseudohermaphroditism,  while  unusual,  is  never- 
theless not  infrequently  observed.  A  great  variety  of  findings  are 
recorded.  Females  with  enlarged  clitoris,  vaginal  cul-de-sac,  ovaries, 
uterus  and  bilateral  testes.  In  certain  patients  primary  and  secondary 
sexual  characters  correspond,  in  others  there  are  male  gonads  with 
female  hair  distribution,  pelvis  formation,  fatty  deposits,  high  voice 
and  enlarged  mammae.  It  is  highly  probable  that  the  adrenal 
interstitial  cells  play  an  important  role  in  these  various  admixtures  of 
hermaphroditic  primars'  and  secondary  traits. 

Agenitalism:  Eunuchs. — The  symptoms  vary  considerably,  depend- 
ing upon  the  age  of  the  individual  when  the  (usually  surgical)  loss  of 
the  gonads  takes  place.  In  early  loss  in  the  male  (castration  before 
puberty,  destructive  orchitides)  the  penis,  prostate  and  seminal  vesicles 
remain  small,  erotic  desire  fails  to  show  itself  and  potency  is  lost.  In 
the  female  a  similar  failure  of  development  takes  place.  The  girl  is 
apt  to  grow  tall,  boyish  in  type,  with  infantile  secondary  characters. 
Later  loss,  after  puberty,  tends  to  increase  the  size  of  the  skeleton — 
a  tall,  thin  type  and  a  short,  fat,  dumj^y  type  with  broad  hij^s,  female 
fat  distribution  on  the  breasts,  buttocks  and  iliac  crests.  The  lower 
extremities  develop  disproportionately  more  than  the  upper,  or  vice 


260 


THE  ENDOCRINOPATHIES 


versa.  The  head  is  flattened  behind,  the  sella  turcica  widened,  the 
superciliary  ridge  is  apt  to  be  prominent.  The  skin  is  usually  smooth 
cool,  marble-like,  poor  in  pigment  and  color;  the  hair  of  the  head  usually 
thick  while  that  of  the  face  is  absent  or  only  downy,  that  of  the  pubes 
follows  the  female  type  of  distribution — horizontal.  Small  thyroids, 
thymus,  larynx  and  wide  pelvis  are  the  rule. 

The  average  castrate  is  apathetic,  with  shambling  gait,  bent  in  his 
posture  and  a  sleepy  or  indolent  mental  attitude.     The  face  is  usually 

fat  with  puft'y  eyelids.  The  voice  is  high 
and  thin.  Erotic  desires  are  not  always  ab- 
sent nor  is  intercourse  impossible,  even 
though  the  penis  is  apt  to  be  small. 

The  female — artificial  menopause — tends 
to  grow  stout  and  irritable,  autonomic  tonus 
is  lowered,  ptoses  are  frequent,  vasomotor 
instability,  with  hot  and  cold  flashes,  with 
darting,  jumping  pains,  anxiety,  nervousness 
and  flightiness  develop.  Alcohol,  bromides 
and  other  drug  habits  not  infrequently  de- 
velop as  attempts  at  relief  of  the  annoying 
symptoms.  The  craze  for  ovariectomy  as  a 
cure-all  having  spent  its  force,  fewer  of  these 
cases  are  seen. 

FAinuchoids.  —  These  result  from  less 
marked  disturbance  in  the  development  of 
the  gonads.  A  great  variation  also  exists 
here  following  various  accidents  to  the  testes 
and  ovaries,  inflammations,  tumors,  infec- 
tions (tuberculosis,  parotitis,  gonorrhea),  etc. 
Two  trends  are  prominent,  the  tall  and  the 
fat  types.  The  changes  are  those  found  in 
eunuchs,  although  for  the  most  part  less  pro- 
nounced or  monosymptomaticin  their  appear- 
ance. Every  gradiant  may  be  encountered, 
hence  the  difficulty  in  describing  a  very  vari- 
able mosaic.  Cryptorchism  is  a  frequent 
complication. 

The  skin  changes  are  present  in  both  forms. 
It  is  usually  thin ,  pale,  anemic,  and  apt  to  be 
slightly  waxy ;  fine  lines  or  wrinkles  develop 
readily,  giving  an  appearance  of  old  age  with  youth.  The  hair  anom- 
alies are  as  already  discussed.  Atrichia,  irregularities  in  development, 
lanugo  substitutions,  alopecias  are  not  infrequent.  The  eyelids  and 
eyebrows  are  sparse.  Single  long  hairs  develop  on  the  chin,  the 
moustache  is  scanty  and  wiry.  Sterility  is  the  usual  result  in  both 
cases.  Many  of  these  patients  are  potent,  some  even  hyperexcitable, 
but  the  reverse  is  the  rule.  Meritorious  psychical  achievements  are 
frequently  found,  but  as  a  rule  the  mental  activities  are  below  the  average. 


Fig.  121.  — •  Eunuchoid. 
Hyporchismus  in  boy.  (A. 
Josefson.) 


DISEASE  OF  THE  GONADAL  SYSTEMS 


261 


A  late  type  of  eunuchoidism  following  disturbances  of  the  testes 
(syphilis,  traiuna,  .r-ray,  alcohol,  gonorrhea,  exposure,  tuberculosis, 
etc.)  develops  somewhat  similar  signs. 

hifantilism. — ^^'arious  types  have  been  described  which  have  been 
keenly  discussed.  More  than  in  any  other  group  perhaps  does  the 
polyglandular  hj^othesis  seem  necessary  to  comprehend  the  many 
major  and  minor  variations.  Lasegue  gave  the  name  to  the  group. 
Lorain  then  described  his  types  as:  (1)  Those  small,  graceful  and 
finely  built,  (2)  those  that  remained  more  or  less  infantile  and  (3)  a 
feminine  type  with  broad  hips,  small  genitals,  scanty  facial  hair,  long 
and  thin  hair  of  the  head,  large  breasts  and  prolonged  primary  denti- 
tion— failure  of  second  teeth,  etc.  Brissaud  then  showed  that  certain 
of  Lorain's  types  were  myxedematous  and  Hertoghe  took  them  out  of 
the  gonadal  group  and  classed  them  as  thjTcopathies.  Later  students 
pointed  out  hj-pophyseal  anomalies,  others  pancreatic,  still  others 
implicated  the  spleen.  Hj^otheses  ran  riot  throughout  this  entire 
field  but  sound  relationships  are  slowly  crystallizing  out. 


Fig.   122. — Hypo-ovarismus.     (Josefson,  Deut.  Ztschr.  f.  Nervenh.,  1910.) 


Dysgenitalism. — Chlorosis. — Without  committing  oneself  to  the  defi- 
nite position  that  chlorosis  is  an  anomaly  due  to  defective  genital  hor- 
mone activity  yet  the  evidence  now  seems  to  point  in  that  direction. 
Its  occurrence  in  girls  at  the  time  of  puberty  is  one  of  the  factors  accen- 
tuating this  relationship.  Von  Noorden  was  among  the  first  to  ascribe 
chlorosis  to  a  defective  secretion  of  the  ovarian  interstitial  cells.  Li 
this  syndrome  other  endocrinous  glands,  notably  the  thyroid,  show 
alterations. 


262  THE  ENDOCRINOPATHIES 

Symptoms. — The  symptoms  are  not  recorded  here,  as  they  are  better 
fomid  in  works  on  general  medicine  and  the  vegetative  paths  involved 
in  control  of  the  blood-making  organs  and  of  the  tonus  tensions  of  the 
bloodvessels  and  the  blood  itself  have  not  been  sufficiently  worked  out 
to  be  stated  definitely  in  this  place.  The  chief  factor  to  be  emphasized 
here  is  that  chlorosis  is  largely  conditioned  by  an  excessive  plasma 
plethora  in  the  bloodvessels.  The  bone  marrow  is  overstimulated  to 
create  an  increase  of  red  cells,  which  because  of  the  great  dilution  from 
the  surplus  of  plasma  volume  are  correspondingly  poor  in  hemoglobin. 
It  may  be  assumed  provisionally  that  this  plasma  retention  is  made 
possible  by  diminished  transudability  of  the  vascular  walls  (sympathico- 
tonic) and  is  an  opposing  picture  to  that  which  is  seen  in  the  disordered 
transudability  in  the  various  types  of  angioneurotic  edema  which  have 
been  discussed  (vagotonic).     (See  Blood  Syndromes,  p.  196.) 

Dysgonadal  Syndromes. —  Hyperovarian  Signs. — Habit  of  precocious 
sexuality.  Precocious  intelligence  active,  bright,  gay  and  jolly.  Pre- 
cocious puberty.  Menorrhagia,  metrorrhagias,  amenorrhea  of  local 
lesion.  Congestive  dysmenorrheas,  nervous  dysmenorrheas.  Harmony 
of  form,  good  constitution.  Terminal  anemic  paleness.  Thyroid  nor- 
mal, with  inconstancy.  Marked  fecundity,  menopause  retarded. 
Nervous  type.     Constipation  not  marked. 

Hypo-ovarian  Signs. — Retarded  habitus,  generalized  or  localized. 
Various  types  of  infantilism,  morons.  Late  menstruation.  Habitual 
amenorrhea,  metrorrhagia.  Nervous  dysmenorrhea  from  flexion  or 
other  malformation.  Pale,  puffy,  pseudomyxedematous,  adiposity, 
acrocyanosis,  cold  extremities.  Thyroid  enlarged,  mild  exophthalmic 
signs,  infecundity  more  often,  early  menopause.  Nervous;  con- 
stipation marked. 

Treatment. — ^\^arious  types  of  testicular  and  ovarian  therapy  have 
been  used.  In  certain  hypo-ovarian  cases  moderate  doses  of  dried 
ovary  (0.10-0.20  gm.)  twice  a  day,  over  a  fairly  prolonged  period  have 
been  reported  to  be  of  some  service.  The  glycerin  extract  hypoder- 
mically  has  been  abandoned.  Corpus  luteum  preparations  are  now 
being  extensively  employed  with  results  as  yet  not  readilj^  interpreted. 
Combinations  with  thyroid,  pituitary,  and  suprarenal  ex:tracts  are 
recommended.  Thus  in  simple  ovarian  insufficiency  ovarian  cachets 
may  be  employed  solely  as  substitutive.  For  the  headaches  and  the 
hot  flashes  pituitary  or  adrenalin  preparations  may  be  used  as  regula- 
tory or  as  hormostimulatory.  Suprarenal  therapy  is  contra-indicated  in 
the  hypertensive  states,  whereas  in  myasthenic,  constipated,  and  wake- 
ful nervous  patients  with  lumbar  pains,  headaches,  digestive  disturb- 
ances and  other  dysovarian  signs  it  has  proved  serviceable. 

Opotherapy  for  gonadal  syndromes  is  still  in  an  experimental  period 
of  development.  Personal  experiences  point  to  it  as  largely  sug- 
gestive, but  in  certain  carefully  analyzed  cases  excellent  results  have 
seemed  to  depend  directly  upon  the  opotherapy.  The  indiscriminate 
use  of  testicular  extracts  (spermin)  has  not  added  any  permanent  gain 
in  therapy.     In   obstetrical   and   gynecological  work  the   action  of 


DISEASE  OF  THE  GONADAL  SYSTEMS 


263 


pituitrin  upon  the  iinstriped  fibers  of  the  uterus  and  bladder  is  very 
marked  and  can  be  made  serviceable. 

Ovarian  and  thyroid  extracts  may  be  added  with  advantage  to  iron 
and  arsenic  in  the  treatment  of  chlorosis. 

There  is  a  frequent  tj'pe  of  compensatory  hyperadrenalema  follow- 
ing the  menopause  which  is  often  very  advantageously  handled  by 
moderate  doses  of  ovarian  substance.  This  h\-peradrenalemia  is  often 
a  producer  of  marked  arteriosclerotic  states,  atheroma,  headaches, 
angina  and  even  cerebral  hemorrhage.  Its  unconscious  psychic  accom- 
paniments are  greatly  in  need  of  careful  investigations.^ 


Fig.   123. — Hyporchi-nm.-.     Man.     Eunuchoid.     (A.  Josefson.) 

Status  Thjnnoljrmphaticus. — The  thymus  has  very  close  relation- 
ships to  the  gonads,  and  it  has  been  thought  that  the  th^Tnus  and  testes 
are  reciprocally  acting  organs.  This  does  not  seem  to  be  true  for  the 
thymus  and  the  ovaries. 

Certain  individuals  have  excess  of  thymus  lymph  tissue  throughout 
the  body.  In  recent  years  the  researches  of  Bartel,^  Wiesel  and  A. 
Paltauf  have  shown  that  this  condition  is  very  frequent.     Notwith- 


1  Maranon:   The  Critical  Age,  Madrid,  1919. 

2  Status  thymicolymphaticus  u.  Status  Hypoplasticus,  Deuticke,  1912.  Symmer?, 
Am.  .lour.  Med.  Sc.  1918,  vol.  clvi.  Emerson,  Tr.  Int.  Cong.  Med.,  1914.  Ewing:  Jour. 
Am.  Med.  Assn.,  November  9,  1918,  vol.  Ixxi, 


264 


THE  ENDOCRINOPATHIES 


standing  the  fact  that  a  pathological  diagnosis  postmortem  is  easily 
arrived  at  the  clinical  diagnosis  during  life  presents  many  difficulties. 
This  latter  is  largely  due  to  the  circumstance  that  the  diseased  organs 
are  difficult  to  examine  and,  furthermore,  the  signs  of  defective  develop- 
ment which  result  from  the  condition  are  often  very  slight. 

The  recognition  of  status  thymolymphaticus  often  requires  exhaust- 
ive chemical,  physical,  .T-ray,  and  other  forms  of  examination.  Such 
recognition  is  highly  important,  however,  since  these  individuals,  if 
they  may  be  grouped,  are  prone  to  react  very  markedly  to  anesthetics, 
bodily  shocks,  infectious  diseases,  and  to  drugs,  particularly  salvarsan, 
sera,  and  mercury.  A  large  medley  of  conditions  accompanying  and 
partly  due  to  status  thymolymphaticus,  have  been  described.  These 
may  be  briefly  summarized  as  follows: 

1.  General  Signs. — As  a  rule,  the  length  of  the  body  exceeds  the 
stretch  between  extended  finger-tips.  The  length  of  the  upper  part 
of  the  body  and  of  the  extremities  is  above  the  average.  The  arrange- 
ment of  fatty  tissue  tends  to  make  the  male  resemble  the  female  type, 
and  vice  versa.  The  mental  status  is  inclined  toward  the  infantile. 
Alcohol  resistance  is  very  slight. 


Fig.   124. — Pubertas  preccjx  (hyijeruifljisnuisj   ek'\eii-ycar-old  boy.      (A.  Josefson.) 

2.  Face. — The  under  jaw  and  the  mastoid  processes  are  under- 
developed and  the  former  results  in  faulty  bite,  anomalies  of  dentition 
by  displacement  and  by  crowding.  The  palatal  arch  is  high;  the 
tonsils  and  tongue  papillae  are  increased  in  size.  The  epiglottis  is 
inclined  to  be  infantile  in  type.  Epicanthus,  eccentric  pupils,  irregu- 
larly pigmented  irides,  adherent  ear  lobules  and  narrow  external 
auditory  meatus  may  be  present.     "Peaches  and  cream"  complexion. 

3.  Neck. — The  thyroid,  cervical,  and  other  glands  are  enlarged. 

4.  Skeleton. — The  thorax  is  long  and  narrow.  Cervical  floating 
ribs  are  present.  Compensatory  lordosis  of  the  spine  is  lacking. 
The  scapulae  are  wing-shaped.     The  pelvis  develops  heterosexually; 


DISEASE  OF  THE  GONADAL  SYSTEMS  265 

the  sacrum  is  small,  the  pubis  high.  H^'pe^dactyly,  flat-foot,  and 
h^perextension  of  the  elbows  may  be  looked  for. 

5.  Hair. — ^Axillary  and  pubic  hair  are  diminished;  the  extremities 
may  be  hairy. 

6  The  thymus  is  enlarged,  the  breasts  resemble  those  of  the  opposite 
sex;  polymastia  may  be  observed.  The  aorta  is  narrow,  the  heart 
small,  the  blood-pressure  low.  Palpitation  is  frequent  and  there  is 
cardiac  dilatation  with  weakness. 

7.  In  the  abdomen  ptoses  are  frequent.  The  jugulopubic  distance 
is  increased,  the  abdominal  circumference  diminished.  The  spleen  is 
enlarged,  the  kidneys  prolapsed.  There  is  a  tendency  to  orthostatic 
albuminuria  and  to  alimentary  gh'cosuria. 

8.  The  blood  picture  shows  a  neutropenia,  lymphocytosis,  and 
eosinophilia. 

9.  The  genital  anomalies  are  in  the  nature  of  cr^-ptorchism,  hypo- 
plasia, disturbances  of  menstruation  and  secondary  sexual  characters 
of  the  opposite  sex. 

10.  There  is  a  marked  disposition  to  other  disease  and  usually  a 
tendenc}^  to  an  increase  in  the  severity  of  the  disorder.  Thus,  tuber- 
culosis shows  more  often  in  other  organs  than  the  lungs;  infectious 
diseases  of  childhood  are  severe;  there  is  a  tendency  to  tetany,  glio- 
mata,  sjTingoses,  hydrocephalus,  tabes,  paresis,  mj'asthenia.  Dia- 
betes, excessive  fat  and  gout  occur.  Pernicious  anemia,  leukemia 
and  chlorosis,  exophthalmic  goiter,  Addison's  disease,  osteomalacia, 
nephritis,  eclampsia,  asthma,  infantile  emphysema,  eczema,  heman- 
giomata,  appendicitis  and  tumor  formation  are  among  other  accom- 
panying disorders. 

Observation  of  many  cases  of  status  thymoljTiiphaticus  shows  that 
little  weight  is  to  be  given  to  the  occurrence  of  isolated  symptoms. 
The  diagnosis  consists  in  the  accumulation  of  the  anomalies.  The 
dift'erences  in  body  dimensions  are  of  universal  importance,  whereas 
the  increase  in  the  tongue  follicles  and  the  infantile  character  of  the 
epiglottis  and  its  frequent  omega  shape,  are  more  characteristic. 
Genital  hypoplasias  are  frequently  associated  with  eosinophilia  and 
lymphocytosis  is  to  be  expected. 

From  the  standpoint  of  endocrinology  the  interrelationships  between 
thymus  loss,  deficiency,  persistence  and  hyperactivity  and  the  influence 
on  other  endocrinous  organs  is  of  great  interest.  As  yet  there  are 
thousands  of  observations  but  they  tend  to  lose  themselves  in  a  maze 
of  confusion  when  one  would  attempt  to  outline  definite  syndromes. 
Timme  has  outlined  a  thymus,  adrenal,  pituitary  combination,^  which 
is  frequently  observed  and  readily  recognized.  The  main  features 
are  fatigability,  low  blood-pressure,  headache  and  growth  anomalies. 
The  syndrome  starts  off  before  puberty  with  thymic  persistence  with 
growi:h  of  the  long  bones  and  many  of  the  characters  of  the  status 
hypoplasticus  of  Bartels,  here  partly  enumerated.    The  second  stage 

1  Timme,  Endocrinology,  July-September,  1918. 


266  THE  E  N  DOC  RI  NOP  AT  HIES 

begins  at  puberty.  The  myasthenia  is  more  pronomiced.  The  sella 
is  usually  small  and  when  an  attempt  at  pituitary  compensation  is 
made  by  hypertrophy  headaches  appear,  especially  if  the  sella  is  cir- 
cumscribed. This  hyperf unction  of  the  pituitary,  if  possible,  provokes 
further  bony  growth  and  giantism  or  acromegalic  features  may 
develop  and  the  headache  becomes  definite.  Erosion  of  the  clinoids 
now  may  begin.  In  from  three  to  ten  years  the  complete  constitutional 
disorder  is  developed.  With  large  sella  turcica,  acromegalic  features, 
marked  fatigability,  headaches  gone,  but  mental  defects  and  a  tendency 
to  convulsive  seizures.  Intercurrent  disease  usually  causes  an  early 
death. 

There  is  a  marked  antagonism  between  the  thymus  and  the  thyroid 
gland. ^  Feeding  thymus  causes  atrophy  of  the  thyroid  and  para- 
thyroid^  and  h^^perf unction  of  the  thyroid  causes  atrophy  of  the  thymus. 
Uhlenhuth  has  fed  thymus  to  amblystoma  larvse  and  produced  typical 
tetany  cramps,  the  cause  for  which  he  interprets  as  a  specific  toxin,  but 
which  we  believe  is  due  to  parathyroid  atrophy,  from  thymic  hj^per- 
activity,  although  these  larvse  have  no  well-defined  parathyroids.  Dustin 
is  disposed  to  see  in  the  thymus  a  regulatory  structuralization  for 
nucleoproteid  synthesis  but  the  precise  mechanism  is  not  certain.  In 
certain  vagotonic  exophthalmic  goiter  cases,  there  is  often  present 
a  persisted  hyperactive  thymus,  with  hypo-adrenalemia.^  These  are 
presumably  those  dangerous  thyroid  cases  in  which  sudden  death 
results  from  operation. 

The  whole  problem  of  thymus  feeding  is  yet  experimental.  Certain 
cases  of  myasthenia  gravis  have  been  helped,  others  not. 

DISEASES  OF  THE  PANCREAS. 

Pancreatic  Syndromes. — Falta  holds  that  the  chief  activity  of  the 
pancreas  is  subserved  through  an  assimilatory  hormone,  which  controls 
the  glycogenesis  of  the  liver  and  muscles.  In  mild  grades  of  pancreatic 
insufficiency  disturbances  of  carbohydrate  metabolism  appear  only 
when  great  demands  are  made  upon  the  glycogenic  function  of  the 
liver  through  excessive  alimentary  carbohydrate  intake.  In  graver 
disturbances  in  addition  to  the  modification  of  anabolism  a  high  grade 
of  catabolic  destruction  takes  place  with  a  failure  to  form  higher  and 
lower  fatty  acids  (ketonuria). 

Pancreatic  syndromes  occur  as  a  result  of  gross  anatomical  disorder, 
acute  pancreatic  hemorrhage,  and  chronic  pancreatitis,  sj^jhilitic 
pancreatitis,  etc. ;  all  of  which  are  discussed  fully  in  works  on  internal 
medicine.  Those  of  interest  here,  however,  are  diabetes  mellitus 
(true  diabetes),  and  pancreatic  infantilism,  all  closely  related  to  dis- 
order of  the  glandular  tissues  of  the  pancreas- — its  internal  secretory 
part.    Although  the  pancreas  seems  primarily  a  digestive  gland  it  also 

1  Dustin  et  Zunz:    Jour,  de  Phys.  et.  d.  Path.,  1918. 

2  Uhlenhuth:    Jour.  Gen.  Physiol.,  1918,  No.  1,  vol.  i. 

'  Rose:     Le  thymus  et  la  maladie  de  Basedow,  Sem.  Med.,  January  21,  1914. 


DISEASES  OF  THE  MUSCLES 


267 


produces  an  internal  secretion  which  holds  in  check  the  mobilization  of 
sugar,  thus  acting  in  a  balanced  relation  with  the  thyroid  and  hypo- 
physeal secretions  which  tend  to  facilitate  the  use  of  sugar  as  an  ener- 
gizing material  by  the  muscles.  This  mobilization  may  be  considered 
to  be  made  effective  by  the  terminals  of  the  vegetative  nervous  system 
in  the  liver  cells;  just  how  it  is  not  known. '^  Pancreatic  infantilism 
shows  polyglandular  disturbances  through  arrested  bodily  growth  and 
arrested  sexual  development.  Vagotonic  symptoms  such  as  excessive 
diarrhea  and  flatulent  distention  are  also  present. 


Fig.  125. — Scheme  of  innervation  of  the  liver,  spleen,  and  kidney.  nX,  nucleus  of  the 
vagus;  X,  vagus;  nv,  vasomotor  nucleus  in  medulla;  s,  sympathetic;  re,  rami  commu- 
nicans;  spl,  splanchnic  nerve;  ps,  solar  plexus;  gs,  semilunar  ganglion;  spl,  spleen. 
(Bechterew.) 

DISEASES  OF  THE  MUSCLES. 

Muscle  Syndromes. — Myasthenia  Gravis. — The  clinical  position  of 
this  disorder  is  very  imcertain.  By  some  it  is  to  be  regarded  as  a 
contrast  picture  to  tetany  and  due  to  vegetative  nervous  disturbance 

I  Eiger:    Zeit.  f.  Biologic.,  vol.  Ixvi,  p.  229. 


268  THE  ENDOCRINOPATHIES 

conditioned  in  part  by  a  polyglandular  endocrinopathy.  It  has  of 
late  been  shown  that  the  striped  muscular  system  is  provided  with 
vegetative  nerve  fibers  which  undoubtedly  regulate  the  muscular 
metabolism.    By  others  it  is  grouped  with  the  muscular  atrophies. 

The  disorder  is  infrequent.  It  was  separated  from  the  progressive 
bulbar  palsies  of  organic  nature  by  Erb  (1878)  and  later  studied  by 
Oppenheim  (1887),  who  termed  it  a  myasthenic  paralysis  without 
anatomical  foundation.  In  1891  Jolly  described  the  characteristic 
electrical  reactions  occurring  in  the  muscles,  termed. the  myasthenic 
reaction.^ 

The  early  symptoms  which  usually  come  on  between  fifteen  and 
thirty  years  of  age,  usually  involve  the  facial  muscles,  particularly 
those  of  the  upper  lid,  causing  ptosis.  Diplopia  from  paresis  of  an 
ocular  muscle  also  may  be  an  initial  symptom.  The  two  often  occur 
together  (asthenic  ophthalmoplegia) .  The  patients  note  the  beginning 
fatigue  of  the  muscles,  which  perhaps  intact  in  the  morning  on  awaken- 
ing, show  fatigue  signs  at  night.  This  muscular  asthenia  then  pro- 
gresses slowly  to  distinct  paresis.  Other  cranial  nerve  innervations 
then  show  a  similar  asthenia.  Difficulties  in  chewing  or  of  swallowing, 
or  of  speaking  develop.  The  muscles  of  the  neck  may  also  be  involved. 
Whatever  group  is  involved  the  chief  features  is  the  great  fatigue  which 
develops  very  rapidly  upon  the  use  of  the  muscles. 

Any  muscle  or  muscle  group  of  the  body  may  be  affected.  Dyspnea 
and  tachycardia  are  among  the  rarities  of  implication  of  the  respira- 
tory and  cardiac  muscles.  Sensory  disturbances  are  not  characteristic. 
Pains  may  occur. 

Leukocytosis  is  usually  present.  The  reflexes  are  not  implicated. 
In  some  instances  fatigue  of  the  tendon  reflexes  has  been  recorded. 

The  chief  feature  is  the  rapidly  developing  fatigue  of  the  muscle. 
This  is  best  demonstrated  by  faradic  stimuli.  These  cause  a  rapid 
loss  in  the  excitability  of  the  muscle  until  it  no  longer  reacts  to  the 
intermittent  faradic  current.  Hoffmann  has  shown  that  this  is  largely 
influenced  by  the  rate  of  the  interruptions.  With  seventy  interrup- 
tions per  second  the  myasthenic  reaction  develops  promptly,  with 
fifteen  it  does  not.  Continuous  faradic  stimulation  produced  a  similar 
myasthenic  fatigue  curve.  This  myasthenic  reaction  seems  to  separate 
the  disorder  from  other  forms  of  muscular  fatigue  such  as  occur  in 
bulbar  palsy,  medullary  syphilis,  multiple  sclerosis,  Addison's  disease, 
exophthalmic  goiter  and  the  fatigue  of  intermittent  claudication. 
There  are  certain  analogies  with  this  last  disorder  which  are  not  yet 
cleared  up. 

Atrophies  develop  in  the  affected  muscles  but  there  are  no  definite 
indications  of  the  reaction  of  degeneration.  Certain  transitional 
cases  which  show  relationships  to  distinct  organic  (nuclear)  cases  may 
evidence  electrical  changes  approaching  R.  D.  Fibrillary  twitches  in 
the  affected  muscles  are  not  the  rule,  but  they  have  been  observed. 

1  Oppenheim:  Die  myasthenische  Paralyse,  1901.  Starr:  Jour.  Nerv.  and  Mental 
Diseases,  1912. 


DISEASES  OF  THE  MUSCLES  269 

Myasthenia  gravis  runs  a  chronic  course  with  at  times  marked 
remissions.  It  has  been  known  to  develop  rapidly  in  three  or  four 
months  with  fatal  issue  in  from  one  to  three  years  and,  on  the  other 
hand,  it  has  been  known  to  extend  over  fifteen  to  twenty  3'ears.  The 
outcome  is  usually  fatal,  but  certain  cases  cease  to  progress. 

Little  is  known  of  the  underlying  causes.  Status  thymicolymphati- 
cus is  frequent.  Many  cases  show  interrelated  thymus,  parathyroid 
and  adrenal  compensating  effort  syndromes  which  do  not  seem  to 
overcome  persisting  localized  vagotonus  of  the  sacroplastic  substance. 
Nothing  is  known  concerning  the  unconscious  psychical  states.  One 
patient  examined  showed  highly  developed  father,  money  and  sadistic 
complexes.  Father  injustice,  relative  to  money  and  ineffective  fighting, 
was  a  constant  dream  symptom.  A  fighting  dream  was  followed  by 
grave  myasthenic  signs. 

The  pathological  lesions  are  not  constant.  In  the  greater  number 
of  cases  the  muscles  are  swollen,  edematous  and  infiltrated  with  lym- 
phoid cells.  These  changes  have  not  been  interpreted.  It  is  possible 
that  they  are  edemas  due  to  disturbance  of  the  vegetative  nervous 
system  control,  in  which  case  myasthenia  gravis  is  to  be  allied  with 
the  circumscribed  edemas.  To  know  this  does  not  help  very  much, 
but  it  does  indicate  that  search  must  be  directed  toward  all  causes  for 
vegetative  nervous  system  disturbance,  toxic,  endocrine  and  psychic. 

The  first  important  therapeutic  agent  is  rest;  absolute  and  pro- 
longed. The  second  is  psychotherapy.  Organotherapy  has  been 
tried,  with  as  yet  little  results,  but  it  probably  has  been  entirely  too 
empirically  applied.  Careful  attention  should  be  given  to  a  complete 
survey  of  the  functions  of  all  of  the  endocrinous  glands,  and  if  a  lack 
of  balance  be  found  an  attempt  should  be  made  to  restore  the  balance. 
Artificial  feeding  is  at  times  necessary.  Atropine  has  been  of  service 
occasionally  as  has  also  calcium.  Alcoholic  preparations  are  to  be 
avoided,  as  are  also  mechanical  forms  of  stimuli,  particularly  severe 
massage. 

Thomsen's  Disease.^ — Myotonia  Congenita.^ — This  is  a  very  rare  dis- 
order first  described  by  J.  Thomsen  in  1876.  Its  relationships  to  other 
nervous  diseases  is  very  obscure.  It  is  hereditary,  and  is  probably 
conditioned  by  a  constitutionally  inferior  thoracic  autonomic  control 
of  the  muscle  metabolism. ^  This  results  in  an  increased  threshold  of 
the  synapse  preventing  immediate  passage  of  the  voluntary  stimulus. 
This  is  often  associated  in  the  affected  families  with  other  signs  of 
inferiority;  neuroses,  psychoses,  tetany,  etc. 

The  chief  anomaly  is  one  affecting  the  muscles.  At  the  beginning 
of  any  voluntary  movement  the  patient  finds  it  difficult  to  overcome 
a  muscle  h\pertonus.  This  makes  the  muscles  stiff  and  unyielding. 
After  repeated  efforts  the  resistance  gradually  disappears  and  in  a 
few  minutes  or  more  the  muscular  activity  becomes  normal.     This 

1  Thomsen:  Ardiiv  f.  Psychiatrie,  1892.  Koch:  Ueber  Thoniseusche  Krankheit, 
Leipzig,  1914. 

2  S.  de  Boer:  Zeitschrift  f.  Biologic,  1914,  Ixv. 


270 


THE  ENDOCRINOPATHIES 


limbering-up  effect  is  lost  after  a  cessation  of  the  movements.  Any 
group  of  muscles  may  be  affected,  but  the  lower  extremities  are 
oftenest  involved.  This  makes  the  beginning  of  walking  difficult.  In 
the  upper  extremities  a  similar  condition  makes  manual  movement 
difficult.  A  patient  cannot  readily  loosen  his  grasp  of  an  object. 
Talking  and  eating,  etc.,  may  be  similarly  affected.     Changing  the 


Fig.   126. — Myatonia  atrophica.     Note  dropped  feet,  atrophy  of  hand  muscles,  and 

facies.     (Abrahamson.) 

tempo  of  a  movement  increases  the  difficulty  and  emotional  stimuli 
invariably  augment  the  stiffness  and  awkwardness.  Mechanical 
stimuli  cause  welts  to  appear  which  subside  slowly.  Atj-pical  cases 
are  reported,  in  some  of  which  the  disorder  appears  intermittently. 
(Compare  with  periodic  paralysis  also  paramyotonia  congenita  of 
Eulenburg.^ 

'  Jendrassik:  Lewandowsky  Handbuch,  vol.  ii. 


DISEASES  OF   THE  MUSCLES  271 

The  pathological  changes  are  slight.  Muscle-cell  hypertrophy, 
analogous  to  that  seen  in  myasthenia  gravis,  is  described. 

The  disorder  begins  early,  is  very  chronic,  is  not  fatal  in  itself,  nor 
does  it  seem  to  get  well  spontaneously. 

Xo  therapy  has  been  shown  to  be  effective.  Strychnine  is  tempo- 
rarily valuable.  If  the  present  h^^Dothesis  is  of  value  some  result 
should  follow  from  polyglandular  therapy,  particularly  from  the  use 
of  such  substances  as  influence  the  bivalent  kations,  Ca,  Mg,  etc.,  to 
regulate  the  electrical  resistances  in  the  motor  synapses.^ 

Myatonia  Atrophica. — This  rare  disorder-  is  possibly  a  definite  dis- 
ease entity  or  a  variant  of  Thomsen's  disease,  as  Pelz  (1907)  first 
announced.  It  is  characterized  by  late  onset,  twenty  to  thirty  years, 
limitation  of  myotonic  reaction  chiefly  to  the  closing  of  the  fist,  the 
tongue  movements,  myopathic  facies,  atrophy  of  jaw  muscles,  sterno- 
cleidomastoid, extensors  of  arm,  especially  supinator  longus,  atrophy  of 
peronei,  loss  of  tendon  reflexes  (late)  with  ataxia,  cataract,  loss  of  hair, 
atrophy  of  testicles,  impotency  or  frigidity,  increased  reactivity  to 
pilocarpine,  vasomotor  disturbances,  increased  tears  and  saliva,  and 
general  emaciation. 

It  is  probably  a  vegetative  nerve  disorder,  showing  a  medley  of 
sympathicotonic  and  vagotonic  signs  (due  to  an  hereditary  defect  in 
the  vegetative  nervous  system)  which  causes  muscular  dystrophies  to 
develop  on  an  endocrinopathic  basis. 

The  Muscular  Dystrophies  or  Myopathies. 

This  very  large  and  extremely  motley  group  has  also  been  built 
up  of  a  variety  of  forms  since  Duchenne,^  in  1849,  first  described  the 
fatty  pseudohA-pertrophies,  and  later,  in  1868,  spoke  of  them  as  myo- 
scleroses.  Leyden^  (1876)  and  Mobius^  (1879)  described  certain  heredi- 
tary forms,  while  Erb,^  in  1883,  first  brought  some  order  in  the  con- 
fusion of  the  atrophies  and  dystrophies  by  showing  that  in  certain 
forms  the  lesion  was  predominantly  muscular  and  not  nervous.  He 
made  the  first  practical  synthesis.  Landouzy  and  Dejerine,^  in  1884, 
described  their  well-known  form,  and  separated  it  from  Erb's  juvenile 
type.  Since  that  time  the  group  has  been  better  unified,  its  limits 
better  recognized,  and  the  various  forms  within  it  more  thoroughly 
studied.^ 

The  myopathies  make  a  fairly  consistent  group,  although  the  forms 
may  not  resemble  one  another  clinically  at  dift'erent  periods  of  their 
development,  yet  they  have  a  number  of  common  factors. 

'  Johnson  and  Marshall:  Quart.  Jour.  Med.,  1915. 

2  Batten  and  Gibb:  Brain,  1909.  Curschmann:  Dcutsch.  Ztschr.  f.  Xervcnheilk,  45. 
Hauptmann:  Ibid.,  55.    Higier:  Zeit.  f.  d.  g.  N.  u.  P.,  April,  1915. 

'  Union  M('d.,  1853.  "  Kiinik  de  R.  II,  1876. 

<-  Volkmann's  Klinik,  No.  171.  «  Neurol.  Ctbl.,  1883,  p.  452. 

•  Couiptes  rendus,  1884,  p.  53. 

»  Batten,  The  Myopathies  or  Muscular  Dystrophics,  Quart.  Jour.  Med.,  April,  1910. 
Lorenz:  Krankheiten  d.  Muskcln,  1904.     Jendrassik:  Handbuch  d.  Neurol.,  1911. 


272 


THE  ENDOCRINOPATHIES 


Heredity  is  a  common  feature;  they  usually  occur  at  an  early  age; 
the  muscles  become  weak  gradually  and  atrophy  in  a  peculiar  manner, 
in  that  true  h\T)ertrophied  fibers  are  mingled  with  atrophied  fibers. 
The  muscular  atrophy  may  involve  all  of  the  muscles  equally,  or  may 
be  irregularly  distributed  both  as  to  the  body  in  general  or  within  the 
muscle  itself.     Reaction  of  degeneration  and  fibrillary  contraction 


"^^ 


Fig.   127. — Pseudohypertrophic  my- 
opathy.     Early   stage.      (Jendrassik.) 


Fig.  128. — Pseudohypertrophic  myopathy. 
Stage  of  atrophy.  (See  Fig.  127.)  (Jen- 
drassik.) 


are  usually  wanting,  although  a  gradual  loss  of  electrical  excitability 
goes  on  coincidently  with  the  atrophy. 

Certain  muscles,  pectoralis  major,  rhomboid,  serratus  magnus, 
are  oftenest  the  seat  of  early  atrophy.  These  are  also  characterized 
as  congenital  aplasias  (Bing).  The  muscle  electrical  reaction  curve  is 
striking. 


DISEASES  OF   THE  MUSCLES  273 

The  tendon  reflexes  gradually  disappear,  but  the  Achilles  is  apt  to 
persist,  or  occasionally  be  increased,  especially  with  much  pseudo- 
hypertrophy. Sensory  disturbances  are  usually  absent,  likewise 
bladder  and  visceral  disturbances.  Pseudocontractures  with  limitation 
of  movement  are  frequent,  causing  peculiar  positions.  The  patients 
hop  like  frogs.  Bony  dystrophies  are  also  frequent,  mostly  showing 
in  thinning  of  the  long  bones,  with  cranial  deformities,    deformed 


)J  k 


Fig.   129. — Pseudohypertrophic  myopathy.     Later  stage  of  atrophy-.     Compare 
Figs.  127,  128.     (.lendrassik.) 

hands,  short  hands  and  short  feet.  A  number  of  accessory  lesions  ha^■e 
also  been  described  such  as  acromegaly,  gigantism,  idiocy  of  thyroid 
type,  leukoplakias,  vitiligo,  diseased  pineals,  etc.,  all  indicating  endo- 
crinopathic  affiliations  of  a  polyglandular  trend. ^ 

Pathology  and  Pathogeny. — Erb  based  his  synthesis  upon  the  changes 
he  found  in  the  muscles,  but  at  the  same  time  was  inclined  to  attribute 

•  Timme:  Arch  of  Internal  Medicine,  1917. 
18 


274  THE  ENDOCRINOPATHIES 

them  to  lesions  in  the  sympathetic  cells  of  the  cord.  These  muscle 
changes  consist  in  the  main  in  hypertrophy  and  atrophy  and  splitting 
of  the  muscle  fibers,  proliferation  of  the  nuclei,  new  connective-tissue 
proliferation  with  hyperplasia  of  the  vascular  tissues  and  fatty  deposi- 
tion. Macroscopically  the  muscles  have  lost  their  normal  color,  vary- 
ing from  pale  pink  to  dark  red.  In  places  where  the  muscle  substance 
has  entirely  disappeared  white  connective  tissue  is  apparent.  The 
muscle  plates  are  frequently  missing. 

Loss  of  cells  in  the  ventral  horns  has  been  described  by  Holmes^  and 
others.  Whether  these  are  primary  or  secondary  cannot  yet  be 
determined. 

The  changes  in  the  bones  is  in  the  nature  of  a  trophic  rarefaction. 


Fig.  130. — Pseudohypertrophic  myopathy.    Later  stage.    Compare  Figs.  127,  128,  129. 

(Jendrassik.) 

The  newer  studies  on  the  vegetative  nervous  control  of  muscle 
metabolism  affiliates  this  entire  group  to  the  vegetative  nervous 
disorders,  in  which  an  endocrinous  myogenic  disorder  is  a  practical 
hypothesis.  Thus  Parhon  and  Savini  have  found  that  some  Landouzy- 
Dejerine  cases  react  to  testicle,  thyroid  and  muscle  Abderhalden  tests. 

Forms.^ — The  forms  are  many  and  show  considerable  intermingling. 
They  are: 

1.  Pseudohypertrophic  (Duchenne). 

2.  Juvenile  (Erb). 

3.  Facio-scapulo-humeral  (Landouzy-Dejerine) . 

4.  Atrophic  myatonia  congenita  (Oppenheim). 

5.  Distal  (Gowers). 

6.  Mixed  and  transitional  forms. 

1.  Pseudohypertrophic  Type  (Duchenne,  1849). — Semmola,  in  1834, 
and  Costa  and  Gioja,  in  1836,  antedated  Duchenne  in  describing 

9%  1  Rev.  Neur.  and  Psych.,  1906,  vi,  p.  136. 


DISEASES  OF  THE  MUSCLES  21 Q 

these  cases,  but  pictures  of  earlier  centuries  give  evidence  of  its  pres- 
ence. It  is  the  type  most  frequently  observed.  It  is  more  common 
in  males  (3  to  1)  and  usually  begins  during  childhood.  A  hereditary 
history  is  very  frequent.  The  parents  first  notice  a  certain  clumsiness 
in  the  gait  of  the  child,  then  the  position  of  the  body  is  peculiar, 
the  head  being  bent  f  orw^ard,  and  the  cervical  vertebrae  are  particularly 
prominent.  There  is  an  early  beginning  lumbar  lordosis.  The  patient 
waddles  then  commences  to  find  it  hard  to  go  up  stairs^ — often  trips  and 
falls.  On  rising  from  a  recumbent  position  the  arms  are  called  in  to 
aid,  and  the  mode  of  rising  is  unique.  The  patient  climbs  up  his 
legs,  as  it  were,  with  his  arms.  In  the  final  stages  the  patient  is  unable 
to  raise  himself  at  all. 

The  shoulder-blades  are  freely  movgible  and  rise  with  the  elevation 
of  the  arms.    Atrophies  are  apparent. 


Fig.   131. — Pseudohypertrophic  myopathy.    Later  stage.    Compare  Figs.  127,  128,  129, 

130.     (Jendrassik.) 

The  gait  varies  somewhat  according  to  the  muscles  chiefly  involved. 
It  is  often  wabbly,  like  a  pregnant  woman;  at  times  it  has  a  high  step 
character;  again  the  patient  walks  on  his  toes.  The  lower  limbs  often 
show  marked  hjiDertrophy  in  the  early  stages,  the  calves  are  plump 
and  firm. 

The  atrophy  advances  unequally.  The  muscles  most  affected  in 
the  lower  extremities  are  the  psoas,  glutei,  quadriceps,  sartorius, 
adductors,  gastrocnemius  and  soleus;  in  the  trunk  the  rectus  ab- 
dominis, latissimus  dorsi,  erector  spinse,  rhdmboidei,  infraspinatus, 
serratus,  trapezius,  and  pectoralis  major,  while  in  the  upper  extremities 
the  muscles  mostly  implicated  are  the  deltoid,  biceps,  brachialis,  and 
brachioradialis. 

In  advancing  cases  all  of  the  muscles  go,  save  perhaps  those  of  the 
face.  In  rare  cases  the  face  is  involved  (myopathic  facies),  and  in  a 
few  cases  the  vagus  is  implicated.  In  the  less  advanced  cases  many 
of  the  distal  muscles  can  be  utilized.  The  patients  usually  die  of 
intercurrent  disorders  after  many  years  of  illness. 


276 


THE  ENDOCRINOPATHIES 


2.  Juvenile  Form  (Erb). — This  usually  develops  about  the  age  of 
puberty,  with  weakness  and  atrophy  in  the  shoulder  girdle.  The 
deltoid  may  show  hypertrophies.  The  arm  is  usually  thinner  and  more 
atrophic  than  the  forearm  and  typical  winged  scapulse  develop.  In 
walking  the  patients  not  infrequently  bend  forward  from  weakness 
of  the  trunk  and  support  the  back  by  holding  the  thighs.  Pseudo- 
hypertrophy of  the  calves  is  not  uncommon. 


Fig.  132. — P.seudohypertrophic  myopathy. 
(G.  Hammond.) 


Fig.  133. — Pseudohypertrophic 
myopathy.   (G.  Hammond.) 


3.  Facio-scapulo-humoral  Type  (Landouzy-Dejerine) . — Here  the 
facial  atrophies  usually  develop  early,  particularly  the  orbicularis 
oris.  The  sphinx-like  face  develops,  the  patients  are  unable  to  whistle, 
tapir  mouth  is  often  present,  and  the  smile  is  distorted.  The  eye- 
lids hang  and  cannot  be  closed  completely.     The   shoulder-girdle 


DISEASE.^  OF  THE  MUSCLES 


277 


atrophy  then  advances,  the  waist  is  small  and  wasp-like  and  the  chest 
flattened;  finally  the  dystrophic  process  becomes  universal. 

4.  Amyotonia  Congenita — ]Myatonia  Congenita — Oppenheim^  (Wil- 
son).— It  is  not  certain  whether  this  disorder  should  be  included 
among  the  dystrophies  or  not.  Pathologically  it  seems  identical, 
clinically  it  is  quite  at  variance.  Spiller  made  the  first  autopsy.  The 
number  of  cases  known  (about  60 — 1911)  prevents  a  definite  answer 
at  the  present  time.^  Rothmann  is  inclined  to  ally  it  with  the  ^Yerdnig- 
Hoffmann  spinal  nuclear  atrophies  as  a  congenital  variety. 


Fig.    134. — Pseudohj-pertrophic  myopathy.     (G.  Hammond.) 

^ymytoms. — The  disorder  is  usually  congenital,  hypotonia  is  char- 
acteristic, with  loss  of  tendon  reflexes.  Active  motion  is  impaired  by 
reason  of  weakness,  but  the  limbs  are  not  paralyzed.  Usually  the 
lower  limbs  are  involved,  in  half  of  the  cases  the  upper,  and  in  a  few 
those  of  the  trunk  and  neck.  These  little  patients  kaleidoscope  as  it 
were.  The  facial  muscles  are  usually  spared.  The  intercostals  are 
but  little  aflected. 

Electric  reactions  are  normal,  or  show  quantitative  reduction. 
The  knee-  and  Achllles-jerks  are  usually  absent;  those  of  the  upper 
extremity  less  constantly  gone.  Atrophy  is  not  definite,  nor  is  pseudo- 
hypertrophy present.  Mechanical  irritability  and  fibrillary  contrac- 
tions are  absent. 

Contractures  are  not  uncommon.  The  sphincters  are  intact.  Sen- 
sibility is  intact,  also  the  special  senses,  and  the  children  are  usually 

•  Monats.  f.  Neurologie  u.  Psychiatrie,  1900,  viii,  p.  232. 

'  Latest  literature:  Collier  and  Holmes,  Brain,  1909;  Batten,  loc.  cit.;  Cassiror,  Hand- 
buch  d.  Neur.,  1911;  Griffith,  Arch.  Kindhk.,  1910;  Griffith  and  Spiller,  Am.  Jour.  Med. 
Sc,  August,  1911. 


278 


THE  ENDOCRINOPATHIES 


bright  mentally.  The  general  condition  is  good,  and  vasomotor  dis- 
turbances are  absent. 

The  disorder  has  some  tendency  to  improve,  although  Batten 
claims  that  not  one  has  gotten  well.  Some  of  the  patients  learn  to 
stand,  but  rarely  unaided. 

Intercurrent  disorders,  particularly  respiratory,  cause  death  in  the 
majority. 

5.  Distal  Type. — Gowers-Spiller,^  and  Spiller^  first  definitely  sepa- 
rated this  myopathy  from  the  apparently  related  Charcot-Marie- 
Tooth  atrophy.  It  varies  little  from  this  latter  save  in  the  absence  of 
sensory  disturbances. 


Fig. 


135 . — Landouzy-De  j  erine 
mj'opathy. 


Fig. 


136. — Landouzy-Dejerine 
myopathy. 


Treatment. — This  has  been  very  unsatisfactory  thus  far  in  this  entire 
group.  The  pathogenesis  is  still  to  be  worked  out.  The  most  hopeful 
of  the  newer  suggestions,  as  yet  only  tried  out  in  a  few  cases,  is  the 
use  of  muscle  substance  preparations.  Carnot  has  experimented  with 
fetal  muscle  substances  trying  to  find  possible  regenerating  elements. 
Testicular  and  suprarenal  lipoids  at  times  stimulate  muscle  growth. 
These  are  purely  empirically  used  remedies.  A  careful  study  of  the 
heredity  for  endocrinopathic  organ  inferiority  may  help  in  the  use  of 
the  glandular  products.  Th\Tnus  and  pineal  have  been  used.  Until 
the  activities  of  the  sympathetic  and  vagus  fibers  in  their  control  of 
muscle  metabolism  are  better  understood,  medicine  will  remain  in  the 


1  Brit.  Med.  Jour.,  1902. 


2  Jour.  Nerv.  and  Ment.  Dis.,  1906. 


DISEASES  OF  THE  MUSCLES  279 

dark  respecting  this  group.  These  patients  should  be  systematically 
studied  by  the  vegetative  methods. 

Fatty  Syndromes. — Obesity. — The  exact  mechanisms  underlying  fat 
metabolism  are  not  completely  understood.^  From  a  chemical  point  of 
view  the  sjoithesis  seems  to  start  with  glucose  which  on  oxidation  forms 
pyruvic  acid.  A  hormone  action  then  converts  this  into  acetaldehyd 
and  carbon  dioxide.  Condensation  produces  higher  ketone  acids  which 
finally  by  further  condensation  and  polymerization  build  up  fatty 
acids.  The  whole  process  is  reversible.  What  the  hormones  are  and  how 
controlled  is  unknown. ^  The  vegetative  nervous  system  is  probably  in 
action  but  how  is  as  yet  purely  conjectural.  Endocrinous  disorders — 
gonads,  h^TDophysis,  possibly  pineal,  are  known  to  modify  the  oxida- 
tions and  permit  fat  storage.  These  take  place  in  very  characteristic 
fashion  and  some  have  been  discussed  under  the  heads  of  adiposis 
genitalis  of  pituitary  and  of  gonadal  origin,  eunuchism  and  eunuchoid- 
ism.   Other  syndromes  will  be  taken  up  here. 

Lorand  described  an  exogenous — possibly  pancreatic — type,  occur- 
ring in  big  eaters  and  developing  diabetes  from  overwork  of  the  oxidiz- 
ing mechanisms — pancreatic  suprarenal (?),  and  an  endogenous  type  of 
endocrinous  origin.  Thus  von  Noorden  differentiated  a  pancreatogenic 
obesity  and  a  th\Togenic  obesity.  Frohlich  separated  his  hypophyseal 
t.^^pe.  Kraus  has  described  a  variety  of  this,  pilous  cerebral  adiposity. 
In  addition  a  group  of  localized  lipomatoses  are  to  be  recognized.  These 
are  the  various  types  of  lipomatoses  of  which  the  adiposis  dolorosa  of 
Dercum  is  an  extreme  form  rather  than  a  separate  disease  since  in 
many  of  the  milder  types  of  symmetrical  and  circumscribed  lipomatoses 
pains,  psychic  and  neurotic  disturbances  are  seen. 

Adiposis  Dolorosa.-^ — Dercum  (1888)  first  named  one  of  these  syn- 
dromes. It  is  characterized  by  adiposity,  pains,  general  muscular 
weakness  and  psychical  changes.  The  patients,  mostly  women  (6  to  1), 
averaging  between  thirty  and  fifty  years,  with  a  tendency  to  family 
obesity,  slowly  develop  fatty  deposits,  often  enormous  in  size.  This 
adiposity  may  show  as  nodular  deposits  (sjnnmetrical  lipomatosis), 
varying  in  size  from  a  bean  to  an  apple.  They  may  be  circumscribed 
or  the  adipose  deposits  may  be  generally  diffused  throughout  the 
entire  body.  The  hips,  shoulders,  upper  arm  and  abdomen  are  pre- 
dilection sites.  The  skin  is  tense.  The  fatty  deposits  in  the  well- 
developed  t^^)es  are  often  painful  to  pressure,  especially  at  nodular 
points,  and  even  at  times  before  there  is  much  fatty  infiltration. 
Hyperesthesise  and  paresthesia  in  the  form  of  tingling,  burning,  numb- 
ness, etc.,  are  frequent.  Spontaneous  pains  occur  with  some.  They  are 
sharp  and  intermittent,  localized  in  the  skin  or  more  deeply,  and  often 
increase  with  motion.  Asthenia  is  a  marked  sign  and  psychical  altera- 
tions are  the  rule.  These  latter  are  mostly  in  the  nature  of  depressions 
with,  at  times,  suicidal  ideas,  irritability,  capriciousness  and  other  signs 

'  Bayliss:  Principles  of  General  Physiology,  ii  edit.,  1918. 
-  Maclean:  Lecithin  and  Allied  Substances,  1918. 
»  Hirschfeld:  Ztschr.  f.  d.  g.  N.  u.  P.,  ref.  Bd.  vi. 


2S0  THE  ENDOCRINOPATHIES 

closely  resembling  manic-depressive  states  {q.  v.)  with  flight  of  ideas 
and  confusions.    Other  patients  show  marked  deteriorations. 

Various  anomalous  conditions  have  been  observed  with  different 
patients,  such  as  vasomotor  signs  with  edemas,  ecchymoses,  hemor- 
rhages, and  pigmentation.  Hyperidrosis,  anidrosis,  and  trophic 
changes  also  are  recorded.  Various  combinations  with  other  members 
of  this  vast  collection  of  chemical  metabolism  anomalies  are  numerous. 
Some  of  these  have  been  hyperthyroidism,  myxedema,  scleroderma, 
Raynaud's  disease,  pluriglandular  dysfunction,  etc. 

The  disorders  usually  progress  slowly  and  vary  greatly  in  intensity 
from  year  to  year.  The  patients  recover  spontaneously  or  finally  die 
of  intercurrent  disease. 


Fig.   137. — Adiposis  dolorosa.     (Dercum.) 

The  organs  involved  have  been  the  hypophysis  and  the  thyroid 
chiefly,  but  the  exact  relationships  are  still  uncertain.  Hypophyseal 
tumors  are  not  infrequently  found,  ovarian  disease  is  present  in  some, 
vascular  neurotrophic  disorders  (blood  gland  disease)  are  present  in 
still  others. 

Treatment. — Opotherapy  with  thyroid  has  been  of  service  in  those 
cases  in  which  a  diseased  thyroid  has  been  associated.  Ovarian  extract 
has  helped  the  ovarian  defect  types.  Electricity,  hydrotherapy,  diet 
and  general  hygienic  control  has  been  of  service  in  others.  Here  as  in 
other  of  the  endocrinopathies  a  careful  survey  of  the  heredity  may  give 
useful  clues  as  to  the  probable  constitutional  defects  and  to  their 
partial  alleviation  by  properly  selected  opotherapy. 

Bony  and  Ligamentous  Syndromes :  Osteopathies,  Arthropathies. — 
Bone  disorders  due  to  disturbances  of  nervous  functioning  are  by  no 
means  infrequent.  The  nervous  mechanisms  underlying  bone  develop- 
ment are  not  clear  but  at  least  two  large  groups  of  neurological  bone 
disturbances  may  be  separated.  They  are:  those  dependent  upon  dis- 
ease of  the  vegetative  nervous  system  (endocrinopathies):  and  those 
accompanying  sensorimotor  disease.^ 

The  chief  bony  endocrinopathies  are  achondroplasia,  osteomalacia, 

'  Sterling,  loc.  cit. 


Fig.   138. — Micromelia  in  achondroplasia,  showing  the  trident  hand  in  twenty-one-year- 
old  patient.     (A.  Josefson.) 


Fig.   139. — Achondrojtlasia  with  micromelia,  showing  shortened  upper  extremity  witli 
curvatures  of  bones  in  twenty-one-year-old  patient.     (A.  Josefson.) 


282 


THE  E N DOC RINOPAT HIES 


the  bony  changes   of  acromegaly,   gigantism,   infantihsm,   leontiasis 
ossea,  etc. 

Achondroplasia. — This  consists  chiefly  in  a  defective  fetal  develop- 
ment of  the  bones  of  the  extremities  (micromelia),  with  comparatively 
normal  development  in  all  other  tissues  of  the  body. 


Fig.  140.- 


-Pseudo-epiphysis  in  second  metacarpal  bone  (proximal), 
(hypothyroid)  stigma.     (A.  Josefson.) 


An  endocrinous 


Symptoms. — The  skull  is  enlarged,  often  hydrocephalic,  with  depres- 
sion of  the  nose  and  prognathism.  Shortening  of  the  extremities 
(micromelia)  is  characteristic,  with  exaggerated  curvature  of  the 
shortened  bones.  The  fingers  are  nearly  all  of  the  same  length  and 
radiate  slightly,  spoke-like,  from  the  metacarpal  joints  with  a  tendency 
to  form  the  trident  hand.  The  thumb,  first  and  second,  fourth  and 
little  finger,  respectively,  are  arranged  in  groups.  Lumbar  lordosis 
with  prominent  abdomen  is  usual. 


DISEASES  OF  THE  MUSCLES  283 

The  interpretation  of  the  disorder  is  difficult.^  Heredity  is  prominent, 
males  chiefly  being  affected. 

Osteomalacia. — This  is  a  type  of  bony  softening  closely  resembling 
rickets  and  apparently  dependent  upon  disturbed  calcium  metabolism 
through  the  mediation  of  the  endocrinous  glands  (thyroid,  parathyroids, 
ovary) . 

Symptoms. — These  are  best  consulted  in  text-books  of  medicine  or 
surgery. 

Oxycephaly,  or  Tiirmschddel,  results  from  a  premature  closure  of  the 
coronal  and  sagittal  sutures  which  restricts  the  growth  of  the  skull 
and  forces  it  into  a  conical  shape.  Optic  atrophy,  with  impairment  of 
vision  and  headache  and  mental  failure  are  the  chief  symptoms. 

Rheumatoid  Arthritis. — Certain  hj-pothyroidisms  {q.  v.)  permit  the 
development  of  a  rheumatoid  arthritis,  possibly  by  the  reduced  capacity 
of  the  individual  to  react  normally  to  minimal  subinfections,  often  of 
cryptogenic  origin,  teeth,  the  frontal,  malar,  and  ethmoid  shmses, 
tonsils,  intestines,  old  vesiculitides,  etc. 

Neurogenic  Arthropathies. — ^These  are  frequent  in  tabes,  paresis, 
syringomyelia  (Raynaud)  neuritis,  leprous  neuritis.  Sterling^  has 
given  a  very  complete  and  monographic  description  of  these. 

Psychogenic  arthropathies  and  arthritides  are  as  yet  not  definitely 
established.  There  is.some  evidence  from  the  psychoanalytic  school  to 
show  that  unconscious  complex  reactions  may  show  themselves  as 
bony  syndromes.  The  classical  relationship  between  excessive  anger 
and  gout  is  a  case  in  point.  Unconscious  sadistic  states  produce  trans- 
itory and  even  chronic  arthritic  changes. 

1  Parhon:  Nouvelle  icon.  d.  1.  Salp.,  1913,  xxvi,  185. 

2  Zeit.  f.  d.  ges.  N.  u.  P.,  Ref.,  vol.  ix. 


PART  II. 

THE  SENSORIMOTOR   SYSTEMS. 

SENSORIMOTOE  NEUROLOGY. 


CHAPTER   IV. 
CRANIAL  NERVES. 

Introduction. — The  organism  as  a  whole  can  be  conceived  as  an 
intricate  mosaic  of  tendencies,  the  partial  tendencies  of  the  several 
parts  or  organs  and  the  larger  tendencies  of  the  whole  biological  unit. 
The  partial  tendencies  constitute  the  field  of  physiolog}' — the  function 
of  the  various  organs.  The  field  of  the  tendencies  of  the  whole  organism 
is  the  domain  of  psychology.  These  tendencies,  be  the}'  partial  or  total 
are  states  of  disequilibrium  which  tend  to  develop  mechanisms  for 
restoring  the  equilibrium  or  neutralizing  the  stimulus  of  the  tendency. 
Repeated  tendencies  of  the  same  character,  each  tending  to  the  pro- 
duction of  the  same  mechanism  form  channels  of  energy  discharge  which 
become  progressively  more  stable  and  definite.  The  reaction  becomes 
fixed,  stereotyped.  This  is  the  process  of  the  structuralization  of 
function  of  which  the  reflex  is  the  tN^je  of  functional  response  at  the 
sensorimotor  level. 

The  various  correlating  functions  of  the  organism  which  have  been 
developed  through  the  centuries  thus  come  to  be  laid  down  in  structures 
and  it  is  of  such  that  the  so-called  central  nerv^ous  system  and  its 
peripheral  ramifications  consist.  Its  leflex  t^^pes  of  response  are  the 
t^T^es  at  the  sensorimotor  level  as  that  term  is  used  in  this  book. 
Definite  nervous  pathways,  therefore,  correspond  to  functions  which 
have  become  so  thoroughly  organized  as  to  be  laid  down  in  permanent 
pathways.  The  relation  between  cardiac  and  respiratory  rate  has 
become  so  thoroughly  established  as  a  necessity  that  nothing  is  left  to 
chance;  a  permanent  interconnection  has  been  so  definitely  structural- 
ized  that  it  can  be  relied  upon  always,  under  all  conditions,  to  bring 
about  the  required,  definite  result. 

In  this  process  of  the  structurally  ation  of  function  there  must  of 
necessity  remain  an  unorganized  residual  which  represents  the  organ- 
ism's capacity  for  adaptation  to  the  unusual  and  the  infrequent.    This 

(285) 


286  CRANIAL  NERVES 

unorganized  residual  is  represented  at  the  psychological  level  by  all 
those,  as  yet  uncrj^stallized  tendencies,  those  unrealized  hopes  and 
ambitions  which  mean  that  the  individual  has  not  as  yet  become 
stabilized  beyond  recall  in  his  habits  of  thought  and  action.  At  the 
organic  (so-called)  end  these  are  represented  by  those  physicochemical 
mechanisms  served  by  the  endocrine  glands  and  their  hormones  (prob- 
ably the  specific  substances  of  all  the  organs)  and  that  oldest  portion 
of  the  nervous  system,  the  vegetative,  which  have  not  yet  become 
crystallized  into  definite  forms  of  response.  These  reactions  are 
general  in  character,  acquisitive  (love),  avertive  (hate)  but  which  as 
yet  have  not  acquired  specific  avenues  of  discharge  which  are  capable 
of  satisfactorily  expressing  the  higher  tendencies  of  the  organism  as  a 
whole,  that  is  at  the  psychological  level.  The  cerebrospinal  pathways 
represent  those  pathways  of  energy  discharge  which  have  been  laid 
down  in  structure  and  represent  those  more  specific,  organic,  physio- 
logical avenues  of  integration  which  meet  the  demands  of  a  greater 
specificity.  The  more  generic  craving,  such  as  hunger,  has  to  develop 
a  mechanism  of  response  which  has  as  its  function  those  specific 
activities  calculated  to  acquire  food:  fear  and  hate  have  to  develop 
those  specific  types  of  response  which  are  calculated  in  these  higher 
types  of  organisms  with  a  cerebrospinal  axis  to  produce  those  niceties 
of  correlation  which  will  lead  to  a  successful  fiight  or  fight,  etc.,  all  of 
which  activities  have  to  be  organized  to  produce  effective  responses  in 
accord  with  the  demands  of  the  organism  at  its  stage  in  evolution.  For 
example,  the  acquirement  of  food  or  the  reaction  of  flight  are  very 
different  in  a  hydroid  or  a  spider  respectively,  and  very  different  in 
those  animals  from  the  like  demands  upon  a  mammal  or  upon  man. 
These  elements  of  structure  might  be  said  to  be  on  their  way  to  a  more 
stabilized  structuralization  but  nevertheless  a  sufficient  residual  of  as 
yet  dynamically  fluid  mechanisms  remains  for  the  purposes  of  dealing 
with  new  problems. 

The  relations  of  these  two  parts  of  the  nervous  system,  however, 
cannot  be  adequately  understood  if  the  more  recent  cerebrospinal 
system  is  simply  conceived  as  overlying  the  phylogenetically  older 
vegetative  system.  The  dynamic  factors  which  have  produced  the 
differentiation  must,  because  of  their  very  nature,,  be  still  active  so 
that  when  demands  are  made  upon  the  organism  which  are  beyond  the 
capacity  of  its  definite  structure  they  may  spill  over  and  flood  the  low- 
lands of  the  less  definitely  organized  portion  of  both  the  vegetative  and 
the  cerebrospinal  apparatuses  with  a  fair  chance  of  ultimately  channelling 
their  way  to  a  reasonably  efficient  outlet  in  action.  Structurally  this 
interrelation  is  evidently  shown  in  the  intimate  admixture  of  unmyelin- 
ated, probably  vegetative  nerve  fibers,  with  the  myelinated  fibers  of 
the  cerebrospinal  system  and  the  double  innervation  of  the  voluntary 
musculature  by  both  sets  of  fibers  as  well  as  the  probable  double  struc- 
ture of  voluntary  muscle  itself.  The  sacroplasmatic  substance  is  clearly 
analogous  to  unstriped  muscle  and  there  is  evidence  that  it  is  inner- 
vated by  the  vegetative  nerve  fibers,  while  the  anisotropic  disk  system 


DISEASES  OF  THE  OLFACTORY   TRACT  287 

is  innervated  by  the  cerebrospinal  myelinated  fibers.  The  organism 
is  thus  constituted  to  respond  both  in  the  more  definitely  reflex  way 
of  the  cerebrospinal  structures  and  in  the  more  diffuse  way  of  the 
vegetative,  physicochemical  structuralizations. 

I.  DISEASES  OF  THE  OLFACTORY  TRACT. 

Olfactory. — In  man  the  olfactory  apparatus  has  seemingly  lost  much 
of  the  importance  it  possesses  in  the  lower  animals.  Other  sensory 
zones,  notably  those  for  the  eye  and  ear  and  language  have  taken 
the  lead  in  the  program  of  evolution  and  have  left  smell,  important 
though  it  be,  in  the  vanguard. 

In  the  primitive  vertebrate^  the  olfactorj^  areas  in  the  cerebral  hemi- 
sphere, and  the  A'isual  and  general  sensory  nuclei  in  the  thalamus 
and  the  midbrain,  are  linked  by  means  of  numerous  tracts  to  the  hypo- 
thalamus, which  at  first  is  a  relativel}^  large  part  of  the  brain.  In  the 
pursuit  of  food,  as  well  as  in  the  animal's  other  vital  activities,  this 
large  hypothalamic  apparatus  is  thrown  into  action  as  soon  as  the 
sense  of  smell  excites  a  response.  By  means  of  numerous  descending 
tracts  the  hypothalamus  exerts  a  widespread  influence  upon  a  variety 
of  visceral  and  vasomotor  functions,  some  of  which  stir  into  activity  the 
glands  and  other  structures  directly  concerned  with  the  processes  of 
swallow^ing  and  digestion,  as  well  as  other  mechanisms  w^hich  enable 
the  creature  to  devote  the  whole  energy  of  its  body  to  the  immediate 
and  vital  purpose  of  the  piu-suit.  And  w^hen  the  consummation  of  the 
reaction  is  attained,  and  the  buccal  mucous  membrane  is  itself  stimu- 
lated, ascending  tracts  carry  to  the  hypothalamus  the  effects  of 
this  excitation  of  the  taste  organs,  and  through  it  stir  up  even  more 
potent  effects  in  the  digestive  and  other  visceral  systems. 

As  Elliott  Smith  says,  it  is  not  "  a  mere  coincidence  that  the  pituitary 
body  is  developed  from  a  combination  of  buccal  epithelium  and  hypo- 
thalamus, and  is  an  organ  which  excites  (no  doubt  by  very  different 
means)  profound  effects  in  the  \^isceral  systems,  in  some  respects 
analogous  to  that  obtained  by  the  neural  bucco-hj^jothalamic  con- 
nection. 

Thus  the  hypothalamus  represents  the  mechanism  whereby  the 
instruments  concerned  with  the  phenomena  of  consciousness  can 
influence  the  visceral  functions  of  the  body,  and  express  those  manifold 
emotional  effects  which  play  so  obtfusi\'e  a  part  in  many  experiences 
that  are  strongly  charged  with  affective  tone. 

The  cerebral  hemisphere  was  derived  from  and  built  upon  what 
in  the  primitive  vertebrate  was  simply  the  receptive  instrument 
for  smell  impressions,  and  the  olfactory  tracts,  unlike  all  the  other 
sensory  paths,  lead  directly  to  the  cerebral  cortex  without  the  inter- 
mediation of  the  thalamus.  The  primitive  hemisphere,  therefore, 
must  have  been  the  instrument  whereby  the  afl'ective  aspect  of  smell 

1  Smith,  G.  Elliott:  Significance  of  the  Cerebral  Cortex.  Croonian  Lectures  II,  III: 
British  Medical  Journal,  June  28,  1919. 


288  CRANIAL  NERVES 

was  appreciated — that  is,  it  performed  the  functions  with  which  the 
thalamus  is  concerned  for  the  other  senses. 

"Guided  by  the  sense  of  smell,  the  behavior  of  the  primitive  verte- 
brate is  brought  under  the  influence  of  other  'distance-receptors,'  which 
secondarily  acquire  an  affective  significance  or  meaning  by  being 
brought  into  intimate  relationship  with  the  dominant  sense.  Speciali- 
zations of  structure  and  function  then  develop  by  the  cultivation  of 
these  other  senses,  often  at  the  expense  of  the  sense  of  smell,  and 
animals  like  the  teleostean  fishes  are  evolved  which  are  guided  mainly 
by  vision.  This  determines  a  high  development  of  the  midbrain  and 
a  retrogression  of  the  cerebral  hemisphere.  Incidentally  such  creatures 
lose  the  possibility  of  further  advancement  in  the  direction  of  the 
higher  vertebrates,  not  merely  because  they  have  become  specialists, 
but  also  because  the  adaptability  of  the  cerebral  hemisphere  upon 
which  real  progress  depends  has  been  sacrificed  in  the  acquisition  of 
visual  efficiency. 

"The  new  mode  of  life  on  land  demanded  not  only  the  development 
of  entirely  new  modes  of  locomoti  du,  but  also  a  transformation  of  the 
distance  receptors  of  the  hind-brain,  as  well  as  the  organs  of  smell. 
For  the  organ  that  was  evolved  to  detect  slight  movements  in  the 
water  became  the  organ  of  hearing,  and  the  olfactory  mucosa,  which 
was  specialized  to  respond  to  very  delicate  forms  of  chemical  stimula- 
tion, now  became  much  more  highly  sensitive  and  adapted  to  appreci- 
ate the  more  subtle  effects  of  minute  air-borne  particles  of  odoriferous 
substances. 

"  The  .heightened  powers  of  smell,  the  new  powers  of  hearing,  and  the 
necessity  for  acquiring  new  methods  of  locomotion,  provided  a  power- 
ful stimulus  to  the  cerebral  hemisphere;  and  there  are  reasons  for  sup- 
posing that  the  earliest  Tetrapoda  were  provided  with  a  brain  with  a 
well-developed  cerebral  cortex.  But  their  surviving  amphibian 
descendants  only  in  part  emancipated  themselves  from  the  aquatic 
mode  of  life,  and  were  satisfied  with  slow-going,  clumsy  movements  of 
a  simple  character.  As  the  result  their  cerebral  cortex  underwent  a 
progress  of  retrogression,  and  the  amphibia  dropped  out  of  the  race  for 
vertebrate  supremacy. 

"  But  one  group  of  proto-amphibians  was  more  progressive.  They 
acquired  t.he  power  of  more  rapid  movement  and  of  more  varied 
response  to  the  influence  of  their  environment.  The  sensitivity  of 
touch,  smell,  vision,  and  hearing  became  heightened  and  the  influence 
of  these  senses  so  intimately  integrated  b}'  evolutionary  changes  in  the 
cerebral  hemisphere  as  to  make  possible  a  much  more  complex  and 
intelligent  behavior.  The  increasing  significance  of  vision,  hearing, 
and  touch,  found  expression  in  the  sudden  growth  of  the  thalamus  and 
the  development  of  larger  tracts  of  fibers  ascending  from  the  thalamic 
nuclei  into  the  lateral  edge  of  the  pallium,  which  then  suddenly 
expanded  and  bulged  into  the  ventricle.  The  great  mass  of  low-grade 
pallium,  which  may  be  called  'hypopallium,'  thus  formed  represents 
the  first  relatively  independent  representation  in  the  cortex  of  the 


DISEASES  OF   THE  OLFACTORY   TRACT 


289 


senses  of  vision,  hearing,  and  touch,  at  first  chiefly  of  the  trigeminal 
nerve.  Then  for  the  first  time  the  cerebral  cortex  became  an  instru- 
ment whereby  these  senses  played  a  combined  part  in  controlling 
behavior  and  added  their  quota  to  the  discriminative  side  of  the 
animal's  consciousness.  The  reptile  thus  came  to  acquire  the  power  of 
learning  by  experience  from  other  senses  than  smell  and  of  correlating 
the  information  acquired  by  touch,  vision,  and  hearing,  to  perform  acts 
of  discrimination  in  which  these  several  senses  took  a  definite  part. 
But  this  hypopallium  was  an  imperfect  instrument;  and  when  it  became 
superseded  by  the  development  of  the  higher  mechanism  of  the  neo- 
pallium, it  (the  hypopallium)  became  converted  into  [the  greater  part 
of]  the  nucleus  caudatus,  the  putamen  and  the  claustrum." 


Fig.  141. — Extent  of  true  olfactory 
receptors  on  the  miicou."?  membrane. 
(v.  Brunn.) 


Fig.  142. — Scheme  of  bipolar  receptors  of  the 
olfactory.     (After  R.  Cajal.) 


The  receptors  for  smell  are  located  in  a  limited  portion  of  the 
Schneiderian  mucous  membranes.  They  react  to  very  minute  chem- 
ical stimuli,  being  for  some  substances  from  1  to  20,000  per  cent,  more 
sensitive  than  the  receptors  for  taste.  One  part  in  8,000,000  of  musk 
is  capable  of  being  consciously  detected.  There  is  marked  variability 
in  individual  threshold  capacity  as  determined  by  Zwaardemaker's 
olfactometer.  Thus  many  smell  reactions  are  practically  non-appre- 
hensible to  consciousness,  yet  minimal  unconscious  stimuli  nevertheless 
may  produce  widespread  reactions.  Horse  and  cat  asthma,  hay  fever, 
etc.,  may  thus  have  psychogenic  etiological  factors  from  unconscious 
odor  associations,  as  well  as  chemical  etiological  factors. 

Changes  in  that  })()rtion  of  the  membrane,  such  as  occur  in  any  acute 
inflammatory  disease,  coryza,  influenza,  diphtheria,  etc.,  cause  diminu- 
tion or  loss  of  ability  to  smell.  Albinism  is  usually  associated  with  loss 
of  smell.  Chronic  inflammatory  processes,  often  accompanied  by  fetid 
odors,  polyps,  frontal  or  maxillary  sinusitis,  lead  poisoning,  usually 
bring  about  unilateral  or  bilateral  loss  of  smell.  Most  of  the  causes 
for  this  mostly  peripheral  loss  of  smell  may  be  estimated  by  direct 
inspection.  Certain  <lriigs  acting  locally,  cocain,  etc.,  influence  smell. 
19 


290 


CRANIAL  NERVES 


From  the  receptors,  unmyelinated  fibers  traverse  the  cribriform  plate 
and  enter  the  olfactory  bulb,  forming  synapses  with  the  mitral  cells. 
The  axones  of  the  mitral  cells  compose  the  olfactory  tract,  forming 
higher  synapses  in  the  olfactory  area.  Lesions  in  and  about  the 
cribriform  plate  from  fractures,  meningitis,  syphilis,  pressure  of  frontal 
tumor,  may  determine  a  diminution  or  loss  of  smell;  possibly  lesions  in 
this  portion  of  the  olfactory  pathway  may  cause  hallucinatory  odors, 

but  this  is  still  debatable.  Certain  tumors 
lying  upon  the  orbital  plate  of  the  sphe- 
noid and  compressing  the  lobus  olfacto- 
rius  have  seemed  to  give  rise  to  unilateral 
and  bilateral  hallucinations  of  smell 
(Fig;  143). 

Disease  of,  or  pressure  upon,  the  ol- 
factory tract  in  its  peripheral,  hypo- 
thalamic, or  cortical  portions  results  in 
either  diminution  (hyposmia)  or  loss 
(anosmia)  of  smell;  hallucinations,  illu- 
sions (parosmia,  ismosmia  or  cacosmia), 
or  hyperesthesise,  causing  excessive 
sneezing.  Odor  influences  taste  directly 
and  by  association  involves  the  entire 
vegetative  nervous  system.  The  differ- 
ent parts  of  the  olfactory  pathways  need 
to  be  taken  into  consideration.  The 
study  of  olfactory  hallucinations,  par- 
ticularly in  certain  psychoses,  and  in 
certain  tumors  in  or  about  the  frontal 
lobes,  and  the  uncinate  gyrus,  renders 
this  of  value. 

It  is  known  that  certain  sneezing  crises 
have  been  determined  by  tabetic  lesions. 
Just  which  parts  of  the  olfactory  tracts 
are  involved  is  not  certain. ^ 

Tertiary,  reflex  and  collateral  neurons 
pass  to  the  hypothalamus,  midbrain, 
and  to  the  cortex.     (See  Fig.  144.) 

Clinical  correlations  with  disorder  of 
this  portion  of  the  olfactory  pathway  are 
not  certain.  Certain  overaft'ective  reac- 
tions to  odors,  disgusts,  nausea,  even  vomitings  from  odors  need  to  be 
more  carefully  sifted  in  this  connection,  especially  in  relation  to  brain 
tumor  localizations.  One  personally  observed  patient  with  chordoma 
of  the  base  complained  of  the  smell  of  "burning  material";  another  with 
a  frontal  fibroma  was  anosmic  to  test  and  yet  had  a  constant  hallucina- 
tory projection  of  smelling  disagreeable  things,  feces,  etc.     They  should 


Fig.  143. — Illustration  of  first 
and  second  neurons  of  the  olfac- 
torius.  The  first  synapse  takes 
place  in  the  glomeruli  (mitral 
cells.)     (Edinger.) 


>  Klippel  and  Lhermitte;  Seni.  Med.,  February  17,  1909. 


DISEASES  OF  THE  OLFACTORY  TRACT  291 

not  be  viewed  as  whims  or  fancies  of  hysterical  patients.  Loss  of  smell 
may  result  from  hj^^othalamic  lesions,  usually  homolateral.  The  cross- 
ing of  the  olfactory  pathways  is  incomplete,  and  takes  place  principally 
in  the  anterior  cerebral  commissure  (Fig.  144.) 


Fig.  144. — Scheme  of  olfactory  paths.  X,  vagus  root  fibers;  ca,  anterior  commissure; 
cm,  mammiUary  body;  cp,  fibers  from  nucleus  habenulse  to  posterior  commissiu'e;  fG, 
tract  from  mammillary  body  to  Gudden's  nucleus;  fi,  fasciculus  mammillo-thalamicus; 
fl,  fasciculus  long,  medianus;  fr,  fornix;  ful,  fibers  of  fornix;  gli,  nucleus  habenulse; 
gi,  interpeduncular  ganglion;  gp,  gjTus  pyriformis;  I,  median  lemniscus;  m,  fibers  from 
Gudden's  nucleus  to  substantia  reticularis;  na,  anterior  thalamic  nucleus;  nG,  Gudden's 
nucleus;  nt,  tegmental  nucleus;  nX,  vagus  motor  nucleus;  peE,  ped.  corp.  mammillaris 
from  fillet;  qa,  quadrigemina;  r,  fibers  from  n-tegmenti  to  cranial  nerve  nuclei;  re,  radix 
lateralis  tractus  olfactorii;  rf,  fibers  of  olfactory  tract  to  trigonum  olfactorii;  ro,  median 
olfactory  tract  root;  s,  fibers  from  interpeduncular  ganglion  to  tegmental  nuclei;  so, 
olfactory  trigone;  th,  optic  thailamus;  tro,  olfactory  tract;  tt,  tenia  thalami;  x,  fasciculus 
retroflexus.     (Bechterew.) 

The  cortical  neurons  end  in  the  cornu  ammonis,  which  is  a  large 
olfactory  association  field  connected  with  all  other  parts  of  the  cortex. 
(See  standard  works  of  Edinger,  Ramon  y  Cajal,  Van  Gehuchten, 
Herrick  and  C.  Winkler,  for  the  details  of  the  anatomy  of  the  olfac- 
tory apparatus.)  Lesions  here  often  result  in  peculiar  olfactory  auras, 
as  seen  in  certain  hippocampal  epilepsies  (uncinate  fits  of  Hughlings 
Jackson).  Such  fits  occur  from  temporosphenoidal  tumors  also. 
Olfactory  agnosias  also  result  from  lesions  in  this  general  region.  Some 
of  congenital  origin,  with  agenesis  of  the  cornu  ammonis  have  been 
described.  Anosmias  or  olfactory  agnosias  are  frequent  in  general 
paresis,  and  in  abscess  and  tumor. 

The  efterent  pathways  and  synaptic  reflex  paths  of  the  olfactory  are 


292 


CRANIAL  NERVES 


extremely  numerous.  The  most  common  motor  reflex  is  that  of  sniff- 
ing, with  dilatation  of  the  nostrils.  This  is  occasionally  seen  as  the 
result  of  a  central  somatic  lesion  (paresis),  or  as  a  purely  symbolic 


iiTIOLOGY. 

I.  Congenital,  Defects  of  Olfactory 


Clinical  Signs. 


II.  Senile  Involution 

III.  Mechanical  Influences 

Compression  by  meningitis . 

Tearing  of  fibers ;  fracture .  . 


^Anosmia 


IV.  Functional  Overuse 

Overstimulation;  empyema  of  sinuses^ 


>  Parosmia 


V.  Toxic  Influences 

Influenza 

Cocain 

Strychnine 

Nicotine 

Alcohol 

VI.    kSECONDARY  TO  OtHER  INFLUENCES 

Lesion  of  V  and  VII 


^Hyperosmia 


Psychogenic  (hysteria,  precox).  .  .  . 

Fig.   145. — General  summary  of  olfactory  disturbances.     (Veraguth.) 


DISEASES  OF   THE   VISUAL  APPARATUS  293 

automatic  or  unconscious  act  (compulsion  neurosis,  hysteria,  schizo- 
phrenia). The  relation  between  odors  and  the  vomiting  reflex  is  to  be 
borne  in  mind  in  hysterical  vomiting,  furthermore  the  very  primitive 
associations  between  odor  and  sexual  complexes.  Hyst  rical  anosmia 
may  be  an  isolated  phenomenon  and  is  usually  classical  of  the  mechan- 
ism of  conversion  in  a  narrow  Freudian  sense.  The  psychology  of  smell 
and  its  complicated  relationships  to  infantile  fantasies  of  disgust,  to 
childbirth  from  intestinal  canal,  to  the  symbolizations  of  feces  with 
money,  make  the  study  of  nasal  disorders  of  great  import  in  the  psycho- 
neuroses  and  psychoses.  Hay  fever,  rose  colds,  etc.,  in  many  instances, 
are  psychogenic  conversion  phenomena,  others  may  be  chemical  reac- 
tions (pollen,  horse  odor,  etc.) .  Many  of  the  so-called  anaphylactic  reac- 
tions probably  rest  upon  a  psychogenic  basis.  In  certain  psychogenic 
epilepsies  the  olfactory  symbolisms  are  highly  determined.  What  rela- 
tions these  have,  either  as  cause  or  result,  to  the  cornu  ammonis  lesions 
found  in  these  epilepsies  (Alzheimer)  has  not  yet  been  determined.^  ^ 

Treatment. — The  underlying  cause  of  the  changed  olfactory  state 
needs  treatment,  not  the  state.  Local  applications  of  cocain,  mor- 
phin,  etc.,  are  usually  unjustified.  The  psychogenic  olfactory  dis- 
orders should  be  treated  by  psychotherapy.  ]\Iarked  psychogenic 
factors  are  nearly  always  present  in  nasal  syndromes  of  the  hay  fever, 
rose  cold,  horse  asthma  types.  Every  nose  receives  the  stimuli,  why 
do  certain  ones  react  and  not  others?  The  term  protein  sensitization 
or  anaphylaxis  needs  to  be  explained. 

n.  DISEASES  OF  THE  VISUAL  APPARATUS. 

The  course  of  the  light  pathways  and  the  topographical  arrangement 
of  its  synapses  are  extremely  complex,  since  sight  has  become  almost 
the  principal  tool  of  advance  in  the  evolution  of  man's  mental  powers. 
The  earliest  light  tropisms  in  animals  began  with  the  protozoa,  pre- 
sumably in  such  an  organ  as  the  red  spot.  (Euglena  and  others.)  From 
protozoa  to  man  the  evolution  of  the  receptors  which  have  been 
elaborated  to  capture  light  energies  has  been  most  complex  and  almost 
unanalyzable.  Science  only  knows  the  merest  rudiments.  Different 
light  modalities  probably  traverse  different  optic  pathways.  Of  these 
little  is  kno\Mi. 

The  range  of  light  response  of  the  human  retinal  receptors  is  very- 
great,  yet  it  does  not  include  the  entire  group  of  vibratory  phenomena. 
What  transforms  the  light  energy  into  nerve  energy — electrical — is  not 
positi\ely  kno^^^l.  Mechanical  and  photochemical  (through  the  visual 
purple  of  the  rods)  hypotheses  are  at  present  in  the  ascendant.  The 
human  eye  resolves  points  separated  by  0.002  mm.  at  the  fovea  but 
this  visual  acuity  rapidly  fades  off  toward  the  periphery.  It  varies 
greatly  for  different  colors.  (See  Tests  by  Snellen  Type  in  ^Methods 
of  Examination.)     The  threshold  of  the  stimulus  varies  also  and  is 

*  Bailey,  P.:  Flaubert's  Epilepsy,  Proceedings  of  Charaka  Society,  New  York,  vol.  iii. 

*  Clark,  L.  P.:  The  Epilepsy  of  Doetoiewsky,  Medical  Record,  New  York,  1915. 


294 


CRANIAL  NERVES 


very  distinct  in  different  individuals  in  whom  enormous  variations  in 
color  values  exist.  The  best-knowai  classical  form  of  this  difference 
is  color-blindness.  The  human  eye  is  sensitive  only  to  vibrations 
approximatelv  0.0008  to  0.0004  mm.  in  length  vibrating  at  a  rate  of 
400,000,000,000,000  to  800,000,000,000,000  per  second.  This  makes 
up  about  one-tenth  of  the  entire  range  from  the  slowest  tactile  (heat) 
stimuli  to  the  highest  vibrations  of  the  .r-rays.  No  human  sense  organ 
is  yet  known  that  responds  to  the  Hertzian  electrical  waves,  the  ultra- 
violet or  the  ic-rays.  The  solar  spectrum  contains  about  10  octaves 
of  this  series.    Many  vibrations  unperceived  by  the  human  eye  seem  to 


Mevibrana 
limitans  interna 
Nerve-fibre  layer" 

Ganglionic  layer— 


Inner  plexiform... 
layer 

Centrifugal  fibre'' 


Inner  nuclear^ 
layer 


Fibre  of  Miiller — L- 


Outer  plexiform. 
layer 


Outer  nuclear 
layer 


Meihbrana      \ 
limitans  externa 

Layer  of  rods  _,, 
and  cones 


.Diffuse  amacrine 
cell 


''Amacrine  cells 
•  Horizontal  cell 

---/Bod  bipolar s 
•Cone  bipolars. 


—Pigmented  layer 


Fig.  146. — Plan  of  retinal  neurones.     (After  CajaJ.) 


be  responded  to  by  the  eyes  of  other  animals.  Von  Kries  estimated 
that  the  human  eye  could  distinguish  between  150  and  250  pure 
spectral  tmts,  thus  making  a  functional  range  of  between  500,000  to 
600,000  possible  distinctions.  Enormous  variability  exists  which  is 
at  the  bottom  of  much  of  the  variation  in  perception  of  the  painter 
or  color  artist.  Evolution  to  wider  and  more  useful  adaptation  is 
undoubtedly  going  on  constantly.  This  is  chiefly  brought  about 
through  the  psychical  stimulus.^  ^ 
The  retina,   the  mostly  decussating   optic  nerve   ending   in   the 

1  Compare  Adler's  study  on  the  inferiority  of  organs,  already  referred  to. 

2  Phillips:  Questions  Relating  to  Eye  Training.    Parsons:  Color  Blindness,  1917. 


DISEASES  OF  THE  VISUAL  APPARATUS 


295 


Fig.  147. — Diagrammatic  scheme  of  optic  paths  and  chief  connections  at  four  levels. 
A,  level  of  II  and  III  nerves;  B,  level  of  IV  nerve;  C,  level  of  VI  and  VII  nerves,  tegmen- 
tum of  pons;  D,  spinal  cord.  Neuron  1.  Receptors  in  rods  and  cones  of  retina  are  not 
indicated  in  the  diagram.  Neuron  2.  2a,  axones  passing  to  pulvinar  of  same  side; 
26,  axones  passing  to  corpus  quadrigeminum  of  same  side ;  2c,  axones  passing  to  external 
geniculate  of  same  side,  all  from  temporal  side  of  retina;  from  nasal  side  2a,  axones 
crossing  in  chiasm  going  to  opposite  external  geniculate;  2/,  axones  crossing  in  chiasm 
to  go  to  opposite  anterior  corpus  quadrigeminum ;  2g,  axones  crossing  in  chiasm  to  oppo- 
site pulvinar.  Papillomacular  bundle  fibers  crossed,  partly  uncrossed  (see  Fig.  149). 
Neuron  3.  Pulvinar  axones  to  occipital  cortex ;  36,  external  geniculate  axones  to  occipital 
lobes;  3c,  d,  e,  corpora  quadrigemina  fibers,  middle  layer  decussating  (Meynert)  to 
median  longitudinal  fasciculus  and  forming  tractus  tectobulbaris  et  spinalis  to  go  to 
medvdla  and  anterior  columns,  forming  synapses  with  third,  fourth  sixth,  and  seventh 
nerves  and  motor  nuclei  of  spinal  nerves  (space  orientation) ;  3/,  g,  fibers  from  interstitial 
nucleus  (Cajal)  of  fasciculus  longitudinalis  posticus  forming  part  of  longitudinal  fasciculus 
passing  to  anterior  columns,  forming  synapses  with  III,  IV,  VI  cranial  nerves  and  motor 
spinal  nerves.    Neuron  4.    Axones  from  oculomotor,  facial,  and  spinal  nuclei.     (Strong.) 


296  CRANIAL  NERVES 

external  geniculate,  the  pulvinar  of  the  thalamus  and  the  anterior 
corpora  quadrigemina,  and  finally  the  optic  radiations  terminating  in 
the  calcarine  region  of  the  cortex  make  up  the  primary,  secondary,  and 
tertiary  incoming  neurons  of  this  pathway.     (See  Fig.  147.) 

The  intricate  and  rich  symptomatology  is  dependent  upon,  and  will 
be  discussed  in  accordance  with,  these  anatomical  divisions.  The  chief 
signs  to  be  considered  are  night-blindness,  color-blindness,  dimness  of 
vision,  blindness  in  one  or  both  eyes,  temporary  or  complete 
scotomata,  temporary  or  permanent  hemianopsia,  hemichromatopsia, 
mind-blindness  or  optic  agnosia,  photophobia,  hallucinations  and 
illusions  of  sight,  conversion  and  other  forms  of  symbolic  blindness, 
malingering  of  blindness,  unilateral  or  bilateral. 

Retinitis.— Involvement  of  the  first  neuron  is  termed  retinitis. 
It  may  result  from  the  extension  of  an  inflammation  or  may  be  due 
to  toxic  or  to  hidden  constitutional  factors.  The  chief  indications  are 
ocular  discomfort  or  photophobia,  diminution  of  the  visual  acuity, 
appearance  of  scotomata,  general  contraction  of  the  visual  fields, 
micropsia,  megalopsia  or  metamorphopsia. 

Different  grades  of  retinitis  are  distinguished  opthalmoscopically. 
The  chief  types  are  simple,  albuminuric,  syphilitic,  diabetic,  hemor- 
rhagic and  anemic  retinitis. 

In  simple  retinitis  there  is  clouding  particularly  of  the  superficial 
layers,  in  patches  or  in  larger  portions  at  the  posterior  pole.  The 
veins  are  dull  and  dark  and  full,  and  seem  imbedded  in  the  swollen 
or  hazy  retina.  Sight  is  dim  and  worse  in  spots  (scotomata).  The 
disorder  usually  involves  first  one  eye  and  then  the  other. 

Albuminuric  retinitis  is  frequent  in  nephritis  (25  to  40  per  cent.). 
Headache  and  loss  of  vision  in  a  middle-aged  to  older  person  are 
the  usual  signs.  There  are  characteristic  changes  in  the  retina  and 
albumin  and  casts  in  the  urine.  Cirrhotic  kidney  is  the  most  fre- 
quently accompanying  somatic  lesion.  The  chief  change  is  an  arterio- 
sclerosis of  the  retinal  vessels.  They  are  unduly  tortuous  and  show 
contractions  and  widenings,  often  being  beaded.  There  is  also  a  trans- 
lucency  in  the  retina,  white  strips  accompany  the  vessels.  The  veins 
are  likewise  tortuous,  and  disturbances  of  circulation  show  particularly 
at  venous-arterial  crossing.  Retinal  edema  with  grayish  opacity 
shows.  Hemorrhage  is  frequent.  The  margins  of  the  disk  become 
obscured,  the  nerve  expanding  into  the  retina  without  sharp  lines  of 
demarcation.  The  disk  may  be  much  swollen,  woolly  in  appearance, 
and  much  extra vasted.  Fatty  degenerations  with  "snow  bank" 
appearances  occur. 

Blindness,  scotomata,  dimness  of  vision  appear  as  in  simple  neuritis, 
but  chronic  cases  of  albuminuric  retinitis  may  be  present  with  little 
loss  of  visual  acuity  in  the  early  stages.  Permanent  impairment  of 
vision  is  the  rule.  Albuminuric  retinitis  accompanied  by  hemorrhages, 
and  fatty  degeneration  of  the  retina,  in  a  patient  over  thirty-five  to 
forty  years  usually  portends  a  fatal  issue  within  comparatively  few 
years. 


PLATE    VI 


Descent  of  a  Form  of  Stationary  Night-blindness.     (Condensed  from  the  Chart  Published    by  Nettleship,  Based  on  Cunier's   Record's  with  Later 

Additions.)       Only  those  Families  which  Contain  Affected  Members  are  Here  Set  Out  in  Detail.       The  Affected    Man    (DR)   Who   Married   the 

Affected  Woman  (DR)  is  Represented  by  the  Sixteenth  Black  Symbol  from  the  Left  in  Generation  VII. 
Black  symbols  show  the  night-blind  individuals,       The    descent   is   always   through    the    affected,    showing  that  the  condition  is  due  to  a  dominant  factor.   ^  According  to  the  records  there  is  a  great 

excess   of   normals  over  the    affected.      It    cannot  be  said  that  the  responsible  factor  is    a   simple    allelomorph.      Nevertheless,    the    table    gives  a  remarkable  illustration    of   the   perma-—    - 

mode  of  descent  of  a  dominant  variety.     (Bateson.) 


DISEASES  OF  THE   VISUAL  APPARATUS 


297 


Syphilitic  retinitis  is  probably  much  more  frequent  than  has  been 
supposed.  It  may  result  from  hereditary  or  acquired  syphilis,  in  the 
latter  case  appearing  soon  after  infection.  Clinically  there  is  contrac- 
tion of  visual  fields,  dimness  of  vision,  maybe  night-blindness,  or 
marked  dimness  of  vision  with  poor  illumination.  Shimmering  lights 
which  are  persistent  and  annoying  occur,  with  micropsia  and  at  times 
metamorphopsia.  Central,  partial  or  complete  scotomata  are  fairly 
constant. 

The  ophthalmoscope  shows  hj-peremia  with  serous  exudation  much 
resembling  the  albuminuric  variety  but  in  milder  degree.  Hemor- 
rhages are  much  rarer,  and  the  "snow  bank"  glistenings  much  less 
pronounced.  Opacity  about  the  disk  is  a  variant  feature,  with  inflam- 
mation of  the  uveal  tract. 


Fig.    148. — Scheme  of  papillomacular  bundle.    (Wilbrand  and  Sanger.) 


Hemorrhagic  retinitis  is  of  importance,  but  the  student  must  be 
referred  to  works  on  ophthalmology  with  the  other  types. 

Retinitis  pigmentosa  is  a  hereditary  variety  of  primary  retinal 
degeneration  showing  night-blindness  with  striking  frequency.  Nettle- 
ship's  famous  study  of  a  French  family  showed  this  to  be  a  striking 
instance  of  Mendelian  dominance.     (Plate  VI.) 

Optic  Nerve. — The  disorders  affecting  the  second  optic  neurons  fall 
into  two  groups:  those  affecting  (A)  the  optic  nerve,  morphologically 
a  true  brain  tract,  (B)  its  terminations  in  the  midbrain  structures. 

A.  Diseases  of  the  Optic  Nerve. — Here  three  situations  need  to  be 
distinguished:  (Ij  whether  the  affection  lies  anterior  to  the  chiasm, 
(2)  whether  it  involves  the  chiasm,  or  (3)  lies  behind  the  chiasm  in  the 
path  of  the  optic  neurons  of  the  third  order. 


298 


CRANIAL  NERVES 


(1)  Diseases  of  the  Optic  Nerve  before  reaching  the  chiasm:  Optic 
Neuritis,  in  general  sense.  Three  types  are  distinguishable  with 
pronounced  symptomatology.  They  are:  (a)  Axial  Neuritis,  (6) 
Interstitial  Peripheral  Neuritis,  and  (c)  Diffuse  Neuritis. 

(a)  Axial  Neuritis. — This  is  a  system  disease  of  the  papillomacular 
bundle,  involving  the  nerve  in  front  of  the  chiasm.  It  may  be  acute 
or  chronic. 

Acute  Axial  Neuritis  occurs  usually  in  young  adults,  twelve  to 
twenty-four  years,  more  particularly  women.  There  appears  sudden 
clouding  or  dimness  of  vision,  and  occasional  photopsias.  A  frontal  or 
temporal  headache,  or  deep  pain  in  the  orbit,  made  worse  by  pressure 
or  movement  of  the  eyeballs,  is  present.  The  loss  of  sight  is  rapid, 
reaching  a  maximum  usually  in  five  days,  and  often  is  so  severe  that  the 
patient  can  just  count  fingers  at  15  feet,  or  is  blind.  With  the  loss  of 
sight  the  headache  lets  up.  There  may  be  no  retinal  changes.  The 
pupil  of  the  affected  eye  is  larger,  and  is  usually  sluggish  to  direct  light 
stimulation,  but  shows  no  consensual  light  reflex  loss. 


Fig.  149. — Central  scotomata  in  acute  axial  neuritis.     (Wilbrand  and  Sanger.) 

After  the  acute  stage  is  over  there  is  gradual  recovery  of  the  sight 
at  the  periphery  with  various  degrees  of  persistent  central  scotomata. 
The  loss  may  be  unilateral  or  bilateral  and  absolute,  or  unilateral  or 
bilateral  to  color  only,  or  various  gradations  of  these  paracentral 
scotomata,  etc.  The  scotomata  gradually  diminish  and  after  six  to 
eight  weeks,  with  proper  therapy,  may  entirely  disappear  (Fig.  149). 

The  fundus  picture  may  remain  normal  throughout  or  show  a 
papillitis.  This  will  depend  upon  how  far  back  of  the  optic  disk  the 
lesion,  which  is  usually  a  vascular  one,  occurred.  When  there  is  a 
papillitis  it  shows  slight  paling  of  the  temporal  half  or  halves  of  the 
fundi  (Fig.  149). 

Etiology. — The  most  frequently  ascribed  cause  is  exposure  to  cold. 
This  is  probably  only  an  incident  to  other  real  causes  such  as  infec- 
tious disease — syphilis,  tuberculosis,  typhoid,  erysipelas,  sinusitis, 
influenza,^  mumps,  pneumonia,  tonsillitis,    cerebrospinal   meningitis, 

*  JeUiffe:  Nervous  and  Mental  Involvementa  of  Influen;;a,  New  York  Med.  Jour., 
November,  1918. 


DISEASES  OF  THE  VISUAL  APPARATUS 


299 


malaria,  beriberi,  etc.,  or  toxemias,  such  as  those  of  pregnancy, 
nephritis,  burns,  CO2  poisoning,  methyl  alcohol,  quinine,  felix  mas, 
morphin,  etc. 

Treatment. — Hot  baths,  and  treatment  of  cause,  as   antisyphilitic  in 
syphilis,  etc. 


Fig.   150. — Scotoma  for  red  and  green  in  tobacco  axial  neuritis.      (Wilbrand  and  Sanger.) 

The  Chronic  Form  is  much  more  frequent.  It  is  the  classical  situa- 
tion in  chronic  alcohol  or  nicotine  poisoning,  and  affects  males  more 
often.  Here  the  course  is  a  chronic  one,  spreading  over  several 
months  or  years.  The  blindness  appears  slowly,  and  at  first  consists 
of  a  central  scotoma  for  colors,  or  of  a  hemeralopia,  the  patient  seeing 
better  in  the  dusk  than  in  the  bright  light  (fatigue).  The  scotomata 
become  more  marked  if  the  poisoning  continues  (Fig.  150). 

The  type  of  scotoma  varies  widely.  Bilateral,  fairly  symmetrical, 
oval  scotomata  for  red  and  green,  lying  between  the  blind  spot  and 


Fig.  151. 


-Beginning  scotoma  for  red  at  the  onset  of  a  tobacco  or  alcoholic  axial 
neuritis.     (Wilbrand  and  Sanger.) 


the  fixation  point  is  the  early  picture.  It  usually  starts  as  a  defect 
for  red,  stretching  toward  the  blind  spot  (Fig.  151).  The  chief  defect 
usually  lies  about  2°  to  8°  from  the  fixation  point.  Absolute  central 
scotomata  are  rare. 

The  acuity  of  vision  is  usually  diminished,  and  more  on  one  side  than 
the  other.  In  monocular  reading  the  type  to  the  right  of  the  fixation 
point  is  not  clear  for  the  right  eye,  while  for  the  left  eye  the  defect  lies 


300 


CRANIAL  NERVES 


to  the  left.    The  defect  in  vision  bears  httle  direct  relation  to  the  size 
of  the  scotomata.    Pupillary  anomalies,  diminution  of  both  light  and 

accommodation  reflexes  and  pseudo- 
tabetic  pictures  are  to  be  found. 

The  fundus  picture  may  be  nor- 
mal with  gross  defect  in  vision  and 
large  scotomata,  or  there  may  be 
hyperemia,  a  mild  neuritis,  with 
some  temporal  pallor.  If  marked 
pallor  is  present  it  speaks  in  general 
for  a  more  severe  process. 

The  course  is  chronic.  Removal 
of  the  cause  may  save  the  sight, 
but  slight  scotomata  may  persist. 
Atrophy  is  the  end  in  the  severe 
cases. 

Pathology. — -The  changes  in  both 
acute  and  chronic  types  occur  in 
the  papillomacular  bundle.  They 
are  degenerative  rather  than  in- 
flammatory changes,  with  greatest 
severity  in  the  optic  canal,  because 
of  the  richer  vascular  supply  of  the 
nerve  trunk  at  this  point. 

Etiology. — The  chief  causes  are 
alcohol  and  tobacco.  Other  less 
frequent  poisonings  are  by  carbon 
bisulphide — in  the  rubber  indus- 
tries— arsenic,  lead,  sulphuric  acid, 
anemia  (anchylostoma),  diabetes, 
aniline,  gasoline. 

Hereditary  Forms. — These  oc- 
cupy intermediary  positions.  One 
type  affects  usually  the  males, 
passing  through  unaffected  fe- 
males (Knight's  move  in  heredity) . 
These  develop  acute  or  subacute 
headaches  about  the  age  of  twenty, 
with  gradually  diminishing  vision. 
Unequal  bilateral  scotomata  ap- 
pear and  gradually  increasing  cen- 
tral blindness.  There  is  usually 
a  subacute  and  progressive  stage 
for  about  six  months,  one  eye 
lagging  behind  the  other  in  its 
deterioration. 

Gradually    increasing    central    scotomata    show.     Nyctalopia    is 
frequent.     The  marginal  vision  is  retained,  save  for  colors.     Total 


Fig.  152. — Course  of  the  papillomac- 
ular bundle  at  different  points  in  the 
optic  tract.     (Wilbrand  and  Sanger.) 


PLATE    VII 


Fig.   1 


Normal  Eye-ground  (average  tint).     (Norris  and  Oliver.) 


Fig.  2 


Ophthalmoscopic  Appearances  in  Early  Stage  of  Optic  Neuritis. 

(Norris  and  Oliver.) 


DISEASES  OF  THE  VISUAL  APPARATUS 


301 


blindness  develops  in  about  two-thirds  of  the  cases.  One-third  remain 
more  or  less  stationary  or  even  improve.  In  mild  cases  central  color 
scotomata  alone  appear,  or  there  is  diffuse  loss  of  color  sense  without 
scotomata. 

The  fundus  picture  is  variable,  but  the  presence  of  atrophic  changes, 
usually  temporal,  stands  in  marked  contrast  with  any  other  changes 
in  the  nervous  system. 

Hereditary  sAphilis  plays  a  role  in  some  cases.  In  others  there  is 
an  anomaly  in  the  germ  plasm. 

(6)  Interstitial  Peripheral  Neuritis. — Here  the  disease  involves  the 
periphery  of  the  optic  nerve  rather  than  the  central  or  eccentrically 
lying  papiliomacular  bundle.  Concentric  limitation  of  the  field  of 
vision  for  white  and  colors  is  the  chief  finding  rather  than  central 
scotomata.  Here  there  is  a  peripheral  inflammation  of  the  nerve 
trunk,  starting  in  the  pia  and  proceeding  inward  in  the  septa. 


Fig.  15.3. — Visual  fields  in  a  patient  with  hereditary  axial  neuritis.  Fields  for  white 
normal,  for  blue ,  and  for  red ,  concentrically  contracted.  Absolute  cen- 
tral scotoma  with  larger  bordering  scotoma  for  blue  and  red.     (Wilbrand  and  Sanger.) 


The  concentric  limitation  of  vision  is  rarely  observed  in  the  beginning. 
As  it  slowly  advances  the  patients  become  uncertain  of  space  localiza- 
tion and  need  to  turn  the  eyes  frequently  to  get  clear  pictures  of 
the  surroundings.    Central  vision  is  usually  sharp  even  for  color. 

The  fundi  show  simple  or  neuritic  atrophy,  occasionally  choked 
disks.    Very  variable  fields  are  observed  (Figs.  145,  147,  148  and  149). 

Hysterical  limitation  of  the  field  is  to  be  considered  here.  Normal 
fundi  are  consistent  with  a  true  interstitial  neuritis,  since  lesions  lying 
far  back  in  the  nerve  trunk  may  cause  little  or  no  disk  changes.  A 
psychoanalytic  anamnesis  usually  will  clear  up  the  diagnosis  of  a 
possible  hysteria.  Tabes  with  neuritis  may  begin  as  an  interstitial 
neuritis.    The  cytobiological  finding.s  will  establish  the  diagnosis. 

FAiology. — Syphilitic  meningitis  of  the  base  is  the  most  frequent 
cause.  A  negative  Wassermann  in  this  group  of  patients  is  not  a  just 
criterion  to  deny  specific  medication.  Other  etiological  factors  are 
measles,  dij)htlu'ria,  influenza,  myelitis,  gonorrhea,  sinusitis,  typhoid, 
lead,  arsenic,  diabetes,  leptomeningitis,  cerebrospinal  and  tuberculous 
meningitis. 


302 


CRANIAL  NERVES 


Fig.   157 

Figs.   154,  155,  156  and  157. — Visual  fields  in  cases  of  interstitial  peripheral  optic  neuritis 

from  syphilis.     (Wilbrand  and  Sanger.) 


DISEASES  OF  THE  VISUAL  APPARATUS 


303 


The  therapy  is  causal,  usually  specific.  Arsenic  or  salvarsan  or  similar 
synthetic  is  less  to  be  feared  than  an  active  sjT)hilis.  Many  so-called 
neurorecidives  causing  blindness  are  due  more  to  the  s>T)hilis  than  to 


Fig.  158. — Visual  field  with  diflfuse  neuritis  due  to  multiple  sclerosis.  On  right  side  a 
temporal  hemianopic  color  scotoma,  on  the  left  intermittently  appearing  hemianopic 
uncertainty  scotoma.     (Wilbrand  and  Sanger.) 


Fig.  159.- 


-Visual  field  showing  atrophy  in  tabes.    Field  for  blue 
(Wilbrand  and  Sanger.) 


for  red 


Fig.   160. — Optic  atrophy  in  tabes  with  loss  of  color  discrimination. 
(Wilbrand  and  Sanger.) 


the  arsenic  content  of  the  drug.    It  must  be  remembered,  however, 
that  arsenic  is  capable  of  producing  an  interstitial  optic  neuritis. 

(c)  Diffuse  Neuritis. — Here  the  inflammatory  ])r()cess   involves  the 
entire  nerve  tract,  resulting  in  marked  amblyopia  or  blindness.     It 


304 


CRANIAL  NERVES 


may  be  implicated  locally  or  throughout  its  entire  course  from  the 
retina  to  the  chiasm.  In  acute  myelitis  at  times  this  severe  inflam- 
mation occurs  as  a  complication.  Infectious  diseases  are  important. 
Influenza  here  plays  almost  as  large  a  role  as  syphilis.^ 

The  infections  and  toxemias  mentioned  in  the  preceding  paragraphs 
may  also  induce  a  total  optic  neuritis.  Malaria,  scarlet  fever,  yellow 
fever,  erysipelas  may  be  added  to  the  causes.  Orbital  sinus  disease 
is  important,  and  also  multiple  sclerosis  (Fig.  151). 

Other  atrophic  states,  double,  one-sided,  total  or  partial,  occur, 
either  in  the  papillse,  from  pressure  of  a  glaucoma,  or  may  be  descending 
atrophies  from  higher  lying  causes  such  as  brain  tumor,  hydrocephalus. 
Primary  progressive  atrophy,  arising  by  itself,  probably  rarely  occurs. 
The  most  suggestive  cause  of  an  isolated,  bilateral,  progressive  optic 
atrophy  without  other  tangible  neurological  signs  is  tabes.  Cyto- 
biological  tests  will  complete  the  diagnosis  (Figs.  159  and  160.) 


Fig.   161. — Quadrant  hemianopsia  of  lower  right  segment  due  to  hemorrhagic  destruction 
within  the  external  geniculate.     (See  following  figures.) 


B.  Disease  at  or  about  the  Chiasm. — The  anatomical  peculiarities,  due 
to  the  crossing  of  the  fibers  at  the  chiasm,  introduces  certain  definite 
signs  which  are  of  value.  Scotomata  and  concentric i.4iniitation  are 
replaced  by  hemianopsias  of  varying  type.  "-'  \ 

In  lesions  in  front  of  the  chiasm  bitemporal  hemianopsia  will  be 
present.  This  is  rare.  A  lesion  behind  the  chiasm,  usually  in  the 
sella  turcica,  and  not  infrequent,  as  in  pituitary  disease,  causes  a 
binasal  hemianopsia,  partial  or  complete.  Lesions  to  the  right  or 
left  of  the  chiasm  will  cause  incomplete  homonymous  hemianopsias — 
whereas  lesions  in  the  tract  back  of  the  chiasm — i.  e.,  in  the  midbrain 
or  optic  radiations  or  occipital  lobes  will  cause  a  usually  more  complete 
homonymous  hemianopsia. 

Horizontal  hemianopsias,  either  superior  or  inferior,  occur  in  chiasm 
lesions  from  pressure  above  or  below.     They  are   readily   explained 

1  Jelliffe:  Loc.  cit. 


DISEASES  OF  THE   VISUAL  APPARATUS 


305 


from  the  position  of  the  crossing  fibers  in  the  chiasm.     Such   hemi- 
anopsias may  rarely  occm-  from  retinal  causes. 

A  common  cause  for  chiasm  changes  is  pituitary  tumor.  Syphilis, 
however,  is  specially  frequent  in  just  this  situation,  most  basal  s\T)hilitic 
meningitic  processes  beginning  here.  The  process  spreading  forward 
to  the  optic  stem  produces  a  multiplicity  of  field  changes.  Thus  one 
may  have  partial  scotomata,  monocular  temporal  hemianopsia,  bitem- 
poral hemianopsia  (the  most  frequent),  temporal  hemianopsia  with 
blindness  of  one  eye,  blindness  in  one  eye  and  nasal  hemianopsias 
of  the  other,  blindness  in  both  eyes.  This  very  great  irregularity 
and  changeability,  advancing  or  receding  under  treatment,  is  of 
much  importance  in  excluding  a  pituitary  tumor.  A  loss  of  the 
hemiopic  pupillary  reaction  is  of  importance  in  making  a  definite 
localizing  diagnosis. 


&^C^sla 


Fig.  1 62. — Site  of  lesion  in  external  geniculate  giving  rise  to  quadrant  hemianopsia  seen 
in  Fig.  161  and  the  secondary  degenerations  in  Fig.  163.  C.  gen.  exL,  external  geniculate; 
hcem,  hemorrhage;  C.i.,  internal  capsule;  F.S.,  fissure  of  Sylvius;  S.S.,  optic  radiations. 

The  pupillary  changes  are  variable.  Other  signs  of  basal  syphilitic 
menmgitis  are  discussed  in  the  chapter  on  Cerebral  S}T)hilis  {q.  v.). 

Affections  of  the  chiasm  are  more  rarely  encountered  as  a  result  of 
trauma,  brain  tumor  with  general  pressure,  cavernous  sinus  disease, 
cerebrospinal  and  tuberculous  meningitis,  bone  disease,  aneurism  and 
arteriosclerosis  of  the  carotids. 

(\  Thalamus  Disease.— In  lesions  of  the  optic  tract  posterior  to  the 
pulvinar,  i.  e.,  in  the  optic  neuron  of  the  fom*tli  order,  pupillary  dis- 
turbances are  absent.  Thus  Wilbrand  and  Wernicke  (three  years  later) 
ha^'e  shown  that  by  careful  illumination  of  the  blind  side  of  the  eye  one 
can  distinguish  between  hemianopsia  in  the  optic  neuron  of  the 
20 


306 


CRANIAL  NERVES 


third  order  (by  loss  of  pupillary  light  reflex)  and  a  hemianopsia  of  the 
optic  neuron  of  the  fourth  order  (intact  hemiopic  pupillary  reflex). 
As  a  matter  of  fact  this  test  is  extremely  difficult  to  perform,  but 
fortunately  lesions  in  the  midbrain — end-station  of  optic  neuron  of  the 
third  order — are  almost  invariably  accompanied  by  other  sensory  signs 
(usually  a  complete  or  incomplete  thalamic  syndrome,  q.  v.),  thus  aiding 
in  the  diagnosis.  The  distributions  of  the  optic  neurons  of  the  third 
order  are  multiform.  Some  fibers  end  in  the  corpora  quadrigemina. 
Lesions  here  cause  pupillary  changes;  others  end  in  the  thalamus 
(pulvinar),  and  their  involvement  causes  no  disturbances  of  vision. 
The  majority  of  the  fibers  form  their  synapses  in  the  external  geniculate 


6i//i-. 


Fig.  163. — Showing  atrophic  degenerations  in  optic  radiations  (air.)  from  hemorrhage 
in  external  geniculate  (Fig.  162),  giving  rise  to  quadrant  hemianopsia  of  Fig.  161.  Cun., 
cuneus;  Calc,  calcarine  fissure;  f.l.i.,  inferior  longitudinal  fasciciilus;  I,  left  hemisphere; 
atr.,  atrophy.     (Henschen.) 


bodies.  These  form  rich  collateral  associations  with  the  auditory  tract, 
the  sensory  tracts,  the  three  or  four  oculomotor  nuclei  and  through  the 
median  longitudinal  fasciculus  with  the  synapses  for  the  cranial  and 
spinal  muscle  fibers.  (See  Plate  VIII).  Hence  lesions  of  the  optic  tract 
in  the  geniculate  region  cause  not  only  homonymous  hemianopsias 
often  only  quadrant,  but  they  are  also  liable  to  be  complicated  by  the 
involvement  of  these  other  near-lying  structures.  Isolated  quadrant 
hemianopsias  may  result  from  small  hemorrhages,  thrombi,  emboli, 
tumors,  or  encephalitis  (poliomyelitis — rare)  in  the  external  geniculate 
as  well  as  from  lesion  farther  back  in  the  tractus  (Figs.  161,  162  and 
163). 


DISEASES  OF  THE  VISUAL  APPARATUS 


307 


D.  Cortex  Diseases. — ^Lesions  of  the  end  stations  of  the  optic  tract  or 
its  associated  areas  in  the  occipital  lobe  may  cause    mind-blindness, 


Fig.   164. — Showing  loss  of  vision  in  lower  left  quadrant  from  bullet  wound  just  above 
level  of  right  calcarine  fissure.     (Lister  and  Holmes.) 


I'll;.  165. — Homonymous  hemianopsia  with  central  scotoma  from  lesion  of  left  opt  it- 
radiations  (occipital  lobe)  of  one  side  and  tip  of  occipital  pole  of  other  side.  (Lister 
and  Holmes.) 


308 


CRANIAL  NERVES 


i.  e.,  optic  agnosia.  Here  the  patient  may  have  no  disturbance  of  sight, 
or  he  may  have  partial  hemianopsia,  but  is  unable  to  recognize  words 
or  objects  previously  known,  speech  being  intact. 


DISEASES  OF  THE  OCVLOMOTOR  NERVES  309 

Visual  hallucinations  are  present  in  disorder  of  the  optic  end-stations 
in  the  occipital  lobe.  When  they  show  definite  projections  in  space 
one  can  make  an  approximate  localization  of  the  portion  of  the  lobe 
involved.  This  may  be  of  value  in  determining  the  site  of  a  tumor 
or  abscess  formation. 

The  chief  arterial  supply  of  the  posterior  nem-ons  is  drawn  from 
the  calcarine  branch  of  the  posterior  cerebral.  The  most  occipital 
portion  is  supplied  by  the  median  cerebral.  The  anterior  cerebral 
sends  branches  which  innervate  the  optic  radiations  just  posterior 
to  the  corpus  callosum,  but  lesions  of  this  artery  at  this  place  cause  no 
definitely  recognizable  lesions. 

Recent  experiences  in  war  surgery  are  permitting  a  more  compre- 
hensive mapping  of  the  cortical  representation  of  the  different  parts  of 
the  retina.  That  of  the  macula  in  particular  has  been  specially  studied 
by  Henschen,  Bolton,  Inouye  and  Lenz.  The  studies  of  Lister  and 
Holmes^  support  Henschen's  and  Inouye's  view  that  in  lower  quad- 
rant hemianopsias  of  cortical  origin  from  gunshot  wounds  the  upper 
halves  of  the  retinae  are  represented  in  the  upper  lips  of  the  cal- 
carine fissure  (Fig.  164).  In  cases  of  homonymous  hemianopsia 
with  central  scotomata  the  penetrating  lesions  caused  injiu'y  to 
the  occipital  lobes  or  optic  radiations  of  one  side  and  passed  through 
or  near  the  tip  of  the  occipital  pole  of  the  opposite  hemisphere 
(Fig.  165).  Central  vision  is  probably  represented  on  either  the  mesial 
or  the  lateral  surface  of  the  posterior  poles  of  the  occipital  lobes.  The 
macular  fibers  are  probably  not  represented  bilaterally  and  are  cor- 
tically  localized  in  the  posterior  limits  of  the  visual  areas,  probably  on 
the  margins  and  the  lateral  surfaces  of  the  occipital  lobes.  It  is  prob- 
ably because  there  is  an  overlapping  of  the  middle  and  posterior  cerebral 
arterial  supply  in  this  region  that  cerebral  hemorrhage  with  visual 
defect  so  rarely  involves  the  macular  regions. 

Annular  scotomata  are  among  the  rarer  anomalies  seen  in  occipital 
lesions. 

For  further  discussion  of  the  cortical  disturbances  of  vision  from 
lesions  of  the  temporal  or  occipital  lobes  see  chapters  on  Aphasia, 
Syphilis  of  the  Brain  (Paresis),  Brain  Tumor,  Hemiplegia,  Thrombosis, 
Arteriosclerosis,  etc.^ 

m.  DISEASES  OF  THE  OCULOMOTOR  NERVES. 

Ocular  Nerves:  Third,  Fourth,  Sixth. — Disorders  of  the  functions 
of  these  nerves  are  best  discussed  under  a  general  head,  since  the  usual 
ocular  palsies  are  often  complex  syndromes  in  which  one  or  more  of 
these  nerves  are  involved. 

'  See  Lister  and  Holmes:  Disturbances  in  Vision  from  Cerebral  Lesions,  Proc.  Roy. 
Sec,  Sect,  on  Ophthalmology,  March  22,  1916,  one  of  the  many  illuminating  papers  on 
this  topic.  (See  also  Brain,  1916,  vol.  xxxix,  parts  1,  2.  Also  Villaret  et  Beaulieu,  Ann. 
de  M^d.,  1918,  v. 

'  See  Wilbrand  and  Sanger  and  Henschen  in  Lewandowsky's  Handbuch,  vol.  iii. 


310 


CRANIAL  NERVES 


The  third  nerve  is  a  motor  nerve  for  all  of  the  muscles  of  the  eye- 
ball, save  the  external  rectus,  and  the  superior  oblique,  which  latter 
received  its  motor  fibers  from  the  sixth  and  fourth  nerves  respectively. 
The  third  nerve  also  supplies  the  levator  palpebrse,  the  ciliary  muscle 
and  the  contracting  fibers  of  the  pupil.  The  dilating  fibers  of  the 
pupil  receive  a  branch  from  the  sympathetic.  Deep  sensibility  fibers 
also  pass  in  the  motor  roots. ^ 

Third  Nerve  Palsies. — These  are  often  very  complicated  and  may 
be  central  or  peripheral,  complete  or  partial.  Complete  paralysis  of 
both  third  nerves  is  rare,  partial  palsies  are  the  rule.  Unilateral 
palsy  of  all  of  the  external  muscles  governed  by  the  third  nerve  (often 
termed  ophthalmoplegia  externa)  is  due  usually  only  to  a  lesion 
involving  the  second  or  peripheral  motor  neurons  of  the  third  nerve. 

Bilateral  external  ophthalmo- 
plegia may  occur  also  from  lesions 
of  the  mesencephalon  and  cortical 
oculomotor  paths.  The  chief 
causes  for  central  palsies  are 
various  types  of  encephalitis, 
polioencephalitis,  either  infectious 
poliomyelitis,  influenza^  or  toxic 
as  in  alcoholism  (Wernicke's 
polioencephalitis  superior) .  Syphi- 
litic tlu'ombosis  may  cut  off  the 
blood  supply  of  the  nuclei.  Pres- 
sure from  the  aqueduct  above,  or 
third  ventricle  may  cause  pressure 
palsies,  usually  of  irregular  dis- 
tribution. (Nothnagel's  Syn- 
drome.    Pineal  Syndrome.) 

Peripheral  palsies  are  more 
frequently  due  to  disease  at  the 
base,  usually  basal  syphilitic 
meningitis,  tumor,  tuberculosis, 
hemorrhage,  traumas  (rarely),  or 
are  occasioned  by  involvement  of  the  fibers  as  they  pass  through 
and  about  the  red  nucleus  by  tumor,  multiple  sclerosis,  or  when 
implicated  in  a  thrombotic  or  hemorrhagic  softening  of  the  cere- 
bral peduncle — Millard-Gubler,  Benedict,  Fovilles'  syndromes,  red 
nucleus  syndromes.  (See  chapters  on  Midbrain  for  description  of 
these  syndromes,  also  see  Fig.  168.)  Infectious  disease  neuritis 
may  also  occasion  peripheral  palsies.  Pressure  from  aneurism  of 
the  internal  carotid,  and  thrombosis  of  the  cerebral  sinuses  (sinus 
cavernosus)  may  also  cause  peripheral  palsies.  A  special  herpes  zoster 
ophthalmicus  is  known.  Exophthalmic  goiter  and  diabetes  are  special 
causes.  Transitory  third  nerve  palsies  occur  in  the  disorder  known  as 
ophthalmoplegic  migraine  {q.  v.). 


Fig.  167. — Cerel:)ral  syphilis  ptosis 
nerve  palsy. 


Third 


1  Sherrington  and  Tozer:  Proc.  Royal  Soc. 
"^  Jelliffe:  Loc.  cit. 


1910. 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


311 


«<r,/^C 


Fig.  168. — Fovilles'  syndromes,  with  anterior  and  posterior  pontine  syndromes. 
Hemiplegia,  cerebral  type,  with  (a)  conjugate  deviation  of  the  head  and  eyes,  (h)  by 
lesions  of  the  upper  portion  of  the  pons,  right  side,  involving  the  anterior  portion  of 
the  pons  and  the  region  of  the  tegmentum.  On  the  left  side  there  is  a  contralateral  hemi- 
plegia of  the  limbs,  of  the  lower  part  of  the  face  and  of  the  tongue,  because  of  the  involve- 
ment of  the  pontine  pj-ramidal  fibers  Py  (corticospinal  pj-ramidal  fibers,  corticonuclear 
facial  and  hypoglossal  fibers).  In  C,  right-hand  figure,  there  is  a  single  lesion  which 
involves  the  tegmentum  at  its  antero-internal  angle  and  destroys  the  head-turning  (cepha- 
logyric)  and  eye  turning  (oculogyric)  fibers  of  the  right  side  which  at  this  level  are  situated 
in  the  pes  lemniscus  and  the  internal  portion  of  the  median  fillet  giving  rise  to  conjugate 
deviation  of  the  head  and  of  the  eyes.  By  reason  of  the  predominant  action  of  the  antago- 
nists the  head  is  inclined  to  the  right  and  the  eyes  look  to  the  right,  the  patient  looks  to  the 
side  of  the  lesion.  In  a  there  are  multiple  isolated  lesions.  Four  large  foci  in  the  anterior 
portion  destroy  the  pontine  pyramidal  fibers  with  a  resulting  crossed  contralateral  hemi- 
plegia of  the  extremities,  the  face,  and  the  tongue.  Another  focus  occupies  the  posterior 
internal  portion  of  the  tegmentum  and  destroys  the  internuclear  oculogyric  fibers  of 
the  posterior  longitudinal  bundle  which  directly  unites  the  nuclei  of  the  sixth  and  third 
nerves  and  vice  versa.  There  results  a  paralysis  of  the  eyeballs  by  which  they  cannot 
turn  sidewi.se  toward  the  right — right  oculorotarj-  paralysis — by  reason  of  the  predomi- 
nance of  the  antagom'sts  the  patient  looks  to  the  left.  The  patient  looks  away  from  the 
lesion  toward  the  paralyzed  members.  The  cortical  oculorotary  fibers  and  the  pes  lem- 
niscus are  intact.  For  details  of  structure  and  abbreviations,  see  chapter  on  Midbrain 
Lesions.     (Dejerine.) 


312 


CRANIAL  NERVES 


Syndromes. — The  compound  character  of  the  nuclei  and  the  loosely 
arranged  bundles  making  up  the  nerve  explain  the  great  range  in 
symptomatology.  Obersteiner  (5th  edition,  19 12)  follows  Bernheimer 
chiefly  in  his  teaching  regarding  the  complicated  question  of  the  local- 
ization of  the  brain-stem  nuclei.^  Thus  it  will  be  seen  that  from  before 
backward  the  nuclei  are  arranged  as  follows:  Levator  palpebrae,  rectus 
superior,  rectus  internus,  obliquus  inferior,  rectus  inferior,  trochlearis. 

A  complete  unilateral  palsy,  probably  nuclear  (ophthalmoplegia 
completa),  would  then  cause  ptosis,  wrinkling  of  forehead  on  same 
side  (from  effort  to  overcome  ptosis  by  occipitofrontalis),  wide  pupil 
from  involvement  of  the  Edinger- Wesphal  nucleus),  irresponsive  to 
light  and  accommodation,  eye  turned  outward  and  slightly  downward. 
Double  vision  is  present  and  some  dizziness  in  the  early  stages.  A 
variety  of  individual  muscle  palsies  may  also  result  from  either  nuclear 


Fig.   169. — Scheme  of  oculomotor  nuclei  Fig.   170. — Scheme  of  oculomotor  nuclei, 
modified  from  Bernheimer.    Basal  projec-  modified  after  Bernheimer.  Sagittal  projec- 
tion.   M,  median  nucleus;  E.W.,  Edinger-  tion.    Lettering  as  before.     (Obersteiner.) 
Westphal  sympathetic  nucleus. 

or  peripheral  involvement  as  indicated — ophthalmoplegia  externa, 
when  the  pupil  is  not  involved;  ophthalmoplegia  interna  when  only  the 
internal  muscles  are  involved — a  rare  condition. 

The  distinction  of  nuclear  from  peripheral  palsies  is  usually  made 
on  the  basis  of  accompanying  symptoms — sensory  or  motor,  due 
to  implication  of  the  red  nucleus,  or  of  the  cerebral  peduncles.  In 
the  absence  of  these  accessory  symptoms  (Weber-Gubler,  Benedict 
s\iidromes,  rubrospinal  sjaidromes)  the  distinction  may  be  impossible. 
There  is  no  single  disease  process  to  which  the  term  ophthalmoplegia 
may  be  rigidly  applied.  Hence  there  is  no  general  course  and  no 
general  treatment.  The  various  palsies  must  be  interpreted  on  the 
basis  of  the  dynamic  factors,  and  the  treatment  must  be  foimded 
upon  the  causation.     Syphilis  is  responsible  for  the  majority  of  these 


1  See  Kidd,  Rev.  Neu.  and  Psych.,  xi,  507. 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


313 


Fig.  171. — Pontine  syndrome,  with  eye  palsies  of  central  origin  and  syringomyelic  dis- 
sociation. There  is  here  a  crossed  hemianesthesia  wath  alternating  paralysis  of  the 
VI  and  VII  cranial  nerves,  anesthesia  of  the  V  nerve  due  to  hemorrhage  in  the  lateral 
and  lower  portion  of  the  pontine  tegmentum  of  the  left  side.  The  right-hand  figure 
shows  the  hemianesthesia,  dissociated  as  in  syringomyelia  (hemianalgesia  and  hemi- 
thermanesthesia  due  to  lesion  of  the  crossed  sensory  pathways  of  the  lateral  portion  of 
the  reticular  formation.  There  is  preservation  of  the  tactile  and  postural  sensibilities 
and  of  the  stereognostic  sense,  because  of  the  incomplete  extension  of  the  lesion  to  the 
median  lemniscus  (Rin).  The  left-haiid  figure  shows  (1)  atrophic  paralysis  of  the  VII 
nerve  with  reaction  of  degeneration,  lagophthalmia,  drooping  of  the  lips,  loss  of  facial 
mimicry,  paralysis  of  the  entire  left  facial  (VII)  indicated  (a);  (2)  anesthesia  of  the 
face,  following  involvement  of  the  descending  root  of  the  trigeminus  (.see  V  on  a') ; 
(3)  paralysis  of  the  external  rectus  with  convergent  strabismus  by  reason  of  the  over- 
action  of  the  antagonists.  Furthermore,  there  is  a  paralysis  of  the  lateral  movements 
of  the  eyeballs  toward  the  left  notwithstanding  the  integrity  of  the  posterior  longitudinal 
fasciculus  (Flp.),  of  the  nucleus  of  the  VI  and  of  the  adjacent  reticular  formation. 
The  lesion  of  Deiters'  nucleus,  and  of  the  labyrinthine  oculorotary  fibers  which  unite 
Deiters'  nucleus  (ND)  to  the  nuclei  of  the  III  and  VI  causes  this.  By  reason  of  the 
overaction  of  the  antagonists  the  patient  looks  to  the  right.  (After  Dejerine.)  For 
abbreviations  of  the  anatomical  sketch  see  section  on  Midbrain. 


314 


CRANIAL  NERVES 


palsies,  and  calls  for  verification  by  the  cytobiological  tests  and  prompt 
antisx'philitic  treatment,  best  by  arsphenamine  and  hypodermic  injec- 
tion of  mercury.     (See  chapter  on  Syphilis  of  the  Nervous  System.) 

Isolated  involvement  of  the  pupillary  apparatus  may  be  discussed 
here.  Contracted  pupils,  irregular  pupils,  unequal  pupils,  dilated 
pupils,  etc.,  have  been  discussed  (see  Symptomatology).  A  dilated 
pupil  with  loss  of  accommodation  reflex  is  infrequently  seen  in  severe 
alcoholism  (Korsakov's  syndrome).  It  may  be  present  also  in  optic 
nerve  disease — combined  with  loss  of  right  reflex  as  well.  A  loss  of 
light  reflex  with  preservation  of  the  accommodation  reflex'  (reflex 
iridoplegia,  x\rgyll-Robertson  pupil)  is  a  frequent  sign  of  syphilis.  Its 
mechanism  has  been  discussed.     It  is  often  unilateral  in  the  begin- 


FiG.  172. — Third  nerve  paralysis. 
and  external  strabismus. 


Ptosis 


Fig.    173. — Cerebral  syphilis.     Exter- 
nal rectus  palsy,  right  eye. 


ning  of  a  tabes  or  paresis,  or  other  type  of  cerebrospinal  syphilis  and 
may  occur  in  a  number  of  other  conditions,  though  rarely.  In  cerebral 
syphilis  it  usually  becomes  double. 

Isolated  ptosis  may  also  be  seen.  This  may  be  due  to  oculomotor 
invasion  (tabes),  or  it  may  be  hysterical.  In  the  former  case  there 
is  usually  a  compensatory  overaction  of  the  corrugators,  in  the  latter 
this  is  less  likely  to  take  place.  Sympathetic  disease,  ptosis  from 
palsy  of  Midler's  muscle,  is  usually  accompanied  by  pupillary  disturb- 
ances and  the  eyeball  lies  somewhat  retracted  in  the  eyeball  cavity. 
The  cervical  sympathetic  is  here  implicated. 

Hypermetropia  is  the  usual  condition  of  the  child  at  birth.  At 
three  emmetropic  vision  is  the  rule.  Persistence  of  the  hypermetropia 
is  a  vegative  neurological  sign  and  is  frequent  in  vagotonic  individuals. 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


315 


^rog^essi^'e  myopias  and  hyperiretropias  may  have  psychogenic 
causes  and  such  individuals  must  often  see  the  world  differently  from 
others. 

Chronic  Progressive  Eye  Palsies. — These  make  up  a  special  group, 
occasionally  congenital,  more  often  they  are  a  part  of  a  progressive 
anterior  poliomyelitis.     (Bulbar  pais}',  q.  v.) 

Fourth  Nerve  Palsy.— The  fourth  (trochlearis)  ner\e  supplies  the 
superior  oblique  muscle  with  its  motor  fibers.  Afferent  fibers  carry 
deep  sensibility  fibers  from  the  muscle.  The  fibers  are  crossed  and 
uncrossed,  the  latter  being  phylogenetically  the  first  to  appear,  but 
later  are  overshadowed  by  the  crossed  fibers.  Isolated  palsy  causes 
a  marked  diplopia,  and  some  dizziness  when  the  patient  looks  down- 
ward and  outward.  The  false  image  stands  lower  and  nearer  than 
the  true  one,  its  upper  end  inclined  toward  the  true  image.     Looking 


Fig.  174. — Paralysis  ol  the  fourth  nerve. 
Inability  to  look  down.  It  will  be  noted 
that  as  the  eyeball  does  not  turn  down- 
ward the  eyelid  does  not  descend;  at  the 
same  time  the  patient  can  close  his  eyes 
when  told  to  do  so.     (Russell.) 


Fig.  17.5. — Paralysis  of  the  fourth 
nerve.  The  same  patient  closing  his 
ej'es    at    command.     (Russell.) 


upward  or  downward  causes  no  diplopia.  These  patients  have  diffi- 
culty in  descending  stairs,  and  they  incline  the  head  forward  and 
toward  the  sound  side  to  adjust  to  their  diplopia. 

The  fourth  ne^^'e  is  frequently  in^'olved  with  the  third  and  sixth 
in  basal  inflammations  or  new  growths,  or  may  be  involved  inde- 
pendently from  pressure  in  the  posterior  fossa  (cerebellar  tumor).  It 
also  is  involved  at  its  nuclear  origin  from  encephalitis,  poliomyelitis, 
toxemias,  etc.     (Figs.  174  and  175.) 

Sixth  Nerve  Palsy.— This  is  perhaps  the  most  frequent  of  the  eye 
palsies.  The  peripheral  motor  neuron  is  exposed  for  three-fourths  of 
an  inch  or  more  on  the  base  of  the  skull,  and  is  therefore  subjected 
to  greater  possibility  of  local  pressure  than  any  other  cranial  nerve. 
When  the  external  rectus  muscle  is  paralyzed  there  is  an  internal 
strabismus  and  a  slightly  smaller  pupil  in  the  afi'ected  eye.  There  is 
also  a  diplopia. 


316 


CRANIAL  NERVES 


Sixth  nerve  palsy  more  usually  results  from  basilar  disease,  fracture 
of  base,  meningitis  (s^-philitic  and  other  types),  tumors  not  only  of 
the  base  but  also  of  the  brain  substance  itself.  Nuclear  involvement 
is  seen  in  encephalitis,  poliomyelitis,  toxemias,  etc.  External  rectus 
palsy  as  a  result  of  a  myositis  is  not  unknown.  It  may  also  be  a 
complication  of  severe  migraine  (periodic  palsy). 

Central  Motor  Neurons. — Isolated  eye  palsies  are  due  to  nuclear 
or  to  peripheral  involvement  of  the  third,  fourth  and  sixth  nerves. 
Supranuclear  disease  of  the  oculomotor  paths  does  not  result  in  the 
loss  of  function  of  a  single  eye,  much  less  of  a  single  eye  muscle.  Such 
lesions  between  the  oculomotor  cortex  and  the  nuclei  in  the  midbrain 
cause  complicated  disorders  of  the  associated  morements  of  the  eyes. 


Fig.   176. — Paralysis  of  upward  movement  ol  the  eyes,  showing  the  excessive  wrinkling 
of  the  forehead  in  the  attempt  to  look  up.    Skew  deviation.     (Holmes.) 


The  most  frequent  of  these  are:  (a)  conjugate  deviation,  (6)  lateral 
associated  palsy,  (c)  vertical  associated  palsy,  {d)  loss  of  convergence, 
{e)  central  nystagmus,  (/)  irregular  tj^^es.     (See  Plate  VIII.) 

(a)  Conjugate  Deviation. — Here  both  of  the  eyes  are  directed  to  the 
side  of  the  lesion,  and  cannot  be  voluntarily  moved  in  an  opposite 
direction.  Yet,  if  the  eyes  are  fixed  upon  an  object  and  the  head 
is  turned  away  from  the  lesion,  the  eyes  will  turn  in  the  direction 
which  voluntarily  is  impossible.  This  is  termed  conjugate  deviation 
of  the  eyes  and  head.  The  eyes'  axes  may  not  be  truly  parallel,  but 
may  diverge  slightly.  In  acute  apoplexies  this  symptom  is  occasion- 
ally seen — i.  e.,  forced  deviation  of  the  head  to  the  side  of  the  lesion. 
(See  Fig.  168.) 


DISEASES  OF   THE  OCULOMOTOR  NERVES 


317 


Lesions  of  the  inferior  parietal,  angular  g^Tus,  and  possibly  the 
foot  of  the  second  frontal  gyrus  may  produce  or  occasion  this  type  of 
forced  position  of  the  eyeball. 

Lesion  of  the  centrum  ovale,  and  of  the  internal  capsule  involving 
the  projection  fibers  of  the  oculomotor  may  cause  conjugate  deviations, 
here  associated  with  hemiplegia  as  a  rule.  The  chief  lesions  causing 
conjugate  deviation  are:  hemorrhage  or  softening,  abscess,  enceph- 
alitis, occasionally  tumor. 

(b)  Lateral  Associated  Palsy. — Here  the  eyes  are  unable  to  pass  the 
middle  line.  The  altered  position  of  the  head  as  seen  in  conjugate 
deviation  is  absent  and  movements  of  the  head  are  unavailing  in 
bringing  the  eyes  past  the  middle  line.  Convergence,  however,  may 
remain  uitact.     Certain  incomplete 

conjugate  deviations  are  found 
here. 

Lateral  conjugate  palsy  is  usu- 
ally due  to  a  pontine  lesion  on 
the  side  of  the  palsy,  and  which 
implicates  the  abducens  fibers  near 
the  nucleus,  and  the  synapses  of 
the  posterior  longitudinal  bundle, 
possibly  Lewandowsky's  tractus 
pontis  ascendens.  Pressure  at  a 
distance  may  also  occasionally 
cause  a  lateral  conjugate  palsy. 

Fractures,  pontine  tumors, multi- 
ple sclerosis,  softening  (after  laby- 
rinthine infection)  are  among  the 
causes  of  this  comparatively  rare 
condition. 

(c)  Vertical  Associated  Palsy. — 
Here  the  motion  of  both  eyes  is 
hindered  only  on  looking  up  or 
dowTi — all  other  associated  move- 
ments are  possible.  AVhen  there 
is  loss  of  ability  to  look  down  usu- 
ally the  ej'elids  do  not  descend  as  they  normally  do.  In  some  patients 
the  palsy  is  not  symmetrical,  one  eye  mo\ing  up  or  (\owi\  more  than 
the  other. 

Certain  forced  positions  of  the  eye,  one  being  higher  than  the  otlier — 
(Magendie-Hertwig  position)  may  be  mentioned  here.  This  syndrome 
often  points  to  implication  of  the  middle  cerebellar  peduncle.  The 
side  involved  is  indicated  usually  by  the  lower-lying  eye.  There  is  also 
nystagmus. 

The  cause  for  the  failure  of  the  lid  to  respond,  analogous  to  von 
Graefe's  symptom  in  exophthalmic  goiter,  is  not  thoroughly  under- 
stood. 


Fig.    177. — -Trochlearis  palsy  in 
cerebrospinal  syphilis. 


EXPLANATION  OF  PLATE  VIIL 

The  Oculorotary  Paths  and  in  Particular  the  Innervation  of  the 
Muscles  which  Turn  the  Head. 

Abbreviations. — BrQa,  arms  of  anterior  corpora  quadrigemina ;  C,  the  cochlea  with 
spiral  ganglion,  the  cochlear  branch  of  the  VIII  pair;  Ca,  anterior  horn  of  the  spinal 
cord;  Cge,  external  geniculate;  Cj,  juxta  restiform  body;  Cio{Rm)  interolivary  region 
of  the  medulla  containing  the  median  lemniscus;  Cirl,  retrolenticular  segment  of  the 
internal  capsule;  CoN,  corticonuclear  contingent  of  the  pyramidal  tract,  CSgt,  sagittal 
view  of  the  occipitotemporal  lobes;  CV,  visual  area  of  the  internal  face  of  the  hemi- 
sphere, transparent  view;  CI-II,  first  and  second  cervical  pair;  DC,  center  of  conjugate 
dcA^ation  of  the  head  and  of  the  eyes;  Dext,  right  external  rectus  muscle;  Dint,  right 
internal  rectus  muscle;  Fa,  ascending  frontal  convolution;  F^,  Fi,  Fs,  three  frontal 
convolutions;  fap,  posterior  and  internal  arcuate  fibers  of  the  medulla;  Flp,  posterior 
longitudinal  fasciculus;  fts,  tectospinal  fibers;  XV2,  central  or  secondary  trigeminal 
pathway;  GG,  Gasserian  ganglion;  G,  Sc,  Scarpa's  ganglion;  NBe,  Bechterew's  nucleus; 
ND,  Deiters'  nucleus;  NVs,  sensory  trigeminus  nucleus;  NIII,  oculomotor  nucleus;  /// 
pair;  NVI,  nucleus  external  rectus;  NVIIIc,  anterior  terminal  nucleus  of  the  cochlear; 
NVIIIv,  triangular  nucleus  of  the  vestibular;  NXI,  spinal  accessory  nucleus  (trapezius- 
sternocleidomastoid) ;  Os,  superior  olive;  Pi,  P2,  superior  and  inferior  parietal  lobe; 
Pa,  ascending  parietal  convolution;  Pc,  angular  gyrus;  Pci,  inferior  cerebellar  peduncle; 
Pcm,  middle  cerebellar  peduncle;  Pul,  pxilvinar;  Qa,  Qp,  anterior  and  posterior  corpora 
quadrigemina;  R,  fissure  of  Rolando;  RgRm,  region  of  median  lemniscus;  Rl,  lateral 
lemniscus;  Rfn,  median  lemniscus;  SgR,  gelatinous  substance  of  Rolando;  SR,  reticular 
substance;  SRg,  gray  reticular  substance;  T\,  T2,  T3,  temporal  convolutions;  Tr,  trapezoid 
body;  V,  vestibular  nerve;  Fi,  Vz,  Vi,  three  branches  of  the  trigeminus,  ophthalmic, 
superior  and  inferior  maxillary;  Vsd,  descending  root  of  the  trigeminus;  xM,  tegmental 
crossing  of  Meynert;  XII,  optic  chiasm;  III,  oculomotor;  VI,  external  rectus;  VI lie, 
cochlear  branches  of  the  auditory;  VIIIv,  vestibular;  XI,  spinal  accessory. 

The  tegmentum  in  its  medullary,  pontine,  peduncular  portions  seen  in  projection  at 
the  level  of  the  aqueduct  of  Sylvius  and  the  fourth  veDtricle,  with  the  reticular  formation 
{SR),  the  posterior  longitudinal  fasciculus  (Flp)  and  the  median  lemniscus  {Rm).  It 
is  limited  laterally  by  the  lateral  lemniscus  {Rl) ,  colored  in  yellow,  and  the  long  sensory 
nuclei  of  the  trigeminus  (F)  and  of  the  auditory  {VIII)  nerves  {NVs,  SgR)  colored  green, 
and  in  yellow  {NVIIIc,  NBe,  NVIIIv,  ND)  and  showing  each  side  of  the  median  line: 
(1)  above  the  rmclei  of  the  III  pair  {NIII)  which  innervates  by  crossed  and  by  direct 
fibers  the  internal  rectus  of  the  eye;  in  the  center,  the  nuclei  of  the  VI  pair  {NVI)  which 
innervates  the  external  rectus  of  the  eye,  and  (3)  below,  the  cephalorotary  nuclei  which 
act  to  rotate  and  incline  the  head  and  neck;  spinal  nuclei  and  spinal  accessory  NXI, 
and  motor  centers  of  the  cervical  cord  {Ca). 

Myelinated  early  are  the  fibers  which  unite  the  nuclei  of  the  sixth  and  of  the  third 
pair  and  of  their  associated  fibers  to  enable  the  lateral  movements  of  the  eye  to  take  place 
in  the  early  stages  of  life.  These  inlernuclear  fibers,  colored  in  red,  take  their  origin 
from  small  cells  in  the  nuclei  in  the  oculomotor.  III  and  abducens  VI,  and  pass  by  means 
of  the  posterior  longitudinal  fasciculus;  the  small  ganglion  cells  of  the  nucleus  of  the 
left  VI  pair  for  example,  can  put  into  action  the  crossed  and  direct  root  fibers  going  to 
the  left  internal  rectus;  and  at  the  same  time  the  ganglion  cells  of  the  nucleus  of  the 
III  left  pair,  can  put  into  action  the  root  fibers  of  the  homolateral  external  rectus  of 
the  same  side  (left).  Thus  there  is  established  a  strict  physiological  association,  per- 
mitting the  action  of  a  dextrorotary  system,  turning  the  eyes  toward  the  right,  or  a 
levorotary  system,  turning  the  ej'es  to  the  left,  an  association  which  can  be  incited 
and  activated  possibly  by  the  cortex,  or  by  various  sensory  or  sensorial  paths,  labyrinthine, 
tactile  or  optic. 

1.  The  cortical  oculorotary  pathway  (colored  dark  red  on  the  right,  pale  red  on 
(318) 


the  left)  belongs  to  the  corticonuclear  path  (CoN)  (see  Plate  I,  0)  and  takes  its  origin 
from  the  center  of  deviate  conjugation  of  the  head  and  eyes  (Dc)  situated  at  the  juxta- 
position of  the  second  and  ascending  frontal  regions.  It  passes  by  way  of  the  knee  of 
the  internal  capsule,  internal  portion  of  the  cerebral  peduncles,  descends  with  the 
aberrant  fibers  of  the  pyramidal  tracts  (see  Plate  I,  D)  in  the  median  lemniscus  and  passes 
to  the  nuclei  of  the  III  pair,  to  the  nuclei  of  the  VI  pair  of  the  opposite  sides,  and  to  the 
eephalorotary  nuclei  of  the  two  sides. 

2.  The  labyrinthine  cculorotary  pathway,  colored  yellow,  contains  a  vestibular  oculo- 
rotary  path,  for  static  equilibrium  and  a  cochlear,  or  acoustic,  oculorotary  pathway. 
The  vestibular  oculorotarj-  pathway  takes  its  origin  in  the  cells  of  the  terminal  nuclei  of 
the  vestibular  root  (XBe,  XVIIIv,  XD).  Its  fibers  belong  to  the  posterior  and  interior 
arcuate  fibers  of  the  medullopontine  tegmentum  (fap).  They  actionate  the  two  VI 
nuclei  and  give  to  the  posterior  longitudinal  fasciculus  (Flp)  direct  and  crossed,  ascending 
and  descending  fibers.  The  ascending  crossed  and  the  descending  direct  fibers  are  the 
most  numerous.  The  ascending  fibers  actionate  the  nuclei  of  both  ///  pairs;  the  descend- 
ing fibers  actionate  the  head-turning  nuclei. 

The  acoustic  rotary  pathway  is  represented  by  the  cells  of  the  anterior  nucleus 
of  the  acoustic  (^NVIIIc),  the  trapezoid  bodj'  (Tr),  the  fibers  destined  to  the  superior 
olive  of  the  same  side  (Os)  and  of  the  crossed  side  and  the  fibers  which  go  in  the  lateral 
lemniscus  (Rl).  This  pathway  can  incite  the  oculorotary  system  by  the  fibers  of  the 
hilum  of  the  superior  olive  which  actionate  (a)  the  motor  cells  of  the  external  rectus 
(VI),  (b)  the  motor  cells  of  the  oculomotor  (III)  acting  on  the  internal  rectus  of  the 
opposite  side  by  the  intermediation  of  small  cells  of  the  intranuclear  oculorotary  system. 
The  labjTinthine  oculorotary  pathways  can  be  activated  not  only  by  peripheral  sensa- 
tions— equilibrium  (vestibular),  auditory  (cochlear),  but  also  by  the  cerebellum,  bj' 
reason  of  the  sjstem  of  internal  fibers  of  the  semicirctilar  canals  and  cerebellovestibular 
fibers  which  terminate  in  Bechterew's  and  Deiter's  nuclei.  These  connections  are 
not  figured  in  the  plate. 

3.  Lateral  movements  of  the  eyeballs  can  be  induced  by  sensory  stimuli  acting  on 
the  skin  of  the  head  and  body.  The  central  trigeminal  oculorotary  pathway  (green) 
takes  its  origin  in  the  nuclei  of  the  V  pair  (NVs),  passes  by  the  arcuate  fibers  of  the 
medullopontine  tegmentum  and  the  posterior  longitudinal  fascicvilus,  and  then  is  divided 
into  ascending  and  descending  fibers  which  actionate  the  oculomotor  nuclei  (///  and  VI) 
and  the  eephalorotary  nuclei  {XI,  ant-horn  cells  Ca).  The  central  sensory  pathway 
(black)  can  actionate  the  oculorotary  and  eephalorotary  nuclei  by  fibers  which  originat- 
ing in  the  reticular  formation  (cSR)  ascend  and  descend  in  the  posterior  longitudinal 
fasciculus. 

4.  The  visual  oculorotary  pathway,  tecto-spinal  (fts)  (light  blue),  leaves  the  anterior 
corpora  quadrigemina,  crosses  the  middle  line  at  the  level  of  the  dorsal  decussation  of 
Meynert  (xM)  descends  in  the  prelongitudinal  fascicle,  giving  off  terminal  and  collateral 
fillers  to  the  nuclei  of  the  ///  pair  of  the  homolateral  side,  to  the  nuclei  of  the  VI  pair 
and  to  the  eephalorotary  nuclei  (XXI, Ca)  of  the  opposite  side. 

It  is  actionated  in  part  by  the  peripheral  visual  pathway  (retina,  nerve,  chiasm) 
(xll,  II)  (dark  blue)  and  in  part  by  the  central  visual  pathway  in  thQ  calcarine  fissure 
(visual  center)  and  the  anterior  corpora  quadrigemina.  It  can  also  be  indirectly  stimu- 
lated by  the  efferent  visual  pathway,  which  originating  in  the  primary  optic  centers 
(Pul,Cge)  irradiates  in  the  calcarine  cortex,  passing  by  way  of  the  retrolenticular  segment 
of  the  internal  capsule  (Cirl)  and  the  optic  radiation  of  the  occipitotemporal  lobes. 

A  destruction  of  the  cortical  oculorotary  pathway  (right)  for  example,  determines  a 
paralysis  of  the  levo-oculorotary  and  levocephalorotary  systems;  that  is  to  say,  a 
conjugate  deviation  of  the  head  and  of  the  eyes  to  the  opposite  side.  A  lesion  of  the 
labyrinthine  oculorotarj'  pathway,  left,  will  also  determine  a  paralysis  of  the  levo- 
oculorotary  system  by  abolition  of  the  lateral  movements  of  looking  of  the  direct 
homolateral  side.  In  both  instances  the  patient  looks  to  the  right  because  of  the  pre- 
dominance of  the  antagonists  (Dejerinej. 

(319) 


320 


CRANIAL  NERVES 


In  the  majority  of  the  cases  lesions  have  been  found  implicating  the 
corpora  quadrigemina  (pineal)  either  directly  or  by  tumor,  or  by  direct 
pressure.  So-called  hysterical  cases  are  usually  mistakes  in  diagno- 
sis. One  such  of  Lewandowsky's  proved  to  be  a  cysticercus  of  the 
corpora  quadrigemina.  A  personal  case  showed  a  sarcoma  of  the 
third  ventricle  pressing  upon  the  anterior  corpora  quadrigemina. 

(d)  Paralysis  of  Convergence. — ^As  an  isolated  symptom  this  is  rare, 
it  is  usually  accompanied  by  other  associated  palsies.  It  is  found 
most  frequently  in  multiple  sclerosis.  A  closely  related  phenomenon — 
weakness  of  the  internus  muscles,  Mobius'  symptom  in  exophthalmic 
goiter,  is  thus  far  difficult  of  explanation. 


Fig.  178. — Inequality  of  pupils.  Left 
pupil  larger  than  right.  Cerebral  tumor. 
Immobile. 


Fig.  179. — Tabes.    External  rectus 
palsy. 


(e)  Central  Nystagmus. — The  extremely  complicated  subject  of 
central  nystagmus  is  more  fully  discussed  in  the  section  on  Vestibular 
Disease.  When  rhythmic,  i.  e.,  possessing  a  quick  and  a  slow  excur- 
sion, it  is  usually  vestibular.  Undulating  nystagmus,  i.  e.,  with  uni- 
form backward-and-forw'ard  movements,  is  more  apt  to  be  due  to 
involvement  of  the  central  or  peripheral  eye  muscle  nervous  pathways. 
Possibly  vestibular  associations  must  always  be  involved.  Undulating 
nystagmus  is  occasionally  seen  in  severe  fatigue,  in  myasthenia,  in 
progressive  muscular  atrophy,  alcoholism,  hydrocephalus,  etc.  Undu- 
lating nystagmus  as  well  as  dissociated  eye  movements  are  normal  in 
infants,  and  are  frequently  seen  in  congenital  defects  (idiots,  imbe- 
ciles, congenital  blindness). 

(/)  Irregular  Palsies. — Dissociated  eye  movements  in  which  the 
eyes  move  irregularly,  each  according  to  its  wish,  as  in  Crustacea, 
in  the  very  young  infant,  in  congenital  defective  developments,  is 


DISEASES  OF  THE  OCULOMOTOR  NERVES 


321 


seen  coming  on  in  adults  from  destruction  of  the  associative  mechan- 
ism of  the  eye  movements,  more  particularly  from  separation  of  the 
nuclei  of  the   oculomotorius.    Lesions  which   cut   the   nuclei  apart 


Anatomical  Site 


In  levator  palpebrse . 


Known  Case. 

'  Mechanical  lesions  from 
traumata,  tumor,  basilar 
meningitis 


In  intraorbital,  intracranial 
extradural,  intradural  or 
infranuclear  portion  of 
peripheral  nerves 


Nuclear . 


Internuclear  in   Projection 
paths 


Clinical  Picture 
1..  Hypokinesias 


ilsolated  Ptosis 


per- 


Rheumatic  toxic  (?) 

ipheral  (?) 

Mechanical  lesions  fror.i 
traumata;  basilar  men- 
ingitis   


Rheumatic      postinfectious 
lesions  (peripheral  ?). .  .  . 


Paresis  or  Paralysis 
one  or  more 
ocular  muscles 


Congenital  defect . 


Bulbar  palsy.  . 
Syringomyelia. 


Mechanical    from    trauma, 
hemorrhage,  tumor 

Defective     nutrition     (em- 
bolism, arteritis) Aj_  coo/dinat 


Toxic 

Diphtheria,  typhoid. ...  1 

Influenza,  syphilis \ 

Botulismus J 


Convergence  Weakness 
and  Paralysis 


Tabes,  paresis .  .  . 
Multiple  sclerosis. 


Polioenceph.  Superior 

Lead 

Alcohol 

Cocain       and       related 
products 

Hemorrhage,  tumor 


Cortical . 


Stimuli  from  cerebellum    .  1 
Stimuli  from  labyrinth .  .  .  / 

Hemorrhage,  tumor,  en- 
cephalitis, abscess,  men- 
ingitis   


Sympathetic f 'hiefly  traumatism 

Hysteria 


Functional. 


Unilateral  conjugate 
palsy 


Total  AsjTiergia 
of  eyeballs 


[  Neurasthenia,  Basedow. .  . 

Fiu.   180. — Summary  of  disturbances  of  exterual  ocular  nerves,     (Veraguth.) 
21 


322  CRANIAL  NERVES 

one  from  another  (multiple  sclerosis,  tumor)  will  cause  this  as\aiergia 
or  ataxia  of  the  eye. 

Skew  deviations  are  conditions  in  which  one  eye  is  directed  outward 
and  downward,  the  other  inward  and  upward.  Such  a  compulsory 
eye  position  is  usually  due  either  to  a  middle  cerebellar  peduncle 
affection  or  to  a  cerebellar  lesion  elsew^here. 

Eyeball  apraxias,  so-called,  or  ideomotor  dissociated  movements 
offer  certain  complex  analogies  with  similar  disturbances  of  the  tongue 
muscles  in  speech,  the  facial  muscles  in  mimicry,  or  the  arm  muscles 
in  expression.  They  are  usually  due  to  lesion  of  the  projection  fibers 
in  the  centrum  ovale  or  internal  capsule. 

Psj'chogenic  dissociation  of  the  ocular  movements  frequently 
occurs.     It  is  a  most  frequent  cause  of  so-called  "eye-strain," 

Treatment. — The  treatment  of  these  various  syndromes  depends 
entirely  upon  the  causative  factors.  These  have  been  discussed  under 
the  respective  syndromes.    Also  see  chapter  on  Midbrain  Disorders. 

IV.   DISEASES   OF   THE   TRIGEMINAL   NERVE. 

Fifth  or  Trigeminal  Nerve. — The  symptomatology  of  lesions  of  the 
fifth  nerve  is  diverse,  as  it  has  both  a  sensory  and  a  motor  part,  and 
has  many  synaptic  junctions  with  cranial,  spinal  and  vegetative  nerves.^ 

Motor  Part. — The  cortical  origin  of  the  motor  part  is  bilateral,  and 
is  located  in  the  lower  third  of  the  central  convolution.  From  here 
the  fibers  pass  through  the  corona  radiata,  enter  the  internal  capsule 
with  the  pyramidal  tract,  and  make  their  first  sj-napsis  with  the  chief 
motor  nuclei,  in  the  dorsolateral  part  of  the  tegmentum  of  the  pons. 
Most  of  the  fibers  cross  about  the  level  of  the  posterior  corpora  quadri- 
gemina.  From  here  the  second  motor  neuron  passes  with  the  inferior 
maxillary  branch  through  the  foramen  ovale,  and  is  distributed  to  the 
masseter,  temporal,  pterygoids,  tensor  tympani,  tensor  veli  palati, 
mylohyoid  and  the  anterior  belly  of  the  digastric. 

Affection  of  the  cortical  motor  neurons  occurs  in  pseudobulbar 
palsy.  Here  the  lesion  is  bilateral  also.  Unilateral  interruption  of 
the  tract  causes  little  disorder  in  mastication.  (Hirt  claims  that  a 
left-sided  lesion  may  cause  bilateral  palsy.)  Bilateral  disorder  is 
nearly  always  associated  with  the  other  features  of  pseudobulbar  palsy 
(q.  v.,  Fig.  188).  The  paralytic  signs  are  the  half-open  mouth,  with 
inability  to  approximate  the  teeth.  The  jaw  cannot  be  protruded, 
and  the  lateral  movements  are  impaired.  The  food  is  apt  to  fall  out 
of  the  mouth,  cannot  be  held  by  the  lips  and  cheeks  or  tongue,  and 
has  to  be  manipulated  by  the  fingers.  Food  is  often  pushed  up  to 
the  pharynx  and  nose.  There  is  no  atrophy  of  the  muscles  of  the 
jaw,  and  no  reaction  of  degeneration.    The  jaw-jerk  is  increased. 

Cortical  foci  may  give  rise  to  chattering  movements  of  the  jaw. 
Grinding  movements  of  the  jaw,  so  frequent  in  paresis  and  occasionally 

'  Map  scheme  of  the  Sensory  Distribution  of  the  Vth  Nerve.    L.  H.  Pegler,  1914. 


DISEASES  OF  THE  TRIGEMINAL  NERVE 


323 


present  in  senility,  are  due  to  cortical  irritation.  The  champing 
movements  of  the  jaw  in  paralysis  agitans  are  possibly  to  be  interpreted 
similarly  to  the  general  tremor  of  the  other  muscles;  namely,  as  an 
interruption  of  the  tonic  impulses  passing  through  the  midbrain 
(corpora  striata)  structures.  (See  Paralysis  Agitans.)  Grinding  of  the 
jaw  is  not  infrequent  as  a  reflex  in  children,  and  it  occasionally  is  seen 
as  a  result  of  basal  meningeal  irritation  of  the  motor  root,  as  in  tuber- 
culosis, syphilis,  or  even  tumor  formation. 

Prolonged  spasm  of  the  muscles  of  mastication  is  seen  in  certain 
toxemias,  such  as  strychnine  poisoning,  tetanus,  tetany.  Here  the 
interpretation  is  not  simple.  It  is  a  result  possibly  of  the  marked 
lowering  of  the  synaptic  threshold  in  the  pontine  motor  nuclei,  causing 
overresponse  to  the  cerebral  or  reflex  motor  impulses.  The  violent 
convulsive  movements  of  the  epileptic  discharge  are  cortical  in  origin. 


Fig.   181. — Paralysis  of  both  the  motor  and  sensory  fifth.     Trophic  ulcer  of  the  right 
eye  and  deviation  of  the  jaw  to  the  paralyzed  side  on  opening  the  mouth. 

Irregular  or  anomalous  spasmodic  movements  of  the  jaws  occur 
in  multiple  sclerosis,  usually  from  midbrain  or  pontine  localizations 
of  the  plaques,  or  they  may  be  reflex  or  psychogenic  (hysteria  or 
dementia  precox).  In  the  latter  instances  in  the  few  cases  analyzed 
the  biting  symbolizes  unconscious  hate  or  sadistic  cravings.  The 
clenching  of  the  jaw  in  food  refusal  as  in  schizoi)hrenia,  depressed 
manic-depressives,  fever  deliria  or  confusion,  expresses  various  sym- 
bolizations.  Fear  of  being  poisoned  is  here  a  frequent  motive  at  the 
conscious  level.  Jaw-grinding  at  night  in  sleep  is  usually  symbolic 
of  protests  and  is  frec|uently  iiiterpretable  from  other  features  of  the 
dream  phenomena  by  p.sychoanalysis. 


324  CRANIAL  NERVES 

Nuclear  disease  of  the  motor  neuron  of  the  trigeminus  may  be 
unilateral  or  bilateral,  partial  or  complete.  In  unilateral  monoplegia 
masticatoria,  the  lateral  movements  of  the  jaw  take  place  to  the 
paralyzed  side.  Bilateral  lesion  causes  the  jaw  to  fall,  and  abolishes 
all  lateral  movements.  The  floor  of  the  mouth  is  flaccid  from  the 
mylohyoid  and  digastric  palsy,  and  there  is  difficulty  of  hearing  notes 
of  low-pitched  tuning-forks.  The  muscles  show  atrophy,  reaction  of 
degeneration  and  the  jaw-jerk  is  absent. 

Peripheral  trigeminus  motor  palsy  is  usually  associated  with  sensory, 
sympathetic  and  taste  phenomena. 

Nuclear  disease  of  the  motor  neurons  is  comparatively  rare.  It 
may  occur  in  multiple  sclerosis,  in  syphilis  of  the  pons,  hemorrhage, 
poliomyelitis,  syringomyelia.  Peripheral  palsies  are  more  frequent, 
and  are  due  to  trauma,  to  pressure  of  carotid  aneurisms,  tumors, 
chronic  meningitis  and  at  times  to  infections  and  toxemias. 

Sensory  Part. — Affections  here  are  much  more  intricate  and  complex, 
and  are  often  combined  with  motor  symptoms.  The  sensory  receptors 
of  the  trigeminus  are  widely  distributed  over  the  face,  the  mucous 
membranes  of  the  superior  and  anterior  nasal  fossa,  the  frontal  and 
ethmoid  sinuses,  tentorium  cerebelli,  teeth,  mucosa  of  posterior 
inferior  nares,  the  sinuses  of  the  jaw,  the  dura  mater,  the  mucous 
membranes  of  the  lips,  cheeks,  posterior  and  inferior  portion  of  the 
mouth,  and  the  filiform  papillae,  which  function  as  tactile  receptors. 
The  taste  buds  are  receptors  for  the  glossopalatine  and  glosso- 
pharyngeal nerves.  The  fifth  nerve  probably  does  not  function  as  a 
gustatory  nerve. ^    The  sensory  ganglion  is  the  Gasserian. 

Collateral  synapses  occur  with  the  ciliary  ganglion  for  the  passage 
of  impulses  from  the  cornea  and  sclera  receptors.  Impulses  from 
the  ethmoid  and  sphenoid  sinuses,  the  pharynx,  posterior  nares, 
hard  and  soft  palates,  maxillary  sinus,  uvula,  pharyngeal  walls, 
tonsils  and  related  mucous  parts  pass  by  way  of  the  sphenopalatine 
ganglion  and  come  into  relation  with  the  glossopalatine  and  glosso- 
pharyngeal. The  submaxillary  ganglia  connections  are  intricate  and 
probably  pass  with  the  vegetative  nervous  system  fibers. 

The  chief  reflexes  arising  from  these  connections  are: 

1.  Winking  reflex. 

2.  Pupillary  (sympathetic  reflex) — pinching  the  cheek  or  neck  causes 
a  dilatation  of  the  pupil  on  the  same  side. 

3.  Jaw  reflex. 

4.  Sneezing  reflex. 

5.  Pharyngeal  reflex — (gagging  and  swallowing). 

The  centripetal  pathways  from  the  Gasserian  ganglion  join  to  form 
a  large  sensory  root  which  is  distributed  to  two  main  end-stations: 
a  mesencephalic  and  a  spinal  one  with  numerous  collaterals.  From 
these  roots  the  second  sensory  neuron  passes  through  the  median 
lemniscus  to  the  opposite  side  to  end  in  the  thalamus.  A  further 
neuron  then  passes  to  the  sensory  brain  area. 

^Kappers:  Psych.  Neur.  Bladen,  1914.     Winkler:  Manuel  de  Neurologie,  1918. 


DISEASES  OF  THE  TRIGEMINAL  NERVE 


325 


The  chief  sensory  symptoms  of  fifth  nerve  mvolvement  are  hyper- 
esthesiae  and  nem-algia,  hypesthesise,  anesthesise.  Loss  of  taste,  trophic 
and  secretory  disturbances,  hay  fever,  rose  colds,  and  psychogenic 
colds  occur  from  implication  of  mixed  vegetative,  glossopalatine 
(chorda  tjTnpani)  and  glossopharyngeal  pathways.  The  apparent  loss 


(  Anterior  corpora 
Quadrigemina  \  posterior  corpora 


Fig.  182. — Scheme  of  intracerebral  trigeminus  pathways.  Cs,  left  hemisphere;  Cd, 
right  hemisphere;  Th-cort.,  thalamocortical  trigeminus  tracts;  Th,  synapses  of  trigemenus 
iu  thalamus;  Sch,  mesencephalic  lemniscus;  mot.  Trig.,  motor  trigeminus  root  nuclei 
in  midbrain;  mot.  Tr.  K.,  motor  trigeminus  nucleus  in  locus  coeruleus;  Rad.  mot.  motor 
roots;  Ggl.  Gass.,  Gasserian  ganglion;  7,  II,  III,  three  trigeminus  branches;  m.s.,  mesen- 
cephalic sensory  trigeminus  roots;  sp.  Tr.  W.,  spinal  trigeminus  branches;  S.  gel.  Rol., 
substantia  gelatinosa  Rolando.  Dotted  line,  motor,  solid  line,  sensory.  (Veraguth- 
Bing.) 

of  taste  in  trigeminus  (Gasserian)  operations  is  frequently  failure  to 
separate  tactile  from  gustatory  impressions,  and  also  failure  to  realize 
the  great  amount  of  damage  done  to  continuous  structures.  Gasserian 
operation  always  involves  other  structures. 

The  most  frequent  of  the  sjudromes  is  trigeminal  neuritis  or  tic 
douloureux  {q.  v.).    Here  the  Gasserian  ganglion  is  often   involved  or 


326 


CRANIAL  NERVES 


the  neuralgia  may  be  due  to  pressure  upon  one  or  all  of  the  roots. 
The  distribution  of  the  hyperesthesia  is  of  value  in   determining  the 


FPoa 


Fig.  183. — Pontine  syndrome.  Alternate  hemianesthesia  of  the  trigeminus  by  lesion 
of  the  median  portion  of  the  left  pontine  tegmentum  involving  the  motor  and  sensory 
roots  of  the  trigeminus,  their  root  fibers,  the  external  portion  of  the  median  lemniscus, 
the  crossed  sensory  paths  of  the  tegmentum  and  extending  as  far  as  the  superior  cere- 
bellar peduncle  behind  and  in  front  as  far  as  the  pons  implicating  peduncular  pyramidal 
fibers.  On  the  right  side  there  is  hemiparesis  of  the  extremities  and  of  the  inferior 
facial  (oblique  cross  hatchings),  hemianesthesia  of  the  extremities,  neck  and  head, 
especially  of  pain  and  temperature  sense  (vertical  lines) .  On  the  leSt  side  there  is  anes- 
thesia of  the  trigeminus;  paralysis  of  the  masticators,  the  pterygoids  especially,  the 
masseter  and  the  temporal.  There  are  choreo-athetoid  movements  of  the  body  due  to 
lesion  of  the  superior  cerebellar  peduncle  (Pes).    (Dejerine.)     (See  Midbrain  Section.) 


DISEASES  OF  THE  TRIGEMINAL  NERVE 


327 


branch  or  branches  involved.    It  is  comparatively  rare  to  find  reflex 
neuritic  pains  from  disease  of  the  teeth,  hence  the  hope  that  removal 


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of  healthy  teeth  will  cure  a  tic  doulom-eux  is  usually  doomed  to  dis- 
appointment. This  is  an  extremely  common  error  and  needs  to  be 
emphasized. 


328 


CRANIAL  NERVES 


Central  or  thalamic  trigeminal  pains  are  possible.  Clinically  little 
is  known  of  them.  Trigeminal  agnosia  is  a  curiosity  merely.  Anes- 
thesia may  be  due  to  interruption  of  peripheral,  pontine,  thalamic  or 
cortical  pathways.  The  diagnosis  as  to  localization  must  be  made  on 
the  basis  of  the  accompanying  s\TTiptoms,  sensory,  secretory,  trophic, 
and  motor. 

An  inflammation  of  the  Gasserian  ganglion  causes  a  trigeminal  herpes 
zoster.  Dryness  of  the  eyes,  with  hyperesthesia  or  anesthesia  is  due  to 
a  peripheral  lesion  of  the  superior  or  first  branch  of  the  nerve.  Uni- 
lateral myosis  may  also  point  to  trigeminal  irritation  here.  Related 
dryness  of  the  mucous  membranes  of  the  nose,  lips,  and  cheeks  with 

anesthesiae,  usually  point  to  periph- 
eral disease  of  the  second  branch, 
while  taste  impairment  of  the  anterior 
two-thirds  of  the  tongue  may  be,  but 
is  not  invariably,  associated  with 
lesions  of  both  second  and  third 
branches,  in  which  chorda  tympani 
fibers  of  the  glossopalatine  are  also 
implicated. 

In  root  lesions,  the  epicritic  loss  is 
usually  less  than  the  protopathic 
loss,  while  the  reverse  is  usually  true 
for  peripheral  lesions .  Pontine  lesions 
show  a  more  general  loss  of  epicritic 
sensibility  on  the  side  of  the  lesion 
with  mono-  or  hemihypesthesiae  or 
anesthesise  on  the  opposite  side  of 
the  body,  while  thalamic  lesions  are 
associated  often  with  anesthesia  and 
analgesia  to  pin-prick,  central  pain 
and  affective  overresponse.  (See 
Thalamus.) 

Trophic  disturbances,  usually  due 

to    peripheral     disease     (?),    cause 

changes   in   the    gums  and  mucous 

membranes,  ulcerations,  herpetic  eruptions.     Corneal  ulceration  and 

loosening  of  the  teeth  are  often  present,  but  whether  trophic  or  not 

is  not  certain. 

Dissociation  of  pain  and  temperature  from  epicritic  touch  sensibility 
may  take  place  in  the  trigeminus  distribution.  For  lack  of  space  here 
a  complete  analysis  of  sensibility  disturbances  of  the  trigeminus  should 
be  sought  in  special  monographs.  A  true  psychogenic  tic  douloureux  is 
alwavs  a  possibility.     It  is  often  curable  bv  psychoanalysis.     (Plate 

Progressive  Facial  Hemiatrophy. — This  rare  condition,  which  shows  at 
its  onset  a  gradual  thinning,  with  wrinkling  of  the  skin  about  the  orbit 
or  jaws,  with  later  progressive  atrophy  of  the  bones,  cartilages  and 


Fig.  185. — Syringomyelia,  begin- 
ning as  hemifacial  atrophy,  then 
developing  "Morvan's  disease,"  and 
finally  showing  classical  syringomyelic 
•end  lesions.     (Hammond.) 


PLATE    IX 


Superposition  of  the    Peripheral   Sensory    Distribution   (in  redi 
and  of  the  Nuclear  (In  black)  of  the  Trigeminus. 

Vl-5,  shows  gradual  involvement  of  the  area  supplied  by  the  trigeminus  in  a  case  of 
syringobulbia;  Vi,  Vii,  Viii,  the  three  branches  of  the  trigeminus;  Ci-v,  distribution  of  the 
cervical  nerves;  Gg,  the  territory  of  the  geniculate  ganglion  (Hunt's  zoster  zone  of  the 
facial).     (Dejerine.) 


DISEASES  OF  THE  FACIAL  NERVE  329 

muscles,  also  of  the  tongue  and  soft  palate,  without  sensory  signs  or 
reaction  of  degeneration  is  at  times  a  result  of  peripheral  or  pontine 
(nuclear)  disease  of  the  fifth  nerve. 

V.  DISEASES  OF  THE  FACIAL  NERVE. 

Seventh  Nerve. — The  seventh  nerve  is  possibly  a  mixed  nerve.  The 
cortical  origin  of  the  motor  neuron  occupies  the  lower  third  of  the  pre- 
central  convolution,  from  here  the  fibers  pass  through  the  knee  of  the 
internal  capsule,  through  the  middle  third  of  the  peduncle  and  make 
their  first  junction  (possibly  by  means  of  intercalated  neurons)  with 
the  homo-  and  contralateral  seventh  nerve  nuclei  in  the  tegmentum 
or  the  pons,  just  ventrolateral  to  the  abducens  nerve  nucleus.  From 
these  nuclei,  four  are  usually  described,  the  second  motor  neuron 
fibers  make  a  dorsal  upward  curve  (genu  facialis)  (see  Fig.  171) 
around  the  abducens  nucleus,  then  pass  ventrally  and  emerge  at  the 
posterior  border  of  the  pons,  lateral  to  the  olive,  where  they  lie  in  close 
relation  to  the  fifth  and  eighth  nerves  in  the  cerebellopontine  angles. 
They  are  finally  distributed  (three  ventral  nuclei)  to  the  muscles  of 
expression  of  the  face,  to  the  muscles  of  the  external  ear,  the  stapedius, 
the  posterior  belly  of  the  digastric  and  to  the  stylohyoid.  The  frontalis, 
corrugator  supercilii,  and  orbicularis  palpebrarum  are  innervated  by 
fibers  coming  from  the  dorsal  group. 

In  its  peripheral  distribution  the  nerve  passes  through  the  facial 
canal  in  the  temporal  bone  (aqueduct  of  Fallopius),  coming  into 
intimate  relations  with  other  cranial  nerves,  eighth,  pars  intermedia, 
and  also  forming  collateral  associations  with  vegetative  fibers  of  more 
than  usual  complexity,  a  study  of  which  is  of  value  in  the  local  diagnosis 
of  lesions  of  this  nerve  and  contiguous  parts.     (See  Fig.  187). 

The  anatomy  of  the  possible  sensory  portion  of  the  nerve  has  not 
been  definitely  homologized.  The  comparative  studies  of  Herrick 
and  Johnson  fail  as  yet  to  show  sensory  components  in  forms  higher 
than  the  amphibia.^ 

By  some  its  sensory  ganglion  is  considered  to  be  the  geniculate,  which 
is  thought  to  contain  the  afferent  fibers  from  touch  receptors  located 
in  the  auricle  of  the  ear,  the  floor  of  the  external  auditory  canal,  the 
tympanum,  and  from  certain  soft  parts  of  the  internal  ear.  The 
nerve  of  Wrisberg  is  considered  to  be  the  sensory  portion  of  the  nerve. 
Hunt's  syndrome  is  a  clinical  picture  thought  to  uphold  this  interpre- 
tation. The  opinion  advocated  here  is  that  these  efferent  systems 
belong  to  the  gustatory  apparatus  and  are  in  reality  parts  of  a  special 
sensory  gustatory  nerve,  the  glossopalatine.  Hunt's  sensory  syn- 
drome of  the  seventh  therefore  may  be  a  faulty  generalization. 

Cortical  Palsies. — Cortical  or  capsular  facial  palsy  is  usually  unilat- 
eral, and  most  characteristically  affects  only  the  lower  facial  distribu- 
tion.   This  is  the  typical  facial  palsy  of  the  upper  neuron  (hemiplegic) 

'  Jour.  Comp.  Noiirol.,  1914.  Milla,  C.  K.:  Sensory  Components  of  Seventh  Nerve, 
Jour.  Nerv.  and  Ment.  Dis.,  1910.     Kidd:  Review  of  Neurology  and  Psychiatry,  1914. 


330 


CRANIAL  NERVES 


type.  The  face  is  drawn  to  the  sounti  side,  the  angle  of  the  mouth 
droops  and  the  nasolabial  fold  is  flattened,  but  the  eyes  can  be  closed 
and  the  forehead  wrinkled.  There  is  paresis  or  paralysis  of  the  lower 
muscles  varying  with  the  severity  of  the  lesion.     The  soft  palate 


Fig.  186. — General  scheme  of  the  course  of  the  pyramidal  fibers  and  the  central  paths 
of  the  motor  trigeminus  and  facial.  Vm.,  lower  division  of  the  trigeminus;  VII,  root 
fibers  of  the  facial;/,  central  facial  fibers;  NV,  motor  fifth  nucleus;  NVII,  facial  nucleus; 
p,  corticospinal  fibers,  lateral  and  anterior;  ra,  anterior  roots;  t,  central  tract  of  the 
motor  fifth.     (Bechterew.) 


may  show  palsy,  pulling  to  the  sound  side  on  phonation.    Babinski 
further  described  a  loss  of  the  contractions  of  the  platysma  of  the 
affected  side  on  forcing  the  mouth  open  against  slight  resistance. 
In  certain  widespread  cortical  neuron  palsies,  however,  the  upper 


DISEASES  OF  THE  FACIAL  NERVE 


331 


branches  may  be  involved,  with  narrowing  (at  times  widening)  of 
the  palpebral  fissure,  and  a  drooping  of  the  outer  angle  of  the  eye- 
brows on  the  affected  side.  Pontine  syndromes  frequently  show  these 
signs.     (See  Midbrain  Section.) 

Apraxia  of  the  facial  musculature  is  met  with  in  cortical,  or  corpus 
callosum  lesions.  Here  the  patient  loses  the  power  to  make  proper 
mimetic  movements.  He  may  not  be  able  to  close  the  eye  on  the 
paralyzed  side,  independently  one  of  the  other.  Furthermore,  in 
cortical  neuron  palsy  the  tongue  protrudes  to  the  paralyzed  side  or 
cannot  be  protruded  at  all.    Speech  disturbances  are  frequent. 


Nucleus  Salivato7-ius 


Nucleus  of 


Superior  MaxiUary  y 
Vidian  X.  \ 

Large  Superficial  Petrosal 

--- A._^^L  Sphe7io 

I      WUperficial  Petrosal^^  VpT^ .palatine 

J  \  Ganglion 

Otic  Ganglion 


y.Intermedius 
ending  in 
Glossopharyngeal 
Nucleus 


Comm animating  Branch- 


To  Auricular 
Branch  of  I'agus  .V. 


Post 
Auricular  Br."' 


To  Digastric 
To  Styto-hyoid 


Afferent  (taste)  fibers 


Efferent  i.excito-glandular) 

fibers  to  submaxillary  and 

sublingual  ganglia  and  glands 


Fig.   187. — Plan  of  the  facial  and  intermedius  nerves  and  their  communications  with 

other  nerves.     (Gray.) 


In  cortical  facial  monoplegias — or  hemiplegias  with  facial  involve- 
ment— there  are  no  atrophies,  the  electrical  reactions  are  not  involved, 
and  secretory  and  taste  modifications  are  absent. 

Cortical  and  subcortical  irritation  may  give  rise  to  facial  convulsive 
movements — spontaneous  laughing  or  crying  movements. 

The  facial  mimetic  movements  of  purely  psychogenic  origin,  tics, 
silly  grimaces  of  the  schizophrenic,  drawn  expression  of  the  depressed 
manic,  tenseness  of  the  paranoid  types,  etc.,  are  numerous. 


332 


CRANIAL  NERVES 


Pontine  Facial  Lesions. — When  the  nuclei  of  the  peripheral  neurons 
are  involved  all  of  the  branches  may  be  affected,  but  inasmuch  as  there 
are  different  groups  of  nuclei,  occasionally,  as  in  poliomyelitis  for 
example,  certain  muscles  are  involved  and  others  are  not.  A  general 
lesion  here  will  cause  a  total  palsy  of  the  muscles  with  atrophy  and  loss 
of  electrical  reactions.  There  are  no  changes  in  taste,  nor  of  hearing  in 
the  pure  nuclear  cases. 

Lesions  here  are  apt  also  to  involve  the  third  nerve,  also  the  pyram- 
idal tract  fibers,  and  the  sensory  fibers  of  the  fillet,  hence  a  variety 
of  hemiplegic  or  hemianesthetic  syndromes — crossed  or  lower  alternate 
hemiplegias  (Figs.  171,  198,  199).    (See  Section  on  Midbrain.) 

Supranuclear  pontine  lesions  may 
rarely  be  double  (Pseudobulbar 
palsy  types). 

Peripheral  Facial  Palsies. — These 
are  called  Bell's  palsies  since  first 
described  by  Bell.  Here  a  great 
variety  of  syndromes  may  occur, 
depending  on  the  exact  site  of  the 
lesion.  These  may  Be  conveniently 
divided  into  five  syndromes  (see 
Figs.  187  and  189).  1.  Most  pe- 
ripheral, due  to  disease  or  pressure 
at  or  outside  of  the  stylomastoid 
foramen.  This  results  in  a  com- 
plete paralysis  of  the  muscles  of  the 
side  of  the  face.  At  rest  the  asym- 
metry is  marked  in  proportion  to  the 
severity  of  the  palsy — all  grades 
are  found.  The  muscles  of  the  fore- 
head cannot  be  contracted  hori- 
zontally or  vertically,  the  eye  remains  partly  or  widely  open  on 
attempts  at  closure,  closing  at  night  in  sleep;  the  nasal  orifice  is 
narrowed,  the  nasolabial  fold  is  obliterated,  the  angle  of  the  mouth 
droops  and  shows  the  teeth,  and  there  is  pulling  of  the  mouth  to 
the  sound  side.  Puffing  the  cheek  is  impossible,  holding  food  and 
saliva  are  difficult,  and  on  attempting  to  whistle  the  air  comes  out 
on  the  paralyzed  side.  Tears  run  down  the  cheek  but  the  reddening 
of  the  eye  is  secondary.  There  is  less  sweating  on  the  paralyzed  side. 
Pressure-pain  sensibility  is  unimpaired.  The  palate  and  tongue  may 
be  apparently  involved,  but  careful  scrutiny  shows  otherwise.  Reaction 
of  degeneration  sets  in  as  a  rule  in  the  severe  cases.  Slight  speech 
disturbance  is  apparent,  especially  in  the  beginning,  and  is  very  marked 
with  a  (rarely  occurring)  double  peripheral  palsy  (see  Figs.  190,  191, 
192  and  193). 

These  palsies  are  due  to  trauma  or  pressure  from  a  tumor,  possibly 
a  perichondritis  of  or  swelling  about  the  stylomastoid  foramen  (called 
rheumatic  or  refrigeration  palsy) .    The  effect  of  cold  upon  the  facial 


Fig.   188. — Pseudobulbar  palsy. 
(Tilney.) 


DISEASES  OF  THE  FACIAL  NERVE 


333 


nerve  itself,  i.  e.,  by  exposure  in  riding  with  one  side  exposed  to  open 
windows,  etc.,  looms  large  in  statistical  enquiries. 

2.  Just  within  the  stylomastoid  foramen,  and  within  the  Fallopian 
canal,  the  chorda  timpani  (part  of  the  glossopalatine)  travels  with 
the  facial  nerve.  A  lesion  here  will  cause  all  of  the  symptoms  just 
enumerated,  and  in  addition  there  will  be  an  impairment  or  loss  of 


Tears 
Taste  &  Saliva 


-Sweat 
-  Tears 
-Taste  £•  Saliva 


Post.  Auricular 
Nerve 


Fig.  189. — Diagram  of  facial  nerve,  showing  course  of  secretory  and  of  taste  fibers  [of 
glossopalatine,  J.].    (Stewart.) 


taste  of  the  anterior  two-thirds  of  the  tongue.  There  will  also  be  a 
diminution  of  the  salivary  secretions.  Pressure-pain  sensibility  is  not 
impaired. 

3.  If  to  this  last  syndrome  hyperacusis  and  tinnitus  alone  be  added 
the  lesion  is  slightly  farther  back  in  the  canal  involving  the  branch 
given  off  to  the  stapedius  muscle  (see  Figs.  187  and  189), 


334 


CRANIAL  NERVES 


4.  Lesions  lying  between  the  geniculate  and  the  stapedius  within 
or  at  the  internal  entrance  to  the  Fallopian  canal,  cause  a  variety  of 


Fig.   190. — Peripheral  facial  paLsy. 
Smiling. 


Fig.    191. — Peripheral  facial  palsy. 
Whistling. 


additional  symptoms,  the  exact  anatomical  relations  of  which  are  still 
somewhat  obscure. 


Fig. 


192. — Peripheral  facial  palsy. 
Closing  the  eyes. 


Fig.   193. — -Peripheral  facial  palsy. 
Showing  the  teeth. 


As  the  geniculate  ganglion  of  the  glossopalatine  nerve  lies  in  the 
sheath  carrying  the  facial  also,  an  inflammatory  swelling  of  this  gan- 
glion, may  cause  a  pressure  palsy  of  this  nerve.  In  addition  to  the  facial 
palsy  other  symptoms  of  the  glossopalatine  disorder  are  added,  chiefly 


DISEASES  OF   THE  FACIAL  NERVE 


335 


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336  CRANIAL  NERVES 

taste  loss.  If  the  acoustic  is  also  pressed  upon  there  may  be  tinnitus  or 
hearing  disturbances.  A  herpetic  eruption  may  occur  also,  from  in- 
volvement of  either  the  geniculate  or  other  posterior  ganglia.  Herpes 
of  the  auricle,  incorrectly  assumed  by  Hunt  to  be  a  sensory  zone  of 
the  seventh,  may  occur  from  involvement  of  the  cephalic  sensory 
ganglia. 

Facial  palsy  of  non-geniculate  origin  from  lesions  in  the  same  region 
may  or  may  not  be  accompanied  by  loss  of  hearing.  The  chief  addi- 
tional diagnostic  feature  of  lesions  here  is  the  lowering  of  the  threshold 
of  deep  sensibility  (Maloney)  from  involvement  of  vegetative  fibers. 

Lesions  of  the  seventh  nerve  at  its  emergence  from  the  pons  usually 
implicate  other  structures,  notably  the  fifth  or  eighth  nerves,  at  times 
the  sixth,  eleventh,  twelfth.  The  facial  palsy  is  of  the  peripheral  type 
with  no  loss  of  taste,  changes  in  the  secretions,  or  suppression  of  the 
lachrymal  secretions.  Basal  symptoms  such  as  anorexia,  nausea, 
headache,  and  optic  disk  changes  are  often  present.  The  chief 
pathological  processes  are  basal  meningitis,  usually  syphilitic,  or  tumor 
formation. 

VI.  DISEASES  IN  THE  AUDITORY  AND  VESTIBULAR  PATHWAYS. 

The  Eighth  Pair. — The  eighth  cranial  nerve  is  in  reality  two  separate 
nerves,  with  distinctly  different  structures,  pathways  and  functions. 
It  is  not  a  single  nerve  with  two  parts.  The  two  nerves  are  the  coch- 
lear or  auditory  proper,  and  the  vestibular — a  portion  of  the  cere- 
bellar apparatus. 

The  former  handles  sounds,  the  latter  serves  to  orient  the  body  in 
space.  Their  chief  receptors  lie  closely  related  in  the  sphenoid  bone. 
By  reason  of  this  close  topographical  relationship  infections  of  the 
middle  ear  are  apt  to  involve  both  structures,  and  by  reason  of  the  close 
associations  with  intracranial  structures,  brain  involvements  such  as 
meningitis,  abscess,  etc.,  may  result.  Their  central  stations  are  wide 
apart  in  the  temporal  cortex  and  cerebellum  respectively. 

Auditory  Nerve. — The  receptors  for  sound  stimuli  lie  in  the  organ 
of  Corti  in  the  cochlea.  They  respond  in  the  average  human  to  sound 
stimuli  of  11  octaves,  i.  e.,  from  10  to  7840  double  vibrations.  Ordinary 
conversational  or  musical  sounds  usually  lie  within  16  to  4032  vibra- 
tions. Space  orientation  through  sound  is  purely  associative.  The 
chief  avenues  for  sound  conduction  to  the  receptors  is  the  auditory 
canal,  but  sound  conduction  is  also  possible  by  way  of  the  bones 
of  the  skull  and  in  part  by  other  bony  structures.  From  the  cochlea 
the  branches  coalesce  to  form  the  acoustic  nerve  which,  passing  in  the 
auditory  canal  with  the  facial,  enters  the  medulla  at  about  the  cere- 
bellopontine angle.  The  sensory  ganglion  is  the  tuberculum  acusticum. 
The  further^^eourse  of  the  pathways  is  illustrated  in  Figs.  196  and  197, 
also  see  Plate  VIII. 

The  chief  disturbances  of  the  auditory  nerve  are:  various  forms 
of  deafness  and  of  tinnitus.    Deafness  may  vary  considerably  and  may 


DISEASES  IN  AUDITORY  AND   VESTIBULAR  PATHWAYS     337 

be  absolute  or  partial.  Certain  tones  may  be  cut  out  and  not  others, 
upper  or  lower  tones,  sometimes  intermediate  tones  drop  out  (hearing 
scotomata,  analogous  to  optic  scotomata,  are  not  infrequent  in  hysteri- 


FiG.   195. — Receptor  topography  of  the  acoustic  nerve.    (Semidiagrammatic.)    (Testut.) 


Fig.   19G. — Central  acoustic  paths,    na,  anterior  acoustic  nucleus;  ta,  acoustic  tubercle; 

Sta,   acoustic   stria;;   on,   superior  olive;  cir,   trapezoid   body;   nVI,   al)ducens   nucleus; 

nil,  lateral  letnni.scus;  cqi,  cqn,  posterior  and  anterior  cf)rpora  quadrigeniina;  cgi,  external 

geniculate  body;  st,  acoustic  path  to  cortex;  It,  temporal  lobes;  th,  thalamus.  (Bechterew.) 

22 


338 


CRANIAL  NERVES 


cal  reactions,  dementia  precox,  in  multiple  sclerosis,  paresis,  tabes, 
etc.).  These  anomalies  of  hearing  are  chiefly  peripheral,  either  in  the 
primary  receptors  or  occasionally  in  the  ganglion.  Paracusia  (buzzing, 
whistling,  crackling),  are  for  the  most  part  peripheral,  but  may  also  be 
central,  as  in  psychotic  or  psychoneurotic  syndromes.  Psychogenic 
deafness  is  a  frequent  complication  of  traumata.  These  are  frequently 
encountered,  particularly  in  war  times — detonation  deafness.  The 
presence  of  intact  auditory  pathways  may  be  detected  by  the  per- 
sistence of  the  auditory-orbicularis  reflex  (g.  v.) .  Also  see  paragraph 
on  malingering  of  hearing  in  Examination  Methods  chapter. 


Fig.  197. — ^Scheme  of  the  central  acoustic  pathways.  Ti,  T2,  first  and  second  temporal 
lobes;  /,  island  of  Reil;  ce,  claustnim;  Li,  lenticular  nucleus;  Ci,  internal  capsule; 
Thalam.,  thalamus;  U.S.,  median  lemniscus;  S.s.,  optic  radiations;  V.L.,  posterior 
quadrigemina;  B.A.,  middle  cerebellar  peduncle;  c.c.p.,  crus  cerebelli  ad  pontem;  c.gen. 
int.,  internal  geniculate;  Cge.,  external  geniculate;  Sta.,  striae  acousticse;  g.s.p.,  ganglion 
spirale;  Ta.,  acoustic  tubercle,     (v.  Monakow.) 

Complete  deafness  from  medullary  lesions  is  rare  for  anatomical 
reasons  already  indicated. 

Cortical  or  subcortical  involvement  of  the  auditory  pathways  cause 
various  forms  of  amnesic  aphasia.     (See  under  Speech  Mechanism.) 

Chart  for  Differentiations  of  Peripheral  and  Central  Lesions. 


Speech  test. 


Air  Conductor  for  forks 
c,  c*,  c'',  c^,  c*  and  for 
Galton's  whistle. 

Weber  (ci). 

Schwabach  (c'). 
Rinne  (c.  c\  c^). 


Disease  of  sound-conducting 

apparatus. 
Deep   tones   heard   worse 

than  higher  ones. 
Deep   tones   heard   worse 

than  higher  ones.  Lower 

tone  threshold  raised. 
Median    or    localized    in 

worse  ear. 
Lengthened. 

Negative. 

According     to     grade     of 

retardation  only   for   c. 

or  for  c.  and  the  higher 

tones  to  c^. 


Disease  of  sound-perception 

apparatus. 
Deep  tones  heard  worse  or 

better  than  higher  ones. 
High   tones    heard    worse 

than  deep  ones.    Upper 

tone  threshold  lowered. 
Median    or    localized    in 

better  ear. 
Rarely      normal,     mostly 

shortened. 
Positive. 


DISEASES  IN  AUDITORY  AND   VESTIBULAR  PATHWAYS    339 

Vestibular  Nerve. — It  has  been  established,  ahnost  beyond  question, 
that  the  lab^Tinth  is  that  organ  of  the  body  which  is  concerned  with  the 
receiving  of  impressions  of  its  position  in  space,  particularly  for  the 


General  Etiology  of 
CmEP  Disturbances. 
1.  Congenital  defects. 


The  Chief  Primary  Localizations. 


The  Resdltino 
Clinical  Signs. 
General  diffioultj-  in 
hearing. 


2.  Functional  strain. 


Not  sharply  localized 


3.  Disturbance  of 
psyohotonio  compound 


In  labyrinth  and  in  the 
auditory  lying  in  the 

sphenoid. 


Partial  deafness 
especially  to  high 
notes. 


Dtafnesa. 


i,  Mechanical    (trauma, 
hemorrhage;  tumor). 


Ringing  in  ears. 


S.  Metabolic  disturbance, 
(a)  Quantitative, 
arteriosclerosis; 
caohe.\ia. 


(b)  Poisoning  from  ex- 
ogenous sources 
(quinin,  .salicylic 
acid,  alcohol, 
COi,  nicotin, 
lead). 


(c)  Poisoning  from 
parasitic  sources 
(syphilis,  diph- 
theria, typhoid). 


In  endocranial  part 
of  the  auditory. 

In  total  peripheral 
nerve. 


In  central  acoustic 
tract. 


Hyperacusis. 


Meniere  vertigoes. 


Deafness  in  oompanj 


Cortical  deafness 
(aphasia). 


(d)  .Associated  inflam- 
mations: 
Middle  ear. 
Meningitis. 


In  cortical  auditory  areas. 


Deaf -mutism. 


6.  Multiple  sclerosis. 

Fig.   198. — Summary  of  auditory  disturbances.     (Veraguth.) 


head.  The  meaning  for  the  human  body  of  the  physical  laws  of 
gravity  is  its  chief  concern.  It  is  adapted  to  the  mechanical  stimuli 
of  gravity,  acting  largely  through  the  otolith  organ,  which  reacts  to 


340  CRANIAL  NERVES 

changes  in  the  incidence  and  degree  of  pressure  upon  its  sensory  end- 
organs,  due  to  changes  in  the  specific  gravity  of  its  surrounding  fluids; 
and  also  through  the  semicircular  canals  which  react  to  changes  in 
position  in  the  three  planes  of  space.  The  slightest  change  of  the 
body  in  space  is  felt  by  this  apparatus,  and  in  the  healthy  central  ner- 
vous system  any  such  change  is  automatically  reacted  to  by  appropriate 
(proprioceptive)  motor  response. 

This  motor  response,  however,  is  a  comiDlicated  mechanism,  and  all 
of  its  elements  are  not  thoroughly  analyzed.  One  of  its  parts  is  that 
of  a  reflex  muscular  tonus,  by  which  the  ordinary  posture  of  the  body 
is  maintained.  It  is  this  function  that  has  entitled  it  to  the  appellation 
of  the  labyrinthine  tonus.  Sherrington^  has  analyzed  the  complicated 
interrelations  between  the  proprioceptors  of  the  limbs,  muscles,  joints, 
etc.,  which  carry  impressions  of  movements,  strains,  tensions,  etc., 
and  the  receptors  in  the  labyrinth.  It  is  impossible  to  enter  into  them 
here.  Suffice  it  to  say  that  the  result  is  the  reflex  maintenance  of  the 
posture  of  the  body,  including  the  compensatory  reflexes  of  the  head, 
and  those  muscles  of  the  head  capable  of  changing  the  sense  of  con- 
sciousness of  position,  particularly  the  muscles  of  the  eyeballs.  (See 
Plate  VIII.) 

The  labyrinth  belongs  to  a  series  of  organs  that  work  in  response 
to  gravity.  It  is  a  part  of  a  great  system  of  connections — which 
Sherrington  has  designated  as  the  proprioceptive  system — which  gives 
animals,  human  as  well  as  others,  a  definite  attitude  toward  the 
external  world  of  space.  It  is  the  most  important  of  these  organs. 
It  is  connected  in  a  system  with  other  nervous  structures  performing 
their  part  in  the  same  general  function,  and  each  segment  of  the  body 
is  caught  up  in  the  chain  of  connections  from  the  lower  end  of  the  spinal 
cord  to  the  frontal  area  of  the  cortex. 

This  whole  complicated  system  of  end-organs,  fiber  connections, 
long  and  short  fiber  tracts,  has  its  chief  center,  just  as  every  other 
reflex  system  has  its  center.  The  chief  center  or  head  ganglion  of 
this  whole  proprioceptive  system  is  the  cerebellum.  The  cerebellar 
connections  of  the  vestibular  system,  the  vestibulospinal,  vestibulo- 
bulbar,  vestibulocerebellar,  and,  finally,  the  cerebellorubrocortical 
components  which  carry  those  fibers  whose  functioning  is  recognized 
in  the  consciousness  of  space  relations,  are  now  fairly  well  known, 
not  in  their  entirety,  but  in  their  main  tracts  and  connections.  Hence, 
disease  or  disorder  which  shows  any  perturbation  of  the  function  of 
orientation  in  space  may  be  more  or  less  accurately  localized  along  the 
fiber  tracts,  carrying  the  necessary  impulses  underlying  these  functions, 
and  an  appropriate  therapy  adopted  (see  Plates  VIII,  X  and  XI). 

Nervus  Vestibularis. — The  fibers  of  the  median  acoustic  root  (Lewan- 
dowsky — mixed)  constitute  the  central  prolongation  of  the  bipolar 
ganglion  cells  which  make  up  the  vestibular  or  Scarpa's  ganglion. 
The  peripheral  prolongations  of  the  cells  are  the  receptors  (hair  cells  of 

'  The  Integrative  Action  of  the  Nervous  System. 


DISEASES  IN  AUDITORY  AND   VESTIBULAR  PATHWAYS     341 


— -I-  NucleuSj.riiber 


Superior  cerebellar]^  _ 
peduncle         ) 


■Roof  nucleus 
Belter's  nucleus 


Tr.  rubro-spinalis-  ,   „      • 

\\  I  It^Tr.  spmo-cereoellans 

Tr.  vestib^ilo-spinalis-- 

Dorsal  longitudinal}^ 
bundle  ) 


Fig.  199.— Diagram  to  illustrate  the  chief  spinal  connections  of  the  cerebellum.  On 
the  right  the  afferent  tracts  are  represented,  on  the  left  the  efferent  cerebellar  tracts. 
(Holmes.) 

F.  thalamo-corticalis 


Nucleus  ruber. 

Superior  cerebellar 

peduncle 


Nude  us 
dentatus 


F.  olivo-cerebellariS'' 


-F.  cortico-pontinus 

\  Central  Tegmental 
\  Tract 

Middle  cei-ebellar  peduncle 

'Inferior  Olive 


Fig.   200.— Diagram  to  illustrate  the  afferent  and  efferent  connections  of  the  cerebellum 
with  the  forebrain.    (Hohnes.) 


342  CRANIAL  NERVES 

the  ampullae)  in  the  walls  of  the  semicircular  canal.  Movement  of  the 
head  or  of  the  body  causes  a  flow  of  the  endol^nnph  which  gives  rise  to 
the  specific  stimuli  in  the  receptor  organs.  The  thick  bundle  of  the 
median  root  pushes  its  way  between  the  spinal  trigeminus  root  and  the 
corpus  restiforme  (inferior  cerebellar  peduncle)  lying  at  first  close  to  the 
median  edge  of  the  spinal  accessory  nucleus,  and  reaches  dorsally  like 
the  tines  of  a  fork  toward  the  end  nuclei.  These  synapses  of  the 
vestibular  are  (1)  Deiters's  nucleus  lateralis,  (2)  Bechterew's  nucleus 
superior,  (3)  Schwalbe's  nucleus  medialis  or  principalis,  (4)  nucleus 
spinalis. 

Of  the  connections  of  the  end-nuclei  of  the  vestibularis  those  of  the 
cerebellum  are  the  plainest.  Strong,  somewhat  swollen  bundles 
of  nerve  fibers  go  from  the  Deiter  and  Bechterew  nuclei  dorsally  in 
the  cerebellum.  Fibers  from  the  nuclei  triangularis  also  join  them. 
The  acoustic  cerebellar  tract  lies  on  the  medial  side  of  the  inferior 
cerebellar  peduncle,  in  the  medial  lateral  portion  from  the  superior 
cerebellar  peduncle,  in  which  a  portion  also  goes.  The  majority  of 
the  fibers  go  to  the  cerebellar  worm  (vermis)  and  end,  mostly  crossed, 
in  the  nuclei  of  the  roof  (tectalis),  probably  also  in  the  nucleus  globosus 
and  nucleus  emboliformis. 

Vestibular  Vertigoes. — At  one  time  loosely  grouped  together  under 
the  name  Meniere's  disease,  the  analyses  of  later  years  have  shown 
a  great  variety  of  these  affections  depending  upon  the  anatomical  sites 
of  the  lesions.    One  must  distinguish  between: 

1.  Disease  of  the  peripheral  end-organ,  (a)  partial,  or  (6)  complete; 
these  are  the  vertigoes  of  partial  or  complete  labyrinthine  disease. 

2.  Disease  of  the  first  neuron,  (a)  paresis,  (6)  paralysis  of  the 
vestibularis. 

3.  Disease  of  the  primary  end-nuclei  in  the  medulla  and  of  Deiter's 
nucleus.  Lesions  of  the  latter  give  a  special  symptomatology  termed 
Bonnier's  syndrome. 

4.  Disease  in  the  region  of  the  posterior  longitudinal  bundle  asso- 
ciated with  eye-movement  vertigoes. 

5.  Disease  of  the  nuclear  region  of  the  eye-muscles  in  the  corpora 
quadrigemina. 

6.  Disease  of  the  pontine  eye  nuclei. 

7.  Disease  of  central  eye  paths. 

8.  Disease  of  cerebellum. 

In  disease  of  all  these  regions  vertigoes  are  to  be  expected  by  impli- 
cation of  the  vestibular  nerve;  the  character  of  the  accompanying 
phenomena,  especially  the  nystagmus,  aids  in  the  localization.  (See 
chapter  on  Examination  Methods:   Vestibular  Tests.) 

In  partial  or  circumscribed  disturbance  of  the  vestibular  end-organs 
in  the  labyrinth  the  vertigo  is  associated  with  nystagmus.  The 
nystagmus  is  spontaneous  and  shows  a  long  slow  movement,  due  to 
the  vestibular,  and  a  quick  return  movement  due  to  the  tegmental 
nuclei,  the  direction  of  the  quick  movement  naming  the  nystagmus. 
Vestibular  nystagmus  usually  increases  when  the  eyes  are  directed  in 


DISEASES  IN  AUDITORY  AND   VESTIBULAR  PATHWAYS    343 

the  direction  of  the  quick  movement,  and  usually  diminishes  or  ceases 
on  looking  in  the  opposite  direction.  There  is  almost  always  a  combi- 
nation of  horizontal  and  of  rotary  nystagmus.  Barany  states  that 
every  other  form  of  spontaneous  nystagmus  is  of  intracranial  origin.  If 
the  nystagmus  movement  is  rotary  and  horizontal  it  must  be  deter- 
mined whether  it  is  peripheral  or  central.  A  peripheral  nystagmus  to 
the  right  should  show  on  caloric,  pressure,  and  rotation  tests  that  the 
right  vestibule  is  functionally  active.  Should  such  tests  show  an 
inactive  right  vestibular,  then  the  nystagmus  must  be  of  central  origin. 
If  the  right  vestibular  is  active,  then  continued  observation  of  the 
nystagmus  will  alone  determine.  Should  the  nystagmus  continue 
uninterruptedly  for  twenty-four  hours  or  more,  it  is  of  intracranial 
origin.  If  it  lasts  a  shorter  interval  and  is  uninterrupted  by  quiet 
intervals,  it  may  be  either  peripheral  or  central.  ^Vhen  there  is  also 
nystagmus  of  the  well  side,  which  lasts  about  two  weeks,  gradually 
decreasing,  then  a  peripheral  disturbance  seems  certain.  Intracranial 
nystagmus  is  not  so  apt  to  diminish. 

The  Meniere-like  attacks  are  either  mild  or  marked.  Buzzing 
in  the  ears  is  rare  in  mild  attacks.  There  is  no  impairment  of  hear- 
ing. In  the  severer  attacks  there  is  little  buzzing,  but  hearing  is  apt 
to  be  impaired.  In  free  intervals  the  nystagmus  diminishes  or  dis- 
appears, the  Barany  caloric  reaction  is  diminished  on  the  affected  side. 

Total  destruction  of  the  labjTinth  may  be  acute  or  chronic ;  the  latter 
may  show  no  symptoms.  The  former  sets  in  wuth  violent  vertigo, 
nausea,  vomiting.  There  is  marked  horizontal  and  rotary  nystagmus 
of  the  sound  side.  The  slightest  movement  of  the  head  increases  the 
vertigo  and  nystagmus  during  the  first  forty-eight  hours;  the  latter 
gradually  disappears  in  three  or  four  weeks.  There  is  marked  loss  of 
coordination,  with  tendency  to  fall  to  one  or  the  other  side.  After  the 
period  of  quiescence  of  the  nystagmus,  caloric  and  rotation  tests  show 
the  defective  function.    The  galvanic  reaction  is  not  usually  affected. 

Disease  of  the  vestibular  nerve,  usually  due  to  tumor  of  base  (acous- 
tic, cerebellopontine  angle),  leads  to  similar  reactions.  Here,  however, 
there  seems  to  be  a  difference  in  that  Neumann  has  found  that  the 
galvanic  reaction  is  reduced  or  lost,  according  to  a  partial  or  complete 
destruction  of  the  vestibular  ganglion.  Other  cranial  nerves  are 
here  involved  as  a  rule.  The  cochlearis  is  frequently  implicated. 
Complete  deafness  does  not  result.  The  trigeminus  is  also  often 
involved  and  pain,  paresthesise,  or  motor  defects  appear.  Cerebellar 
symptoms  may  also  complicate  the  picture.  The  nystagmus  is  apt 
to  continue  in  intensity  with  tumors,  and  may  be  on  the  sound  as 
well  as  the  affected  side.^ 

Involvement  of  the  nuclei  (encephalitis,  abscess,  syphilis,  tumor) 
brings  about  similar  attacks  of  nausea,  vomiting,  vertigo,  and  nys- 
tagmus. The  sjTTiptoms  continue  and  increase,  as  a  rule,  beyond  the 
three  weeks  ordinarily  seen  in  labyrinthine  disease. 

•Gushing:     Tumors  of  the  Acousticus  and  Cerebellopontine  Angle,  1917. 


344 


CRANIAL  NERVES 


The  method  of  continuous  observation  aids  in  locating  the  diseased 
focus. 

Bonnicr's  Syndrome,  (kie  to  implication  of  Deiters'  nucleus  and 
contiguous  structures,  usually  causes  a  marked  attack  of  nausea, 
vomiting,  vertigo,  and  nystagmus  with  buzzing  in  ears  and  deafness 


Fig.  201. — General  scheme  of  the  cerebral  paths  of  the  vestibular.  VII,  facial  nerve; 
VIII,  acoustic  nerve;  a,  Bechterew's  nucleus;  D,  Deiter's  nucleus;  d,  dentate  nucleus; 
g,  nucleus  globosus;  p,  nucleus  emboliformis;  ra,  anterior  root  fibers;  sc.  sc\  subcortical 
fibers  of  the  red  nucleus  and  of  the  thalamus  to  the  cortex;  t,  tegmental  nucleus. 
(Bechterew.) 


(Meniere's  syndrome),  with  irradiations  to  the  ninth  and  tenth  nerves 
causing  anxiety,  tachycardia,  and  hemiplegic  weakness.  The  trigem- 
inus and  oculomotor  are  also  apt  to  be  involved.  Bonnier  has  also 
described  peculiar  somnolent  attacks  accompanying  his  syndrome. 
Little  can  be  done  for  these  cases  unless  the  focus  is  of  syphilitic  origin. 
Here  vertigo  and  nystagmus  are  associated  in  various  ways,  but 


DISEASES  IN  AUDITORY  AND   VESTIBULAR  PATHWAYS     345 


the  vertigo  disappears  on  closing  the  eyes,  and  forced  movements, 
conjugate  deviations,  and  various  skew  deviations  afford  a  ckie  to 
diagnosis.  Caloric  and  other  tests  determine  the  integrity  of  the 
labyrinthine  functions. 

TABLE  OF  DIFFERENTIAL  DIAGNOSIS   OF  LABYRINTHINE  AND  CEREBELLAR 

DISTURBANCES. 


I.                                                 II.                                      !                       III. 

Circumscribed  '<                        Diffuse  labyrinthitis.                             Diffuse  purulent  labyrinthitis 

labyrinthitis.    !                                                                                   j          and  cerebellar  abscess. 

Tests. 

1 

Ji'**-1       ■         Se"ous          Acute  purulent  £^?Tv^"f?fr         Type  A. 
formation.      |   labyrinthitis.       labyrinthitis.   |  lent  labyrinthi- 

Type  B. 

Spontaneous 

May  be  absent  Mostly  on           Rotatory  and   Weak   on   well  Strong     rotarj' 

Rotatory  and 

nystagmus 

on   sound    or      sound      side; '    horizontal;          side,     or    on      and    horizon- 

horizontal    on 

diseased  side      also  on  both     strong  on  the      both  sides            tal  on  sick  side 

well  side. 

sides                   well  side 

Turning 

Produced  both  Both  sides  with   Cannot  be          i  Residual:      on  Produced  both 

nystagmus 

sidessamein-     sameintensity     tested  because  ^     well  side,  nor- ;    sides, samein- 
tensity                                          of     excessive ,    mal  time;  on  \    tensity. 

1                            i    reaction           i    sick  side  short- 

1 

ened 

Caloric 

Diminished    or  Absent 

Absent. 

Absent 

Absent. 

nystagmus 

not  present 

Compression 

Present               Absent              1  Absent 

Absent 

Absent. 

Hearing 

Hears  with  dif-   Hears  with  dif-   Deaf 
ficulty  or  deaf ,    ficulty  or  deaf 

Deaf 

Deaf. 

Other 

Vertigo,  nausea  Marked  vertigo,  Vertigo,  nausea  '  Slight   vertigo, 

Changeable  ver- 

symptoms 

and     nystag- :    nausea,  fever      and  vomiting      also  slight      tigqandequi- 
mus  on  rapid                                                        I    equilibrium         librium     dis- 

movement  of 

disturbances        turbances; 

head 

1 

!    finger  test 
i    positive,  eye- 
grounds  posi- 

1                          ,    tive    . 

Treatment 

Radical  opera-  No  operation      Radical  andRadical  and   Radical,    laby- 

Radical  and 

tion 

labyrinthine        labyrinthine 

rinth  and  cer- 

labyrinth   op- 

operation           operation 

ebellar  opera- 

eration;        if 

tion 

nystagmus 
continues, 
cerebellar  op- 
eration. 

Cerebellar  vertigoes  have  a  number  of  special  features.  So  far  as 
the  vertigo  is  concerned  they  may  not  be  separable  from  the  laby- 
rinthine or  vestibular  vertigoes.  Hearing  s^Tnptoms  are  usually  absent. 
The  nystagmus  is  less  apt  to  be  horizontal  and  rotary,  but  may  be  up 
or  down  or  oblique,  and  is  usually  directed  toward  the  affected  side. 

There  are  usually  also  sjniptoms  of  a  tumbling  gait  toward  the  side 
of  the  lesion;  there  is  asynergia  and  usually  adiadokokinesia.  No 
real  distinction  as  to  the  side  of  the  lesion  affected  can  be  gained  from 
the  fact  as  to  the  subjective  or  objective  motion  of  the  objects  during  a 
vertiginous  attack.  Closure  of  the  eyes  has  no  marked  affect  upon  the 
vertigo,  nor  upon  the  gait.  Caloric  and  other  tests  determine  a  normal 
labyrinth. 

In  conclusion  it  is  emphasized  that  the  subject  of  vertigo  is  a  very 
large  one  and  it  is  necessarily  only  very  hastily  summarized  in  these 
few  paragraphs.  More  complete  knowledge  may  be  gained  from  a 
study  of  the  various  works  mentioned  in  the  footnotes,  but  special 
attention  is  called  to  the  valuable  study  of  the  subject  of  vertigo  and 


346  CRANIAL  NERVES 

equilibrium  by  Isaac  H.  Jones/  a  work  which  every  student  of  the 
neurological  disturbances  of  the  ear  mechanisms  should  consult. 

Treatment. — Here  there  comes  into  consideration  the  surgery  of 
the  ear  and  the  surgery  of  the  cerebellum  and  the  cerebellopontine 
angle.  The  ear  specialist  should  treat  the  labyrinthine  cases,  not  the 
neurologist.  Rest  in  bed,  quinin,  and  the  usual  medical  treatment 
which  shuts  one's  eyes  to  the  danger  of  a  suppurative  labyrinthitis, 
brain  abscess,  etc.,  is  folly. 

In  the  apoplectic  form  of  Meniere's  syndrome  (hemorrhagic  laby- 
rinthitis) often  mistaken  for  a  cerebral,  or  cerebellar  hemorrhage, 
the  patient  must  be  kept  absolutely  quiet,  the  eyes  should  be  kept 
closed,  the  room  darkened,  and  all  noises  excluded  as  far  as  possible — 
telephone,  house  bell,  etc.,  shut  off.  Ice  should  be  applied  to  the 
mastoid.  Leeches  are  at  times  of  value.  The  continuous  vomiting 
may  be  in  part  relieved  by  swallowing  cracked  ice.  Surgical  inter- 
ference may  be  called  for. 

In  syphilitic  cases  mercurial  injections,  salvarsan,  or  inunctions  are 
called  for.  It  may  be  noted  that  the  acute  labyrinthine  disturbance 
which  has  been  known  to  occur  after  the  use  of  salvarsan  is  possibly 
due  to  the  syphilis  and  not  to  the  arsenic  (Benario). 

Treatment  of  Seasickness. — Seasickness  is  a  special  form  of  dis- 
turbance of  the  labyrinth  due  to  the  continuous  movements  of  the 
endolymph  and  irritation  of  the  receptors.  As  the  stomach  has  little 
or  nothing  to  do  with  seasickness,  diet  has  little  or  no  effect  upon  this 
malady,  and  the  ocean  traveller  need  pay  no  more  attention  to  the 
question  of  food  than  that  dictated  by  common  sense.  Eat  one  should, 
for  there  is  nothing  worse  than  continued  retching  with  an  empty 
stomach. 

If  one  is  predisposed  to  seasickness,  morning  walks  on  deck  before 
breakfast  should  be  dispensed  with.  One  should  try  to  breakfast 
immediately  upon  rising,  and  a  little  fruit  or  other  light  food  eaten 
before  rising  may  be  found  helpful.  What  one  eats  is  of  small  moment; 
the  great  thing  is  to  eat;  but  one  should  avoid  food  which  one  does  not 
like.  There  is  no  potency  in  any  particular  food  in  the  prevention  of 
seasickness. 

Nor  is  alcohol  of  any  use,  unless  enough  be  taken  to  anesthetize 
the  patient.  Indeed  it  is  far  more  likely  to  prove  an  irritant,  espe- 
cially if  the  individual  be  unaccustomed  to  its  use.  The  value  of 
champagne  is  largely  psychogenic. 

One  should  not  go  to  dinner  until  it  is  just  about  to  be  served,  thus 
avoiding  the  discomfort  of  waiting  in  a  stuffy  and  perhaps  overheated 
dining  room.  When  the  meal  is  over  it  is  well  to  lie  down,  rather 
than  go  for  a  tramp  on  deck  in  the  hope  that  it  will  aid  digestion. 

Warm  clothing  and  wraps  should  be  taken  on  a  sea  voyage  even  in 
very  warm  weather.  Cold,  damp,  and  foggy  weather  is  apt  to  be 
met  with  on  the  ocean  at  any  time  of  the  year,  and  the  consequent 

1  Equilibrium  and  Vertigo,  Philadelphia,  1918. 


DISORDERS  OF  THE   TASTE  APPARATUS  347 

chilliness,  added  to  that  of  an  unstable  vasomotor  control,  through  the 
labyrinth,  is  a  great  cause  of  discomfort,  which  may  be  removed  or 
alleviated  by  wearing  warm  outer  garments. 

If  the  sea  be  at  all  rough  and  the  motion  of  the  vessel  appreciable, 
the  sensitive  traveller  should  lie  down  at  once,  as  it  is  easier  to  accustom 
oneself  to  the  labyrinthine  h;y'perstimulation  in  a  recumbent  position, 
especially  if  one  adopts  the  position  in  which  the  motion  is  least  felt 
in  the  superior  canals,  i.  e.,  one  should  lie  down  as  flat  as  possible — 
semireclining  does  not  so  place  the  plane  of  the  semicircular  canal  as 
to  cause  the  least  possible  flow  of  fluid  within  it.  One  flat  pillow  is  all 
that  one  should  use  since  half-sitting  up  is  as  bad  as  standing  up. 
Chairs  should  be  shifted,  if  possible,  according  to  the  pitch  or  roll  of 
the  vessel. 

As  soon  as  the  first  s^Tiiptoms  of  seasickness  are  felt  the  patient 
should  lie  down,  if  possible,  on  deck.  As  eye  movements  aid  in  causing 
seasickness,  one  should  close  the  eyes  if  there  is  much  motion  of  the 
ship,  so  as  to  relieve  the  muscles  from  the  constant  adjustment  neces- 
sary in  watching  a  rising  and  falling  horizon,  and  in  very  bright 
weather,  colored  glasses  should  be  worn  to  subdue  the  glare.  It  is  a 
good  plan  to  face  the  cabin  rather  than  the  sea. 

Reading  continuously  is  rather  to  be  avoided,  therefore  books 
should  be  chosen  which  will  allow  one  to  close  one's  eyes  and  meditate. 
Cards  or  other  games  which  hold  the  attention  are  very  helpful. 

In  making  choice  of  rooms,  one  should  give  preference  to  those  in 
the  middle  of  the  boat  where  the  motion  is  less.  To  overcome  the 
smells  and  stuffiness  incident  to  ocean  travel,  one  should  keep  plenty 
of  air  circulating  in  one's  stateroom,  unmindful  of  drafts,  which  are 
of  much  less  consequence  that  one  is  prone  to  think  them. 

Eating  fruits  and  salads,  drinking  plenty  of  liquids,  and  occasion- 
ally taking  a  pill  of  aloes,  aloes  and  mastiche,  or  similar  laxative,  is 
generally  sufficient  to  counteract  the  constipation  which  is  a  frequent 
consequence  of  the  unusual  change  of  habits,  especially  when  one  eats 
very  little. 

The  headache  of  seasickness  is  best  combated  by  eating,  by  coffee, 
and  by  small  doses  of  bromids  and  phenacetin.  The  widely  used 
headache  mixtures  incorporating  caft'ein  and  antipyrin  in  the  elixir 
of  sodium  bromid  are  useful.  The  sodium  salt  of  barbital  in  doses  of 
from  8  to  10  grains,  given  by  rectum  in  suppository,  is  a  very  useful 
remedy  in  causing  sleep  and  in  relieving  excessive  irritability  of  the 
labyrinth. 

Vn.  DISORDERS  OF  THE  TASTE  APPARATUS. 

Glossopalatine  and  Glossopharyngeal  Nerves. — The  taste  apparatus 
is  a  highly  complex  one.  It  is  intimately  bound  up  with  the  olfactory 
apparatus  and  in  many  respects  its  anatomical  developments  have 
followed  along  analogous  lines.  Taste  discrimination  is  largely  a 
chemical  dift'erentiation  which  from  the  lower  animals  up  has  become 
more  and  more  circumscribed.    In  fishes  the  skin  contains  taste  buds 


348  CRANIAL  NERVES 

which  have  certain  powers  of  differentiation  and  the  gills  of  fishes  also 
contain  receptors  to  react  to  chemical  stimuli.  The  comparative 
physiology  of  taste  cannot  be  followed  further  here,  important  though 
it  be,  for  an  understanding  of  the  different  physiological  functions  of 
man  in  that  extremely  important  of  all  vegetative  needs,  food  selection.' 

The  chief  receptors  for  taste  in  man  are  distributed  in  the  tongue, 
the  sides  of  the  mouth  at  the  lateral  edge  of  the  tongue,  the  posterior 
part  of  the  mouth  and  in  parts  of  the  nasopharynx.  These  taste 
buds  are  chiefly  located  in  the  fungiform,  the  vallate  and  circum val- 
late papilla?.  The  receptor  organ  proper,  by  which  chemical  stimuli  are 
reacted  to  and  provide  means  of  chemical  discrimination  is  the  gusta- 
tory filament  from  which  the  nerve  fiber  proceeds  to  make  up  the  two 
chief  nerve  structures  destined  to  carry  the  various  energy  stimuli, 
the  glossopalatine  and  the  glossopharyngeal.  Vegetative  fibers  are 
closely  intermingled  in  the  peripheral  course  and  cannot  be  more  fully 
discussed.     (See  Winkler,  Kappers,  Cajal,  etc.). 

The  first  synapses  are  located  in  and  around  the  papillae  and  present 
many  analogies  with  the  olfactory  synapses  of  the  Malpighian  glomeruli 
of  the  smell  apparatus,  and  the  neuro-epithelial  synapses  of  the  light- 
receiving  apparatus.  Both  vegetative  and  sensorimotor  synapses  are 
probably  present  already  combined. 

The  centripetal  system  begins  with  the  perigemmal  plexus  and  both 
types  of  nerve  fibers  and  cells  are  found  in  its  course. 

The  discussion  as  to  the  taste  nerves  has  undergone  many  vicissitudes, 
but  it  seems  fairly  well  established  that,  apart  from  the  vegetative 
systems,  the  anterior  two-thirds  of  the  tongue  gives  rise  to  a  special 
gustatory  nerve,  the  glossopalatine,  and  the  posterior  part  of  the  buccal 
cavity  gives  rise  to  the  gustatory  parts  of  the  glossopharyngeal  nerve. 
The  trigeminus  probably  carries  no  taste  fibers.  It  does  carry  tactile 
sensations  from  the  tongue,  how^ever,  which  tactile  sensations  are  often 
mistaken  by  patients,  or  confused  with  taste  sensations.  There  exists 
a  certain  correlation  between  the  two  sets  of  sensations :  the  tactile  are 
largely  exteroceptive,  whereas  the  fibers  of  the  glossopalatine  have 
an  interoceptive  significance. 

The  glossopalatine  nerve  is  made  up  of  the  peripheral  branches 
from  the  anterior  two-thirds  of  the  tongue,  the  chorda  tympani,  the 
intermediary  nerve  of  Wrisberg,  and  its  chief  ganglionic  synapse  is  the 
geniculate.  Certain  gustatory  paths  from  the  nose,  and  the  palate 
possibly  pass  by  way  of  the  sphenopalatine  ganglion,  the  vidian  nerves, 
and  the  large  superficial  petrosal  to  reach  the  glossopalatine. 

The  glossopharyngeal  carries  the  major  portion  of  the  taste  fibers 
from  the  circumvallate  papillse  of  the  posterior  part  of  the  tongue  and 
buccal  cavity.  Certain  fibers  from  the  pharynx  seem  to  traverse  by 
way  of  the  superior  laryngeal  branch  of  the  vagus,  but  the  larger 
portion  pass  by  the  dorsal  radicular  root  of  the  glossopharyngeal  to  the 
petrosal  ganglion. 

1  Kappers:  Der  Geschmack,  1914.  See  also  Smith,  G.  E.:  The  Cerebral  Cortex, 
Croonian  Lectures,  1919.    Bean:  The  Cephalic  Nerves,  Anatomical  Record,  July,  1913. 


DISORDERS  OF  THE  TASTE  APPARATUS 


349 


The  third  (?)  neuron  proceeds  from  the  petrosal  and  geniculate 
ganglia  to  make  up  a  circumscribed  tract,  the  tractus  solitarius,  which 
runs  along  beneath  the  epend\Tiia  of  the  fourth  ventricle. 

Winkler  distinguishes  at  least  four  sets  of  fibers  in  the  tractus 
solitarius:  (1)  Some  small  myelinated  fibers,  coming  from  the  region 
of  the  skin  innervated  by  the  vagus  or  the  glossopharyngeal.  (2)  A 
larger  group  of  larger  myelinated  fibers  follow  the  tractus  solitarius  to 
form  synapses  in  its  ventral  nucleus.  These  carry  proprioceptive 
impulses  and  enter  into  the  formation  of  the  median  lemniscus.  (3) 
A  still  larger  group  of  very  fine  fibers  myelinated  and  unmyelinated, 
entering  the  dorsal  nucleus  in  part  and  other  nuclei,  the  sensory  nuclei 
of  the  IX,  X  the  nucleus  rotundus  X  and  the  nucleus  commissuralis,  XI. 
These  fibers  conduct  interoceptive  impressions  derived  from  chemical 


Fig.  202.— The  taste  pathways.     (Winkler.) 


receptors  of  the  internal  surfaces  of  the  body  determining  very  unpor- 
tant  conditioned  reflexes  for  the  digestive  functions,  saliva,  gastric 
juice,  etc.,  and  are  related  to  the  vegetative  reactions  of  vomiting, 
hiccoughing,  strangling,  etc.  (4)  A  group  of  fine  fibers  with  little  or 
no  myelin  sheathing,  which  coming  from  the  glossopalatine  and  glosso- 
pharyngeal nerves  are  the  chief  gustatory  fibers.  This  portion  of  the 
tractus  solitarius  containing  chiefly  the  gustatory  fibers  is  termed 
the  pars  praevagalis  tractus  solitarii  and  enters  the  primary  gustatory 
nucleus.  This  nucleus  is  a  gelatinous  circumscribed  mass  situated 
along  the  para  prtevagalis  between  the  nucleus  of  tiie  trigcmiiuis  and 
the  dorsal  sensory  nucleus  of  the  vagus. 

The  mesencephalic  tract  seems  to  pass  from  the  gustatory  nucleus 
bv  wav  of  fine  mvelinated  fibers  in  the  field  of  the  tractus  dorsali 


350  CRANIAL  NERVES 

tegmenti  of  Schultz,  and  pursues  a  course  somewhat  analogous  to  the 
fibers  of  the  olfactory  apparatus,  both  forming  synapses  with  the 
ganglion  dorsalis  tegmenti  proximalis  (Gudden). 

The  synaptic  collaterals  of  this  tract  are  of  considerable  importance. 
Those  to  the  hypoglossal  nucleus  furnish  stimuli  for  projicient  action  of 
the  tongue  to  respond  to  the  conditioned  reflexes  of  taste  and  of  smell. 
A  tractus  reuniens  of  Fuse  permits  salivary  reflexes  to  the  same 
stimuli.  An  important  set  of  fibers  are  in  connection  with  the  vege- 
tative (autonomic)  nucleus  of  the  vagus.  Vascular,  gastric,  cardiac 
and  visceral  vegetative  conditioned  reflex  possibilities  pass  this  way. 
The  cortical  synapses  for  taste  are  not  completely  worked  out.  In  all 
probability  they  are  contained  in  the  rhinencephalon. 

The  tests  for  taste  have  already  been  described  in  the  examination 
methods.^ 

The  chief  disturbances  are  loss  of  taste  of  the  glossopalatine  and 
glossopharyngeal  regions  respectively,  hyperesthesise,  various  vege- 
tative reflex  association  disorders,  often  termed  subjective  disorders 
of  taste. 

Peripheral  lesions  of  the  glossopalatine,  as  in  the  course  of  the  chorda 
tympani  in  the  auditory  canal  cause  loss  of  taste  in  the  anterior  portion 
of  the  tongue.  The  salivary  secretions  may  or  may  not  be  involved  at 
the  same  time  from  simultaneous  involvement  of  vegetative  fibers. 
Peripheral  lesions  of  the  glossopharyngeal  (rare,  save  from  war  wounds) 
causes  loss  of  taste  of  the  posterior  third  of  the  tongue  and  the  pharynx. 

Total  loss  of  or  one-sided  loss  of  taste  may  occur  from  lesions  of  the 
tractus  solitarius  (syphilis,  tumor,  multiple  sclerosis,  syringomyelia 
or  from  involvement  of  the  primary  gustatory  nucleus  from  similar 
processes  (influenza  or  poliomyelitis,  etc.). 

Taste  and  smell  anomalies  are  usually  combined  in  lesions  from  the 
mesencephalic  to  the  cortical  synapses  (corpus  mamillaria,  tractus 
tegmenti  dorsalis  of  Schultz  and  from  rhinencephalon  lesions  (Ammon's 
horn);  taste  alterations  in  epilepsy  resembling  smell  hallucinations. 
A  large  group  of  taste  reflex  perversions  are  found  in  vagotonic  indi- 
viduals chiefly  of  gastric  origin. 

Psychogenic  taste  modifications,  such  as  metallic  tastes,  interpreted 
as  poisons,  are  frequent  in  hysterical  conversions  and  in  certain  pro- 
jections of  paranoid  individuals.  (Cyclothymias,  various  compulsion 
neuroses.) 

Vm.  DISEASES  OF  THE  LARYNGEAL  NERVES. 

Laryngeal  Disorders.^ — The  laryngeal  muscles  are  supplied  by  the 
inferior  or  recurrent  laryngeal  nerve.  The  cortical  origins  are  as  yet 
not  definitely  known  in  spite  of  the  immense  amount  of  experimenta- 
tion.2  That  they  are  in  the  frontal  region  seems  undoubted  but  where 
is  uncertain.    The  conduction  paths  pass  with  other  corticomedullary 

1  See  also  Frankl-Hochwart,  Die  nervose  Erkrankungen  des  Gescbmackes  und 
Geruches. 

2  V.  H.  Grabower:  Zeitschr.  f.  N.  u.  P.,  ref.  1,  p.  611. 


DISEASES  OF  THE  LARYNGEAL  NERVES 


351 


fibers  through  the  knee  of  the  internal  capsule,  apparently  near  the 
corticomedullary    spinal   pathways   for   voluntary   breathing.     The 


Fig.  203. — Organic  laryngeal  paralyses.  /,  left  adductor  paralj-sis  during  inspiration; 
//,  left  adductor  paralysis  during  phonation;  ///,  left  recurrent  laryngeal  paralysis, 
during  inspiration;  IV,  left  recurrent  laryngeal  paralysis,  during  phonation.     (Barwell.) 


Fig.  204. — Psychogenic  laryngeal  paralyses.  F,  abductor  paresis;  all  the  adductors 
affected — phonation;  VI,  adductor  paresis;  arytenoideus  unaffected — phonation;  VII, 
Paralysis  of  the  thyroarytenoide — phonation;  VIII,  Paralysis  of  the  arytenoideus — 
phonation.     (Barwell.) 


352  CRANIAL  NERVES 

medullary  stations  are  better  known,  and  correspond  with  the  nucleus 
ambiguus.  Laryngeal  respiratory  movements  have  their  bulbar 
nuclei  in  the  nucleus  reticularis.    Both  nuclei  are  intimately  associated. 

An  interesting  pathological  series  (Xissl)  from  Ziehen's  clinic  from  a 
patient  with  tabes  and  complete  laryngeal  palsy  showed  degenerative 
changes  in  the  nucleus  ambiguus  and  was  the  basis  of  an  important 
thesis  by  one  of  his  students.^  The  personally  seen  series  leaves  no 
doubt  as  to  the  interpretation  of  the  localization  of  the  phonation  fibers. 
The  peripheral  fibers  seem  definitely  to  pass  with  the  vagus  rather  than 
with  the  spinal  accessory.  The  larynx  also  has  a  rich  vegetative 
innervation. 

In  unilateral  paralysis  of  the  vagus,  usually  peripheral  or  bulbar,  at 
times  capsular  (Avellis  syndrome),  there  is  an  associated  anesthesia  of 
the  paralyzed  side.  In  recurrent  laryngeal  palsy,  from  neuritis,  aortic 
aneurism,  involvement  by  carcinoma  in  neck,  poliomyelitis,  etc.,  the 
voice  is  rough  or  harsh,  the  vocal  cord  in  immobile,  half-way  fixed 
between  abduction  and  adduction.  In  double-sided  palsy  the  phona- 
tion is  lost. 

The  chief  laryngeal  palsies  are  (1)  abductor,  unilateral  or  bilateral, 
(2)  adductor,  and  (3)  thyroarytenoid.  In  unilateral  abductor  palsy 
the  voice  is  unaffected  or  it  breaks  readily  or  may  be  harsh  at  times,  the 
involved  vocal  cord  is  immobile  during  aspiration.  In  the  bilateral 
palsies  the  voice  is  unchanged,  inspiration  is  made  difficult  and  inspira- 
tion choking  and  coughing  frequent. 

Adductor  palsy  is  frequently  psychogenic.  The  patient  loses  the 
voice  and  talks  in  a  whisper.  The  cords  can  move  outward  but  do 
not  come  together. 

Thyroarytenoid  palsy  causes  hoarseness,  the  cords  are  orally  mar- 
gined although  freely  movable. 

E.  DISEASES  OF  THE  SPINAL  ACCESSORY  AND  HYPOGLOSSAL 

NERVES. 

Accessorius. — The  spinal  accessory  nerve  innervates  the  sterno- 
cleidomastoid and  the  upper  fibers  of  the  trapezius.  Branches  to  the 
vagus  are  known.  Its  cortical  origin  is  not  definitely  localized.  The 
supranuclear  pathways  pass  through  the  internal  capsule  to  end  in 
part  in  the  medulla  near  the  olive  and  vagus  nuclei,  in  part  in  the 
anterior  horns  of  the  six  upper  cervical  "  spinal"  segments.  The  com- 
bined branch  passes  by  way  of  the  jugular  foramen  to  its  muscle 
distribution,  being  combined  with  vegetative  fibers  from  the  cervical 
plexus.     Properly  speaking,  it  is  a  spinal  not  a  cephalic  nerve.^ 

The  chief  lesions  causing  disorder  of  the  spinal  accessory  functions 
are  traumata  (bullets,  operations  for  tuberculous  glands),  lesions  of 
the  cervical  cord,  syringomyelia  of  the  cervical  vertebrae,  multiple 
sclerosis,  neuritides  and  poliomyelitides. 

'  Wyscheslawtzewa:  Berlin  Thesis,  1909. 

2  Bean,  R.  B.:    The  Cephalic  Nerves,  Anatom.  Record,  July,  1913,  vii. 


DISEASES  OF  ACCESSORY  AND  HYPOGLOSSAL  NERVES     353 

Clinical. — Cortical  disturbances  (first  motor  neuron)  cause  irregular 
and  spasmodic  actions.  These  are  seen  in  certain  epilepsies,  usually 
conditioned  by  cerebral  s\']Dhilis,  multiple  sclerosis  or  other  brain 
disorder. 

The  various  tics  (wry-neck,  etc.)  are  cortical,  mostly  psychogenic  in 
origin.  They  represent  compulsion  neuroses  for  the  most  part.  (See 
Psy  choneur  OSes . ) 

Nuclear  affections  of  the  accessorius  are  rare. 


Fig.  205. — Spasmodic  torticollis.  Head  drawn  backward  and  chin  up,  due  to  involve- 
ment of  the  right  trapezius,  probably  with  certain  deep  neck  muscles,  in  addition  to  the 
manifest  spasm  of  the  sternomastoid.     (Massachusetts  General  Hospital.) 


Peripheral  palsies  are  not  infrequent  and  are  due  to  various  injuries, 
direct,  through  disease  of  the  base  of  the  skull,  syphilis,  osteitis. 
They  cause  degrees  of  loss  of  power  to  pull  the  face  to  one  side,  with 
tendency  to  contraction  of  the  opposite  side  (caput  obstipum).  Elec- 
trical changes,  R.  I).,  atrophy,  loss  of  reflexes,  are  present  in  the 
nuclear  and  peripheral  palsies,  but  are  absent  in  the  central  palsies 
or  centrally  induced  torticollis.  Trapezius  palsy  causes  an  altered 
neck  line  from  ])rominence  of  the  levator  anguli  scapulae,  the  scapula 
is  also  dis])lac('d  outward  and  downward  and  rotated  outward,  the 
inner  border  ruiuiing  upward  and  outward  rather  than  parallel  with 
the  spine. 

Treatment  will  \ary  with  cause.  It  should  be  emphasized  that  the 
surgical  treatment  of  s})asm()di('  torticollis,  which  in  the  overwhelming 
majority  of  cases  is  a  psychogenic  reaction,  usually  a  compulsion 
23' 


354 


CRANIAL  NERVES 


neurosis,  is  useless.  Psychoanalysis  and  reeducation  have  been  much 
more  valuable. 

Hypoglossus. — The  hypoglossal  nerves,  twelfth  pair,  are  the  chief 
motor  nerves  of  the  tongue.  Through  collaterals  they  also  send 
motor  fibers  to  the  sternohyoid  and  sternoth\Toid  muscles.  The 
cortical  origins  lie  in  the  lower  portion  of  the  central  convolutions. 

The  supranuclear  pathways  are  followed  with  considerable  difficulty. 
In  the  cerebral  peduncles  they  lie  in  the  center  somewhat  more  median 
than  the  facial;  within  the  internal  capsule  they  lie  at  the  knee.  The 
supranuclear  pathways  decussate  freely  and  make  their  medullary 
synapse  (nucleus  of  the  h^'poglossus)  in  the  lower  two-thirds  of  the 
medulla,  stretching  as  far  do^Aii  as  the  pjTamidal  crossing,  ventrally 

from  the  central  canal  to  the  mid- 

«line.  At  least  ten  to  fifteen  root 
bundles  pass  from  the  hypoglossal 
nuclei  between  the  p;vTamidal  tracts 
and  the  olive,  and  join  together  for 
a  short  distance  within  the  hypoglos- 
sal canal,  at  the  orifice  of  which  the 
hjT)oglossal  vein,  which  is  in  con- 
nection with  the  occipital  sinus,  sur- 
rounds it.  The  canal  is  narrow  and 
short — ^less  than  half  an  inch — lying 
close  to  the  occipito-atlantic  articu- 
lation, at  a  place  where  fracture  of 
the  base  of  the  skull  is  very  apt  to 
affect  it. 

At  its  exit  from  the  skull  the  hypo- 
glossal lies  median  to  and  dorsal  of 
the  vagus  and  of  the  internal  jugular 
vein,  proceeds  laterally,  passes  be- 
neath the  stylohyoid  muscle  and  the 
posterior  belly  of  the  digastric,  splits 
into  its  various  branches,  and  inner- 
vates the  muscles  already  mentioned. 
Anastomoses  with  the  ganglion  nodosum,  superior  cervical  ganglion, 
the  lingual  and  first  and  second  cervical  nerve  take  place.  A  special 
branch,  the  ansa  hypoglossi  is  formed  by  anastomoses  of  the  descend- 
ing branch  of  the  h\T)oglossal,  and  branches  from  the  second  and 
third  cervical  nerves. 

The  chief  physiological  functions  by  symmetrical  innervation  are 
as  follows:  The  genioglossus  muscle  moves  the  tongue  forward  and 
down,  the  hypoglossus  muscle  moves  the  tongue  back  and  up,  the 
styloglossus  moves  the  base  of  the  tongue  up  and  back.  In  asj-m- 
metrical  innervation — that  is,  loss  on  one  side — the  combined  action 
of  these  three  muscles  causes  the  tongue  to  deviate  in  toto  to  the 
paralyzed  side. 
Affection  of  the  longitudinal  muscle  by  symmetrical  innervation 


Fig.  206. — Paralj^sis  of  right  spinal 
accessory  nerv-e. 


DISEASES  OF  ACCESSORY  AND  HYPOGLOSSAL  NERVES     355 

causes  shortening  of  the  tongue,  either  pulUng  the  top  of  the  tongue 
up  or  do\Mi.    By  as;)Tnmetrical  innervation  the  anterior  portion  of  the 


Fig.  207. — Pathways  of  the  taste  fibers.  7,  ophthalmic  branch  of  V;  II,  maxillary 
branch;  ///,  mandibular  branch;  eg,  cortical  taste  area;  fg,  central  ascending  taste 
fillers  in  median  Icmni.scus;  fg,  subcortical  paths;  Gg,  geniculate;  Gvp,  jugular  and 
petrosal  ganglia  of  the  glos.sophuryngeus;  (a,  central  ascending  fibers  of  trigeminus  in 
median  lemniscus;  <«',  subcortical  connections  of  the  thal.TDuis  with  the  inferior  posterior 
central  gyrus.     (Bechterew.) 

tongue  is  pushed  to  the  paralyzed  side.    Any  loss  of  the  function  of 
the  branch  which  innervates  the  transverse  muscles  brings  about  a 


356 


CRANIAL  NERVES 


narrowing  of  the  tongue,  whereas  symmetrical  affection  of  the  vertical 
muscles  produces  a  flattening  of  the  tongue.  The  geniohyoid  raises 
the  hyoid  bone,  pulling  it  forward  when  the  lower  jaw  is  fixed,  or 
pulls  the  loAver  jaw  down.  When  the  hyoid  bone  is  fixed  the  sterno- 
hyoid and  the  thyreohyoid  pull  on  the  hyoid  bone. 

Peripheral  lesions  of  the  h^-poglossal  are  the  result,  usually,  of 
mechanical  causes,  resulting  either  from  fracture  of  the  base  of  the 
skull,  from  tumors,  direct  injury  or  tuberculosis,  or  dislocation  of  the 
upper  cervical  vertebrae.  Cerebrospinal  s^'philis,  particularly  of  long 
standing,  is  a  not  infrequent  cause  of  peripheral  palsies,  while  poison- 
ing from  lead,  arsenic,  alcohol,  carbon  monoxide  may  cause  peripheral 
lesions. 


Fig.  208. — Atrophy  of  right  half  of  tongue.     Cerebral  syphilis  and  injury. 


Nuclear  and  supranuclear  affections  of  the  hypoglossal  are  due 
to  hemorrhage  within  the  medulla.  Poliomyelitis,  tumors,  sx^^hilis, 
and  multiple  sclerosis — these  are  the  most  frequent  cause  of  nuclear 
or  supranuclear  lesions  of  these  nerves. 

Isolated  cortical  lesions  cause  anarthrias,  dysarthrias,  tongue 
ataxias.  Psychogenic  speech  disturbances  abound  in  various  forms 
of  stuttering,  stammering  and  other  compulsive  disorders. 

Clinical. — The  most  frequent  lesion  of  the  hypoglossal  is  unilateral. 
There  is  atony  of  the  longitudinal  muscles  of  the  paralyzed  side,  and 
when  the  tongue  lies  quiet  in  the  mouth  its  apex  deviates  slightly  to 
the  non-paralyzed  side.    The  base  of  the  tongue  usually  rises  higher 


DISEASES  OF  ACCESSORY  AND  HYPOGLOSSAL  NERVES     357 

on  the  paralyzed  side  than  on  the  sound  side  as  a  result  of  atony  of 
the  hypoglossal  muscle. 

Movements  of  the  tongue  are  diminished;  it  becomes  difficult  to 
remove  food  which  lies  between  the  teeth  and  the  cheek,  and  it  becomes 
difficult  for  the  patient  to  direct  the  tongue  to  the  teeth  on  the  para- 
lyzed side.  On  thrusting  the  tongue  out  it  deviates  to  the  sound  side. 
Speech  disturbances  are  present,  especially  involving  the  labials  and 
Unguals. 

In  long-standing  disease  atrophies  develop  with  fibrillary  twitchings 
and  tremors  and  pronounced  motion  irregularities.  Electrical  stimu- 
lation shows  reaction  of.  degeneration. 

In  bilateral  paralyses  speech  disturbances  are  very  marked.  Chew- 
ing and  swallowing  are  rendered  difficult,  and  the  movements  of  the 
tongue  are  markedly  diminished  in  all  directions. 

In  nuclear  palsies,  atrophy  and  fibrillary  twitching  are  marked,  the 
speech  disturbances  are  pronounced,  the  chief  characteristic  being 
what  is  termed  "hot-potato  speech."  The  patient  speaks  as  though 
he  had  a  hot  morsel  in  his  mouth.  Reaction  of  degeneration  is  also 
present.  Supranuclear  palsies,  such  as  occur  in  hemiplegia,  involve 
the  muscles  as  a  whole,  cause  deviation  of  the  tongue  to  the  paralyzed 
side,  and  other  signs  of  hemiplegia  are  present.  Isolated  cortical 
spasm  of  the  hypoglossal  may  be  present. 

Psychogenic  hypoglossal  disturbances  are  by  no  means  rare.  These 
consist  of  tongue  tics,  lisping,  stammering,  stuttering,  of  constant 
tongue  movements,  such  as  are  seen  in  hysterias,  in  patients  with 
dementia  precox,  compulsion  neurosis,  and  in  various  paranoid  types 
of  thinking. 

In  hysterical  tongue  paralyses,  which  are  by  no  means  uncommon, 
resistance  to  passive  motion  of  the  tongue  is  seen.  There  are  no 
electrical  changes  and  the  speech  disorder  is  apt  to  be  obvious. 

Treatment  of  hj-poglossal  disturbances  varies  according  to  the 
cause  and  is  usually  purely  symptomatic. 

Speech  Disturbances. — Human  expression,  articulatory,  mimetic, 
tactile,  or  by  writing,  is  a  highly  complicated  mechanism.  It  includes 
all  of  those  movements  resulting  from  optical,  auditory  or  tactile 
contacts  by  which  communication  between  individuals  is  brought 
about  for  social  purposes.  Language  as  it  fully  develops  is  therefore 
a  tool  with  which  one  may  cut  into  reality  and  utilize  the  facts  of 
nature  for  purposes  of  adaptation. 

All  kinetic  speech  disturbances  may  be  at  first  separated  into  those 
in  which  the  receptive  (sensory)  side  of  the  pathways  are  involved  and 
into  those  in  which  the  productive  (or  purely  motor)  part  of  the  arc  is 
implicated. 

On  the  sensory  side  one  finds  the  gradual  accumulation  of  expe- 
rience, chiefly  through  auditory  stimuli  with  the  gradual  evolution  of 
speech  symbols  (language),  which  stand  in  the  developing  psyche  for 
the  image  of  things,  ideas  or  feeling  values.  Thought  is  symbolic 
action. 


358  CRANIAL  NERVES 

Optical  stimuli — objects,  signs,  various  gl^-^^hs,  words,  letters — ■ 
unite  to  form  complex  types  of  expression  in  writing  (psychically 
developed  to  conquer  spatial  limitations),  while  tactile  stimuli  are 
an  integral  part  of  language  from  the  more  restricted  side  of  tactile 
reading  of  blind  and  tactile  speech  of  the  deaf  and  dumb  to  the  sensory 
stimuli  of  the  movements  of  the  muscles  and  tongue  and  lips  in  speech. 
A  complete  analysis  of  the  great  complex  of  sensory  factors  which 
ultimately  finds  outlet  in  speech  symbolism  with  its  infinite  psychical 
implication  is  not  possible  here.  It  would  involve  the  entire  problem 
of  the  evolution  of  civilization  and  culture.^ 

The  productive  side  of  the  speech  mechanism  is  less  complicated. 
Originally  showing  itself  in  the  child  as  a  noisy  symbol  of  crying,  there 
is  gradually  shaped  by  the  libido  more  and  more  accurate  sounds  of 
expression  to  meet  the  needs  of  hunger  and  of  love.  Grunts,  laughs, 
gurgles  evolve  into  more  precise  formulations,  until  the  rich  symbol- 
izations  of  speech  are  gained,  with  all  their  advantages  of  precision 
and  efficiency. 

Here  the  muscles  of  the  mouth,  lips,  larynx,  chest,  abdomen,  arms, 
and  pelvis  all  come  into  a  gradually  refining  and  orderly  series  of 
coordinated  activities. 

As  a  celebrated  French  philosopher  has  phrased  it:^  "If  the  ants 
have  a  language,  the  signs  which  compose  it  must  be  limited  in  number, 
and  each  of  them,  once  the  species  is  formed,  must  remain  attached  to 
a  certain  object  or  a  certain  operation;  the  sign  is  adherent  to  the  thing 
signified.  In  human  society,  on  the  contrary,  manufacture  and  action 
are  of  variable  form,  and,  moreover,  each  individual  must  learn  his 
part,  because  he  is  not  preordained  to  it  by  structure.  So  a  language  is 
required  which  makes  it  always  passing  from  whatsis  known  to  what 
is  yet  to  be  known.  There  must  be  a  language  whose  signs  which, 
cannot  be  infinite  in  number,  are  extensible  to  an  infinity  of  things. 
This  tendency  of  the  sign  to  transfer  itself  from  one  object  to  another 
is  characteristic  of  human  language.  It  is  observable  in  the  little 
child  as  soon  as  he  begins  to  speak.  Immediately  and  naturally  he 
extends  the  meaning  of  the  words  he  learns,  availing  himself  of  the 
most  accidental  connection  or  the  most  distant  analogy  to  detach 
and  transfer  elsewhere  the  sign  that  has  been  associated  in  his  hear- 
ing with  a  particular  object.  'Anything  can  designate  anything'  is 
the  latent  principle  of  infantile  language."  The  truth  of  this  is 
amply  confirmed  in  the  studies  of  symbolism  in  the  psychoneurotic 
symptoms,  the  language  of  dreams  and  of  delusional  thinking.  These 
are  discussed  in  the  chapters  dealing  with  disorders  of  social 
adjustment.  Part  III. 

Many  schemes  have  been  devised  to  set  forth  graphically  some  of 
the  phases  of  these  kinetic  speech  disturbances.  One  of  Veraguth's 
is  here  utilized. 

Here  1    represents  the   incoming  auditory  stimuli   (tone,   sound, 

1  Gutzmann :   Sprach-Storungen.     Various  monographs  on  Aphasia. 
*  Bergson:   Creative  Evolution,  Henry  Holt  &  Co.,  1911. 


DISTURBANCES  OF  SPEECH 


359 


words)  with  their  more  or  less  sharply  defined  sensorial  perceptions 
gained  gradually  through  experience.  They  constitute  in  their  totality 
the  various  audible  components  of  speech  and  are  constituents  of 
organic,  auditory  memories.  The  circle  /  represents  such  a  psycho- 
physiological combination,  rather  than  an  anatomical  hearing  area  or 
zone,  which  latter  is  roughly  outlined  in  the  first  and  second  temporal 
convolutions. 

Pathway  2  represents  the  optic  as  well  as  the  tactile,  and  kines- 
thetic neuron  chain  which  conveys  to  the  brain  centrals  graphic  symbols 
(pictures,  diagrams,  graphs,  letters,  etc.).  The  general  assembly  place 
of  these  is  represented  by  the  circle  II.  It  represents  not  an  anatom- 
ically circumscribed  area,  but  rather  a  functional  capacity,  but  not 
unrelated  to  an  optical  sensory  area  in  the  cuneus  and  precuneus  of  the 
occipital  lobes. 


12 


II 


y_— — -""^ 

8 

/ 

\ 

8                ~~-^-/ 

^^^--^ 

/ 

A 

\ic 

J^-^^ 

N, 

5 

/ 

=4< 

7     T 

[J 

--.A 

•"''  1 

I    i 

A-'- 

1 

~~-- 

~~~ 

— f — 

.—'-""' 

3 

1 

N 

' 

Fig.  209. — Scheme  of  speech  connections.     (Veraguth.) 


Outgoing  pathways  3  and  4  represent  the  motor  side  of  the  arcs 
of  expression  by  all  those  motions  by  w^hich  the  act  of  articulation 
with  infinite  variation,  shades  and  nuances,  and  those  of  graphic 
representation  are  carried  out.  Both  pathways  are  related  to  cortical, 
bulbar,  spinal  localizations,  which  make  functional  unity  possible 
as  speech  and  writing  (in  widest  sense).  These  are  symbolized  by 
circles  ///  and  IV .  All  of  these  are  brought  together  in  an  enlarged 
concept  (circle  F),  w^hich  symbolizes  the  heard,  read,  spoken,  or  written 
mode  of  expression  (words,  acts,  representation,  mimic,  etc.). 

The  lines  which  bind  these  various  centers  represent,  therefore,  a 
series  of  possibilities.  Whereas  an  anatomical  substratum  underlies 
these  possibilities,  no  attempt  will  be  made  to  represent  them  here. 

1.  Repetition  of  words  without  comprehension — Pathways  1,  5,  3. 

2.  Reading  aloud  without  comprehension — Pathways  2,  7,  3. 

3.  Writing  to  dictation — Pathways  1,  6,  4- 

4.  Writing  to  dictation  without  sense — 2,  8,  4- 

5.  When  heard  word  is  comprehended — /,  9. 


360  CRANIAL  NERVES 

6.  When  read  word  is  comprehended — S,  12. 

7.  Spontaneous  speech  of  an  idea — 10,  3. 

8.  Spontaneous  graphic  expression  of  an  idea — 11,  4- 

9.  When  heard  word  is  comprehended  and  reproduced  by  speech — 

1,  9,  10,  3. 

10.  When  heard  word  is  comprehended  and  reproduced  graphically 
-1,  9,  11,  4. 

11.  ^^^len  read  word  is  comprehended  and  reproduced  by  speech — 

2,  12,  10,  3. 

12.  When  read  word  is  comprehended  and  reproduced  graphically 
-2, 12,  11,  4. 

The  scheme  also  attempts  to  show  an  internal  and  external  speech. 
At  the  present  time  exact  correlation  between  all  tj'pes  of  speech 
disturbance  and  definite  pathways  cannot  be  made.  But  in  the  main 
enough  broad  facts  have  accumulated  to  permit  certain  fairly  exact 
generalizations.  In  the  first  place  the  general  speech  mechanisms 
are  located  predominantly  in  the  left  hemisphere  in  right-handed 
individuals.  In  the  left-handed  the  localization  is  predominantly  in 
the  right  hemisphere.  Ambidextrous  brains  are  known  and  educable 
opposite  speech  areas  are  known. 

While  in  the  discussion  of  the  aphasias,  one  speaks  of  speech  areas, 
auditory  (temporal),  optic  (occipital),  motor  (Broca's  convolution) 
and  attempts  to  localize  them,  the  fact  is  that  the  architecture  of  the 
brain  is  so  complex,  the  pathways  utilized  in  the  speech  mechanisms 
so  widespread,  that  it  is  better  to  speak  of  aphasia  areas.  These  are 
areas  of  special  predilection  for  the  occurrence  of  speech  disturbances 
which  are  more  or  less  stereotA^ped.  These  are  areas  which  are  sup- 
plied more  particularly  by  the  1,  2,  3,  4  branches  of  the  Sylvian  artery, 
distributed  to  the  third  frontal  convolution  and  operculum  of  the 
central  convolution,  Broca's  area  (motor  speech  asphasias),  the 
insula  (Heschl  convolution),  the  posterior  part  of  the  first  temporal 
auditory  centers  (amnesic  aphasia)  and  the  angular  gyrus,  cuneus 
(optic  alexias). 

These  areas  are  well  shown  in  v.  Monakow's  diagram  here  repro- 
duced. 

Clinical  Forms. — These  may  be  subdivided  into  external  and 
internal  speech  disturbances. 

I.  Deafness  brings  about  a  special  form  of  speech  disturbance 
(deaf -mutism).  Even  though  the  speech  apparatus  be  intact,  it 
lacks  the  dynamic  stimuli  to  be  utilized.  When  speech  is  acquired, 
it  has  a  peculiar  monotonous  quality.  Certain  forms  of  mutism 
from  faulty  hearing  are  to  be  distinguished. 

II.  Dysarthrias. — Used  in  a  broad  sense,  these  include  disturbances 
in  speech  due  to  defect  in  the  productive  pathway. 

(a)  They  may  be  of  purely  psychogenic  origin,  i.  e.,  compulsive 
ideas,  hysterical  conversions,  psychotic  symbol  distortion,  such  as 
are  evidenced  by  stammering,  stuttering,  hysterical  speech,  katatonic 
speech. 


DISTURBANCES  OF  SPEECH  361 

(b)  Peripheral  motor  palsies;  facial,  palate  (rhinolalia). 

(c)  Bulbar  palsies  (mouthful  speech),  as  seen  in  progressive  muscular 
atrophy  (Aran-Duchenne  type,  often  s\'])hilitic)  in  amyotrophic  lateral 
sclerosis,  in  acute  poliomyelitis,  in  multiple  sclerosis,  syringomyelia, 
in  tumors  of  the  medulla  and  pons  and  in  general  paresis. 

(d)  In  disorders  of  the  muscular  tonus  mechanism  of  the  midbrain, 
cerebellar  paths,  corpora  striata,  as  in  acute  choreas,  Huntington's 
chorea,  paralysis  agitans,  multiple  sclerosis,  one  finds  incoordination 
or  scanning,  jerky  or  monotonous  speech. 

The  disturbances  of  inner  speech  are  termed  aphasias.  They  are 
here  divided  into  (.1)  Receptor,  and  (B)  Productive,  which  are  prac- 
tically s\aionymous  with  sensory  and  motor  aphasia  respectively. 

Sulcus  centralis 


Kossa  S3'lvii 
Fig.  210. — The  aphasia  regions  in  the  left  hemisphere.     (Veragiith.) 

Aphasias. — Although  a  separation  of  all  aphasias  into  two  groups, 
receptive  and  productive,  is  theoretically  possible,  practically  this  is 
not  the  case.     The  actual  clinical  pictures  seen  vary  enormously. 

In  view  of  the  fact  of  the  millions  of  years  of  continuous  growth  in 
complexity  and  of  effectiveness  of  this  particular  series  of  purposeful 
movements,  it  is  no  wonder  that  the  many  problems  comiected  with 
speech  and  the  mechanism  of  its  production  are  still  far  from  being 
clearly  resolved,  nor  will  this  be  undertaken  here. 

What  is  here  attempted  is  simply  a  general  sketch  of  certain  dis- 
turbances of  speech  which  have  for  many  years  been  called  aphasia. 
No  attempt  will  be  made  to  detail  all  of  the  many  interpretations 
that  have  been  given  to  the  term.  Such  may  be  found  in  large  and 
valuable  works  upon  the  subject,  notably  in  the  monographs  of  Elder, 
Bastian,  Collins  in  English;  of  Kussmaul,  Wernicke,  von  Monakow, 
Niesl  von  Mayendorf  in  German;  and  those  of  Dejerine  and  Miraille, 
Marie  and  Moutier  in  French.  The  analysis  of  the  problem  has  been 
found  to  be  more  and  more  complex,  successively  more  time-consum- 
ing,  money-costing  and   scientifically  difficult.     Each   advance  has 


362 


CRANIAL  NERVES 


cast  into  the  scrap  heap  many  of  the  classics  of  a  generation  past  and 
it  can  be  said  that  the  problems  now  can  be  solved  only  by  the  most 
exacting  and  rigorous  technical  methods  coupled  with  most  penetrat- 
ing and  comprehensive  clinical  observations.  To  completely  analyze  a 
single  case  of  aphasia  according  to  present-day  requirements  requires 
years  of  painstaking  observation,  two  or  three  years  of  more  or  less 
continuous  microscopic  technical  manipulations,  and  at  the  cost  of 
thousands  of  dollars. 

Arm  Centre 


6enu  Corfjus  Callosum 


Splenium Corpus  Callosum 


Occipital 
Lobe 


F3  op. 

Motor  Aphasia  """' 

Sensory  Aphasia 

Fig.  211. — Scheme  of  the  chief  areas  and  pathways  involved  in  aphasic  disturbances. 
Nos.  S,  4'  5,  6,  7,  sensory  pathways;  Nos.  1,  2,  8,  9, 10, 11,  motor  pathways.  Str.,  corpus 
striatum;  Li,  lenticular  nucleus;  Tho,  optic  thalamus;  op.,  operculum;  1,  path  from  left 
temporal  to  right  temporal  by  way  of  the  corpus  callosum;  2,  path  from  occipital  lobe 
to  the  arm  region;  3,  path  from  the  arm  region  to  the  internal  capsule  and  peripherally; 
4,  path  from  temporal  lobe  to  arm  region;  5,  paths  from  the  prefrontals  to  F3;  6,  connect- 
ing path  with  the  right  hemisphere  by  means  of  the  corpus  callosum;  7,  paths  from  F3 
through  the  internal  capsule  downward;  8,  connecting  paths  from  F3  to  Ti,  operating 
both  ways;  9,  path  from  the  internal  genicxilate  to  Ti;  10,  connections  between  angular 
and  supramarginal  gyri  and  Ti;  11,  path  between  occipital  and  Ti.  (Veraguth,  after 
V.  Monakow.) 

As  Adolf  Meyer  once  expressed  it,  "  Now  that  the  North  Pole  and 
the  South  Pole  have  been  discovered,  perhaps  someone  will  take  inter- 
est in  that  most  important  undiscovered  country,  the  human  brain." 
Certainly  no  better  expedition  could  be  fitted  out  than  one  to  explore 
the  uncharted  seas  of  the  human  speech  areas. 

History. — The  work  that  the  early  explorers  did  is  work  that  cannot 
be  thro^Ti  away.  It  outlined  the  chief  landmarks  of  the  country 
which  now  must  be  left  to  the  skilled  pilots  of  the  State  institutions, 
endowed  laboratories  and  university  organizations  with  their  corps  of 
skilled  technicians,  neophytes  to  whom  a  year  is  but  a  day,  and  to 
trained  interpreters  learned  in  the  difficult  charts  of  brain  anatomy. 
The  old-time  dabbling  in  these  problems,  though  dabbling  is  but  a 
relative  term,  has  gone  out  of  fashion. 

To  Bouillaud,  in  1825,  history  turns  for  its  first  noteworthy  find  in 
this  field.    Tinctured  with  the  notions  of  Gall,  Bouillaud's  work  was 


APHASIAS  363 

worth  while,  in  that  he  controverted  the  long-prevailing  and  ortho- 
dox doctrine  of  Flourens  that  the  brain  had  no  influence  either  direct 
or  indirect  upon  the  muscles.  Bouillaud  maintained  that  the  brain 
was  indispensable  for  movement  and  he  very  roughly  localized  the 
organs  of  articulate  speech  in  the  anterior  lobes.  As  Soury^  well  says, 
arguing  from  Bouillaud 's  own  writings  of  1847  and  1865,  he  was  not 
really  a  seeing  pioneer  in  this  limited  field,  although  his  work  was 
full  of  remarkably  clear,  valuable  material.  His  work  was  too  much 
colored  by  the  conceptions  of  Gall,  but  he  was  a  precursor  of  Broca 
who  in  1865  really  charted  the  first  outlines  of  the  aphasia  sea.  Bouil- 
laud, however,  noted  that  articulate  speech  could  be  abolished  without 
paralysis  of  any  of  the  muscles  of  phonation  and  separated  completely 
motor  aphasia  from  dysarthria.  Bouillaud  also  apparently  had  an 
idea  of  what  is  spoken  of  as  internal  speech,  for  he  wTote  as  early  as 
1825  that  "the  loss  of  speech  depends  at  times  upon  the  memorj^  of 
words,  at  times  upon  that  of  the  muscular  movements  of  which  speech 
is  composed,  or  what  is  the  same  thing,  at  times  upon  a  lesion  of  the 
gray  matter  and  at  times  of  the  white  substance  of  the  anterior  lobes." 

Marc  Dax,  writing  in  1836,  how^ever,  made  a  series  of  interesting 
observations  in  which  he  concluded  "that  not  all  diseases  of  the  left 
hemisphere  can  alter  verbal  memory  but  when  this  memory  is  altered 
by  disease  of  the  brain  it  is  necessary  to  seek  the  cause  of  the  disorder 
in  the  left  hemisphere."  Bouillaud  contested  the  notion  which  Dax 
could  not  support  by  autopsy  findings.  Furthermore,  Bouillaud, 
following  a  custom  of  the  times,  offered  a  prize  of  500  francs  to  him 
who  would  show  him  a  single  case  of  the  loss  of  speech  described. 
This  was  in  1848,  and  the  whole  story  of  the  times  and  Broca's  presen- 
tation in  1861  is  well  told  in  an  admirable  critical  essay  by  Marie. 

The  clinical  pictures  noted  by  Bouillaud,  Gall,  by  Dax,  father  and 
son,  IMarce,  Jackson,  Lelut,  Leidat  and  others,  received  their  ana- 
tomical explanation  bj'  Broca,  in  1861,  in  his  first  autopsy  case  of 
Leborgne,  a  man  fifty-one  years  of  age  who  since  the  age  of  twenty- 
one  had  lost  his  use  of  language.  He  could  pronounce  only  a  single 
syllable  which  he  repeated  two  or  three  times  in  succession,  tau,  tau, 
tau.  He  understood  practically  everything  said  to  him.  A  second 
case,  Lelong,  soon  followed,  and  Broca,  then  thirty-seven  years  of 
age,  made  the  deduction  that  the  seat  of  the  lesions  of  motor  aphasia 
(aphemia  he  called  it  at  that  time)  was  in  the  third  left  frontal  con- 
volution. These  two  brains  were  conserved  in  the  Dupuytren  museum 
at  Paris  and  have  been  seen  by  many.  In  1863  Broca  reported  11 
cases  in  which  the  left  third  frontal  convolution  was  involved  and  in 
1865  he  presented  a  general  thesis  upon  the  subject.  He  noted  the 
influence  of  right-sided  lesions  in  left-handed  persons. 

Broca  defined  his  aphemia  in  the  following  words:  "There  are  indi- 
viduals in  whom  the  general  faculty  of  language  persists  unaltered,  in 
whom  the  auditory  apparatus  is  intact,  and  where  all  the  muscles,  not 
excepting  those  of  the  voice  and  of  articulation,  obey  the  will,  and  in 

*  Lcs  Centres  Nerveux.     A  work  which  every  student  of  neurology  should  read. 


364  CRANIAL  NERVES 

whom  as  a  result  of  a  cerebral  lesion  the  articulate  speech  is  abolished. 
This  abolition  of  speech,  in  individuals  who  are  not  paralytics,  nor 
idiots,  constitutes  a  symptom  sufficiently  distinctive  which  it  seems  to 
me  useful  to  designate  under  a  special  name.  I  shall  call  it  aphemia 
(a,  without,  and  4>r}iJLL,  I  speak,  I  pronounce)  for  that  which  these 
patients  lack  is  solely  the  faculty  of  articulating  words.  They  li«ar 
and  understand  all  that  is  said  to  them;  they  have  their  reason,  they 
emit  sounds  with  facility;  they  can  move  the  tongue,  and  the  lips 
much  more  energetically  than  is  necessary  to  articulate  sound  and 
yet,  notwithstanding,  the  response,  while  they  understand  perfectly 
what  they  would  wish  to  say,  is  reduced  to  a  few  articulatory  sounds, 
always  the  same  and  always  uttered  in  the  same  manner.  Their 
vocabulary,  if  one  can  call  it  such,  is  composed  of  a  short  series  of 
syllables,  often  of  a  monosyllable  which  expresses  everything,  or 
rather  which  expresses  nothing,  for  this  unique  word  is  most  often  a 
stranger  to  all  vocabularies.  Certain  patients  have  not  even  a  vestige 
of  articulate  speech;  they  make  vain  efforts  without  pronouncing  a 
syllable." 

Going  into  the  anatomical  correlations  Broca  says,  "Aphemia,  that 
is  to  say,  the  loss  of  speech  without  other  intellectual  disorder,  and 
without  any  paralysis,  has  been  the  consequence  of  a  lesion  of  one  of 
the  frontal  lobes.  In  our  patients  the  site  of  the  lesion  was  in  the 
second  or  third  left  frontal  convolution,  most  probably  in  the  latter. 
It  is  then  possible  that  the  faculty  of  articulate  speech  is  located  in 
one  or  the  other  of  these  convolutions."  It  is  not  necessary  to  proceed 
further  with  his  very  temperate  statement  and  his  careful  wording,  in 
which  we  can  see  his  desire  to  support  a  superior  in  the  Faculty,  Bouil- 
laud,  and  yet  not  offend  him  nor  the  most  active  believers  in  the 
localization  of  Gall.  In  1862  he  saw  a  patient  with  aphemia  from  a 
right-sided  lesion  which  caused  him  to  be  cautious.  In  1863  Parrol 
demonstrated  a  case  of  extensive  destruction  of  the  right  third  frontal 
convolution  without  any  speech  or  intelligence  defect,  which  also 
contributed  much  to  a  very  active  discussion  going  on  in  Paris.  Fin- 
ally in  1865  Broca  came  out  flat-footed  and  said  that  the  third  left 
frontal  convolution  was  the  seat  of  his  aphemia.  Thus,  to  use 
Moutier's  phrase,  the  "dogma"  was  created,  although  it  was  not  by 
any  means  accepted  by  all.  All  of  the  acrimony  of  a  great  discussion 
raged  but  Trousseau  gave  it  the  weight  of  his  great  authority  and  it 
prevailed  for  many  years  and,  gradually  subjected  to  certain  modifi- 
cations, prevails  at  the  present  time. 

Then  began  the  great  period  of  electrical  stimulation  of  the  cortex. 
Localization,  a  fantastic  dream  for  Gall,  became  a  scientific  reality 
for  the  English  physiologists  and  when  in  1870  Fritsch  and  Hitzig 
published  their  studies  upon  electrical  excitability  of  the  cortex  an 
entirely  new  method  of  localizing  muscular  movements  and  the  speech 
mechanism  became  possible.  Meanwhile  the  aphasia  question  was 
actively  studied.  Fleury  (1865)  distinguished  Broca's  aphemias  from 
what  he  called  aphrasics;  they  could  pronounce  but  did  not  use  the 


APHASIAS  365 

right  meaning.  Gardner  (1866)  separated  internal  speech  trouble 
from  intact  ideation.  Ogle  (1867)  fell  upon  the  idea  of  agraphia  and 
Bastian  (1869)  really  discovered  word-deafness.  Paraphasia,  jargon 
aphasia,  were  also  described  by  Jackson  and  other  English  writers. 

The  honor  has  been  given  to  \Yernicke,  however,  for  discovering 
that  the  incoming  receptive  side  of  the  speech  mechanism,  i.  e.,  the 
auditory  understanding  of  speech,  separated  from  the  hearing  of 
sounds,  was  of  immense  importance  in  solving  the  problems  of  speech 
disturbances  and  he  formulated  that  form  of  aphasia  which  is  termed 
sensor}'  aphasia.  These  patients  heard  sounds  but  they  might  as 
well  have  been  Chinese  or  Choctaw.  They  had  lost  their  meanings. 
This  was  in  1874,  and  the  defect  he  then  stated  was  due  to  a  defect  of 
the  first  left  temporal  convolution. 

The  aphasia  problem,  at  first  comparatively  simple,  and  not  yet  a 
general  problem,  began  to  become  complicated.  Wernicke  described 
his  zone  of  language.  There  existed  a  motor  aphasia,  the  aphasia  of 
Broca  and  due  to  disturbance  of  the  F3  left  and  a  sensory  aphasia  due 
to  disease  of  the  first  temporal  of  the  left  side  and  posterior,  in  which 
comprehension  of  spoken  words  was  lost.  This  served  as  a  control  of 
the  motor  center  and  lesions  here  produced  the  various  clinical  pictures 
described  particularly  by  the  English  as  amnesic  aphasia,  paraphasia, 
agraphia,  etc. 

A  most  searching  reanalysis  of  the  entire  question  followed  IVIarie's 
iconoclastic  utterances  in  1906.^  Insufficient  comprehension  of  speech, 
i.  e.,  as  an  intellectual  defect — a  dementia,  Marie  called  it — was  the 
principal  concept  that  ruled  this  presentation  of  aphasia.  The  whole 
problem  is  extremely  technical  and  involved,  and  the  discussion  cannot 
be  entered  into  here.  At  all  events  the  work  of  Marie  caused  several 
realignments  of  former  attitudes  but  did  not  essentially  modify  the 
accepted  types,  a  brief  resume  of  which  is  here  given: 

Clinical  Forms. — ^Aphasia  as  here  understood  consists  in  a  dis- 
turbance or  loss  of  speech  resulting  from  a  failure  to  evoke  or  control 
the  memory  of  those  auditory  or  visual  symbols  used  in  social  inter- 
change of  concepts  or  ideas.  This  loss  of  control  or  failure  to  evoke 
the  proper  symbols  is  due  to  a  definite  structural  change  involving 
the  complex  brain  pathways,  or  it  may  result  from  purely  psychological 
blocking.  It  has  been  seen  that  for  speech  as  for  any  other  volitional 
act,  receptor  and  effector  pathways  must  be  open.  The  receptor 
pathways,  i.  e.,  the  sensory  part  of  the  process,  include  the  hearing 
and  the  seeing  of  word  symbols.  The  memories  functionate  in  certain 
areas  or  zones  which  have  been  termed  auditory  and  visual  word  areas. 
Centers  is  an  older  term  but  is  somewhat  objectionable.  The  audi- 
tory word  zone  or  area  is  located  about  the  upper  surface  of  the  tem- 
poral lobe  in  the  anterior  transverse  gyrus  of  Ileschl  and  extending  also 
into  the  adjacent  portions  of  the  ])osterior  and  of  the  first  temporal 
convolution.    The  gyrus  angnlaris  serves  as  a  visual  area  for  those  who 

'  Seniiiine  M6dicale,  1906,  Nos.  21,  42,  48;  see  also  Moutier's  Thesis  foi  complete 
discussion  of  the  whole  problem. 


366  CRANIAL  NERVES 

have  learned  to  read.  Lesions,  which  occupy  these  locations  or  are 
in  such  a  position  as  to  cut  the  pathways  immediately  related  thereto, 
give  rise  to  word-deafness — auditory  aphasia,  or  word-blindness, 
visual  aphasia.     These  are  the  types  of  so-called  sensory  aphasia. 

The  productive  or  effector  side  of  the  speech  reflex  arc,  i.  e.,  the 
motor  side  wherein  motor  images  more  or  less  functionate,  is,  as 
has  been  pointed  out,  in  and  about  Broca's  convolution  and  the 
adjacent  areas  of  the  precentral  and  insula  convolutions.  Lesions 
here  result  in  motor  aphasia,  or,  as  Broca  called  it  aphemia.  It  may 
be  added  that  Marie's  searching  attempt  to  break  do\\Ti  the  classical 
motor  aphasia,  by  calling  it  anarthria  plus  a  lenticular  lesion  causing 
dementia,  has  not  stood  the  test  of  careful  investigation. 

The  chief  lesions  causing  these  aphasias  are  hemorrhages,  abscess, 
encephalitis,  tumors,  thromboses,  emboli  and  acute  edemas.  Func- 
tional losses  are  seen  resulting  from  the  epileptic  discharge,  from 
certain  hysterical  dissociations,  in  uremia  or  in  severe  angiospasms,  as 
in  migraine  for  example. 

Motor  Aphasias. — In  this  tjT^e  of  aphasia  the  patient  has  lost  the 
capacity  to  express  himself  in  speech.  He  cannot  read  or  talk  spon- 
taneously. He  may  say  a  few  words,  is  usually  able  to  say,  yes,  yes, 
no,  no,  is  irritated  over  his  loss,  frequently  saying  "damn"  or  other 
expletive  in  the  fruitless  and  exasperating  search  for  words.  Typical 
expletives  are  frequent  and  are  often  repeated  over  and  over  again. 
It  is  convenient  to  distinguish  two  types  of  motor  aphasia. 

Subcortical  or  So-called  Pure  Motor  Aphasia. — Here  the  motor  speech 
pathways  on  the  way  out  to  the  medullary  nuclei  are  cut  off.  The 
patient  is  usually  quite  able  to  understand  spoken  and  wTitten  symbols, 
but  cannot  speak  spontaneously,  read  aloud,  or  even  repeat  what  is 
spoken  to  him.  He  usually  is  able  to  write.  Hysterical  dissociation 
may  bring  about  this  tx^ie,  as  well  as  the  concrete  lesions  already 
mentioned. 

Cortical  Motor  Aphasia. — ^This  type,  less  common  and  theoretically 
more  difficult  of  comprehension,  suffers  a  greater  loss  of  internal  speech. 
The  patient  is  u.sually  unable  to  write  and  usually  fails  to  bring  together 
long  sentences  or  complicated  word  relations.  He  fails  to  grasp  them 
in  their  entirety.  The  more  severe  the  speech  defect,  the  more  pro- 
nounced, as  a  rule,  is  the  agraphia. 

Auditory  Aphasias  (Word-deafness) . — The  patient  hears  without  diffi- 
culty but  heretofore  known  symbols  are  now  as  though  foreign.  He 
is  usually  able  to  repeat  the  phrases,  imitate  the  sounds,  but  they 
might  as  well  be  Chinese  for  all  his  comprehension  of  them  or  his 
ability  to  use  them  properly  is  concerned.  Here  also  two  trends  in 
the  symptoms  are  capable  of  fairly  sharp  separations. 

Subcortical  or  Pure  Auditory  Aphasia. — The  pathways  between  the 
receptors  and  their  central  station  are  blocked  just  distal  to  the 
auditory  area.  Spontaneous  speech  does  not  suffer  but  cannot  be 
counted  upon  unless  through  visual  correction.  By  the  guidance  of 
the  written  word  the  patient  may  express  himself  perfectly.    Repeti- 


APHASIAS  367 

tion  of  sound  s\Tnbols  may  be  impaired,  hence  these  patients  cannot 
repeat  spoken  phrases. 

Cortical  Word-deafness. — Spontaneous  speech  is  much  more  involved 
and  the  patient's  talk  is  usually  quite  disturbed  whether  he  attempts 
spontaneous  speech  or  when  reading  aloud.  Internal  speech  is  ser- 
iously disturbed.  The  patient  cannot  repeat,  nor  copy  to  dictation. 
Mistakes  in  orthography  are  frequent.  While  he  may  be  fluent  in  his 
speech  it  may  be  fairly  clear  or  a  mixed-up  jumble.  Furthermore, 
the  patient  is  not  aware  of  his  mistakes  or  only  partially  aware  of  them. 

A  great  variety  of  partial  forms  are  met  with.  One  patient  will  lose 
the  value  of  names,  of  nouns,  of  objects,  others  are  merely  confused 
(paraphasia) . 

Auditory  aphasics  usually  clear  up  but  in  those  patients  who  are  rich 
in  auditory  forms  of  memory,  in  contrast  to  those  whose  memory  type 
is  more  apt  to  be  visual,  the  disability  is  usually  greater. 

Visual  Aphasias  (Word-blindness). — This  type  is  also  spoken  of  as 
alexia  at  times.  The  patient  sees  but  does  not  take  it  in.  Previously 
recognized  signs  are  now  as  though  EgA^tian  or  cuneiform.  Shapes 
are  recognized  and  may  even  be  copied  but  are  not  comprehended. 
They  have  lost  their  acquired  context.  Two  types  are  here  to  be 
recognized  also. 

Subcortical  or  Pure  Word-blindness. — ^The  area  or  center  being  intact 
the  lesion  involves  the  incoming  pathways  adjacent  to  the  cortex. 
The  patient  fails  to  understand  WTitten  words,  cannot  read  aloud  but 
may  write  or  may  copy  from  dictation.  Partial  blindness  (hemianop- 
sia) usually  accompanies  this  disturbance.  These  patients  are  unable 
to  read  their  own  writing  even  though  that  writing  may  convey  sensible 
meanings  and  be  a  correct  medium  of  intercommunication. 

Cortical  World-blindness. — Spontaneous  writing,  writing  from  dicta- 
tion, or  -wTiting  from  copy  are  lost.  The  patient  is  agraphic.  He  has 
lost  all  memories  of  written  or  printed  symbols,  words,  designs,  or 
what  not.  Minor  grades  of  the  defect  cause  "paragraphia"  in  which 
the  patient  writes  with  mistakes  in  misplacing  letters,  or  syllables  or 
words.     Such  paragraphias  are  very  frequent  in  paresis,  for  instance. 

In  actual  practice  the  more  or  less  sharply  cut  forms  here  summarily 
described  are  less  often  met  with  than  the  numerous  mixed  forms. 
A  frequent  type  is  a  complete  or  global  aphasia  in  which  the  entire 
brain  speech  mechanisms  are  wiped  out.  These  are  the  forms  seen 
following  severe  hemorrhage  with  the  middle  cerebral  syndrome, 
hemiplegia,  etc.,  accompanying. 

Treatment  of  the  Aphasias. — Certain  forms  clear  up  spontaneously. 
No  matter  what  the  form,  however,  immediate  retraining  should  be 
begun,  as  soon  as  the  patient  has  recovered  from  the  shock  of  the 
original  insult.  This  retraining  should  be  persistently  followed 
according  to  si)ecial  methods  for  the  various  types  which  cannot  even 
be  outlined  here. 

Writing  Disturbances. — A  great  variety  of  disturbances  in  writing, 
quite  analogous  to  speech  disturbances,  are  known.    Thus,  writer's 


368  CRANIAL  NERVES 

cramp  is  analogous  to  stuttering  and  stammering.  It  is  probably 
psychogenic  in  origin.  In  the  psychoses,  very  characteristic  writing 
features  and  failures  are  present.  The  paretic  may  write  just  as 
as  he  speaks,  slurring,  leaving  out  syllables  or  words,  etc.  The  kata- 
tonic  may  show  stilted  writing  just  as  he  shows  a  stilted  affected  speech. 
The  haste  of  the  manic  is  seen  in  writing  as  in  speech.  In  time  a  true 
science  of  chirography  may  be  built  up  on  a  psychical  basis,  just  as  a 
true  science  of  phonetics  has  been. 

Paralysis  agitans,  multiple  sclerosis,  show    analogous   features  in 
writing  and  in  speech. 


CHAPTER  V. 

AFFECTIONS  OF  THE  PERIPHERAL  NERVES:  SENSORY 

AND  INIOTOR. 

NEURALGIAS. 

The  most  characteristic  feature  of  disordered  peripheral  sensory 
nerves  is  pain.  In  the  presence  of  pain  alone  one  speaks  of  neuralgia; 
pain  with  trophic  disorders,  tender  nerve  trunks  and  altered  muscular 
function  when  peripheral  is  usually  termed  neuritis;  radiculitis  if  in 
the  roots  or  the  plexus;  whereas  pain  associated  with  peculiar  skin 
eruptions  due  to  ganglionic  root  involvement  is  called  herpes  zoster 
or  zona.  The  conversion  pain  of  hysteria,  and  the  central  pains  of 
thalamic  lesions  are  not  now  considered. 

The  boundaries  between  these  affections  are  largely  artificial.  Thus 
a  very  mild  neuritis  presents  only  its  neuralgic  features;  and  a  zona 
may  be  so  slight  as  to  cause  no  eruption.  From  a  clinical  standpoint 
separation  of  these  processes  may  be  impossible.  It  is  not  always 
necessary.  Pathologically  speaking,  one  locates  the  lesion  of  zoster 
in  the  sensory  ganglion,  yet  tic  douloureux — or  trigeminal  neuralgia — 
is  preeminently  a  disease  of  the  sensory  ganglion,  the  Gasserian,  and 
yet  there  is  rarely  any  zoster  eruption.  One  evokes  the  etiological 
factor  of  an  acute  infection  element  in  herpes  zoster  yet  there  are 
zoster  cases  due  to  other  than  bacterial  causes.  The  differentiation 
between  a  radiculitis  and  a  neuritis  is  often  solely  a  question  of  termin- 
ology. 

Too  much  weight,  therefore,  is  not  to  be  laid  upon  the  classifications 
given.  For  practical  purposes  these  affections  are  treated  under 
three  heads,  but  their  fluctuating  separations  should  not  be  forgotten. 
It  is  misleading  to  call  neuralgia  a  functional  disorder. 

Like  many  other  conditions  in  nature,  these  affections,  when  seen 
in  an  accentuated  and  pure  form,  for  practical  pui*poses,  represent 
different  entities,  yet  the  partial  and  intermediary  forms  are  so  many 
that  the  description  of  the  clear-cut,  classic  tj'pes  does  not  do  justice 
to  the  whole  subject. 

Definition. — A  painful  affection  of  the  nerve  trunk  or  its  branches, 
characterized  by  remittent  or  intermittent  flashes  of  acute  pain,  with 
free  intervals,  not  usually  accompanied  by  trophic  disturbances  of  the 
muscles,  unless  its  severity  limits  the  activities  of  an  organ,  occasionally 
associated  with  painful  nerve  trunks  and  with  disturbances  in  the  skin 
structures. 

Neuralgias  are  l)nt  the  expression  of  many  diverse  lesions  which 
may  involve  the  body  in  general,  as  toxic  and  infectious  states,  a  nerve 
24  ( :im ) 


370  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

trunk  itself,  the  sensory  ganglia,  contiguous  structures,  or  they  may 
be  the  reflex  expression  of  a  disorder  in  a  viscus  remote  from  the 
site  of  the  pain.  They  may  be  of  purely  psychogenic  origin,  mostly 
hysterical  conversions,  occasionally  delusional  projections.  Neuralgia 
therefore  is  to  be  considered  solely  as  a  symptom,  a  syndrome,  or  a 
painful  somatic  reflex.     There  are  no  idiopathic  neuralgias. 

Etiology.^ — An  extraordinarily  wide  range  of  causative  factors  may 
determine  mild  or  severe  neuralgias  in  very  diverse  regions  of  the 
body.    The  most  frequent  causative  factors  are: 

(a)  Anemias  due  to  hemorrhages,  chlorosis,  pernicious  anemia,  kid- 
ney disease,  endocrinopathies,  malaria,  syphilis,  intestinal  parasites, 
etc. 

(b)  Toxins  of  exogenous  origin,  inorganic,  and  organic  or  purely 
endogenous  toxins:  thus  poisoning  by  lead,  mercury,  arsenic  and 
copper.  Alcohol  and  tobacco  are  frequent  causes.  Morphinism 
causes  neuralgia  as  an  abstinence  symptom.  The  toxins  of  many 
infectious  disorders  are  especiaUy  prone  to  bring  about  neuralgias. 
Tonsillitis  and  malaria  are  examples.  T^^^hoid  fever,  measles,  gonor- 
rhea, influenza,  syphilis  and  streptococcic  infections  are  frequently 
accompanied  by  neuralgias.  The  endogenous  toxemias  of  diabetes 
and  latent  nephritis  are  further  examples. 

(c)  Inflammation  of  the  sensory  ganglia,  which  may  be  either  of 
infectious  or  non-infectious  nature,  gives  rise  to  some  of  the  severest 
forms,  as  seen  in  herpes  zoster.  These  zosters  occur  from  involve- 
ment of  any  ganglion,  from  the  uppermost  to  those  farthest  caudad. 
They  are  usually  dealt  with  in  books  on  dermatology,  but  they  are 
essentially  nervous  disorders.  Ganglion  involvements  of  non-infec- 
tious types  give  rise  to  neuralgias,  such  as  tic  douloureux,  while  tumors 
of  the  sensory  ganglia  may  condition  persistent  and  obstinate  neuralgias 
in  the  aftected  sensory  nerves. 

(d)  Involvements  of  the  nerve  trunks  themselves,  either  by  mild 
neuritic  processes,  perineuritis,  pressure  from  anatomical  structures, 
pressure  from  lesions,  cuts,  bullets,  wounds,  tears,  tumors,  periostitis, 
osteitis  (often  infectious  in  tj'pe),  aneurism,  exostoses,  fractures,  or 
displacements  may  cause  severe  neuralgic  pains.  If  the  nerves 
degenerate  neuritis  results. 

(e)  Reflex  or  associated  neuralgias  are  numerous  and  puzzling.  Pul- 
monary, cardiac,  gastric,  hepatic,  renal,  ureteric,  intestinal,  vesical, 
uterine,  ovarian,  prostatic,  testicular  and  affections  of  other  viscera 
may  give  rise  to  herpetic  eruptions,  with  painful,  sensitive  skin  areas 
and  neuralgias ;  in  many  instances  the  neuralgia  is  not  accompanied  by 
herpes.  Head's^  complete  analysis  of  this  class  of  cases  is  of  para- 
mount importance.  Thus  a  persistent  sciatica  may  be  the  reflex  of  a 
prostatic  disturbance.  An  anemic  woman  may  not  suft'er  from  pain, 
but  on  menstruation  her  referred  neuralgic  pains  may  become  very 
severe  over  the  tenth  dorsal  nerve,  and  pain  and  tenderness  are  fre- 

'  Brain,  x^n,  1;  xvii,  339;  xix,  153. 


NEURALGIAS  371 

quent  over  the  areas  of  the  sixth  dorsal  (heart),  seventh  dorsal 
(stomach),  and  there  mav  be  occipital  and  midorbital  neuralgia  (Head) . 
(See  Figs.  194  and  195./ 

(/)  Somatic  disease  of  the  nervous  system.  General  paresis,  tabes 
dorsalis,  spinal  or  cerebral  disease,  thalamus  disease,  s}"philitic  men- 
ingomyelitis,  etc.,  are  often  accompanied  by  neuralgic  pains. 

(g)  Constitutional  Factors:  The  arthritic,  gouty,  anaphylactic  and 
scrofulous  may  be  said  to  be  predisposed  to  neuralgic  disturbances. 
Unknown  factors  thought  to  be  related  to  atmospheric  pressure, 
humidity,  high  electrical  tension,  etc.,  play  a  role  in  many  of  these 
cases.  Here  emotional  factors  may  release  vegetative  or  endocrino- 
pathic  imbalances.     Endocrinopathies  themselves  may  be  causative. 

(h)  Chronic  vascular  disease,  and  especially  arteriosclerosis,  is  a 
frequent  cause,  particularly  in  the  aged,  the  senile  and  the  presenile. 

(i)  Exposure  to  cold  is  an  important  factor.  It  is  not  certain  that 
all  neuralgias  caused  by  cold  are  not  really  mild  t^pes  of  neuritis  or 
perineuritis;  discussion  of  the  distinction  is  fruitless.  The  older 
writers  found  colds  a  predisposing  cause  in  from  25  to  40  per  cent,  of 
the  cases.     In  damp,  cold  countries  this  is  particularly  noticeable. 

(j)  Psychogenic  Factors:  These  play  a  large  part  in  practical 
medicine  in  determining  neui'algic  pains. 

Sjnnptoms. — Pain  is  the  main  feature  in  neuralgia.  For  the  most 
part  it  is  the  only  expression  of  the  nerve  disturbance.  The  character 
of  the  pain  varies  considerably,  but  in  general  it  may  be  described  as 
unilateral  and  paroxysmal.  It  is  characteristic  of  most  neuralgias 
that  they  are  not  primarily  localized  in  the  periphery.  The  pain 
seems  to  begin  beneath  the  surface,  and  may  then  shoot  out  to  the 
periphery.  It  may  be  described  as  biting,  boring,  tearing,  darting, 
cutting,  like  an  electrical  shock,  like  a  hot  iron,  etc.,  each  patient 
having  his  owti  pet  expression.  It  may  come  and  go  in  lightning-like 
flashes,  or  throbbing  pulsations,  persisting  for  a  shorter  or  longer 
time,  then  stopping  for  minutes,  hours,  or  days,  then  recurring.  ^Yhen 
continuous,  the  pain  varies  considerably  in  its  intensity. 

The  painful  area  usually  conforms  to  the  peripheral  distribution  of 
the  sensory  nerves.  In  the  herpetic  and  referred  neuralgias  the  root 
zone  area  is  involved. 

Certain  tender  points  seem  to  be  foci  from  which  the  pains  start. 
These  are  usually  situated  along  the  nerve  trunks,  and  pressure  upon 
them  is  often  sufficient  to  cause  an  exacerbation  of  a  mild  attack,  or 
to  provoke  an  attack  in  a  period  of  intermission.  Valleix  attached 
considerable  importance  to  these  points.  They  are  found,  according 
to  him:  (1)  at  the  point  of  emergence  of  the  nerve  trunks  from  bony 
foramina;  (2)  at  such  situations  where  a  nerve  trunk  traverses  a  muscle 
to  reach  the  skin;  (3)  at  points  where  the  nerve  fiber  breaks  up  into 
branches;  (4)  at  points  where  the  nerve  becomes  very  superficial; 
(5)  at  Trousseau's  apophyseal  points.  \'alleix's  points  are  of  diag- 
nostic importance  particularly  in  separating  the  neuritic  from  the  neu- 
ralgic types. 


372 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


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Accompanying  phenomena  are  fre- 
quent. In  some  patients  a  sense  of 
apprehension  may  precede  the  coming 
on  of  an  attack;  vague  sensations  of 
discomfort  often  antedate  the  neuralgic 
outbreak.  Ripples  of  pain,  like  pin- 
pricks, short  twinges,  etc.,  announce  the 
advent  of  a  more  serious  attack,  or 
may  be  the  sole  evidence  of  an  abortive 
one.  Such  mild  phenomena  are  ex- 
tremely frequent  in  certain  of  the  so- 
called  predisposed  or  neuralgic  individ- 
uals; some  feel  that  they  cannot  live  at 
high  altitudes;  others  fear  rain,  or  an 
east  wind ;  a  thunder  storm  causes  others 
to  have  twinges;  while,  again,  certain 
dietary  indiscretions  make  others  com- 
plain of  painful  twinges  for  days.  Just 
what  conditions  are  at  the  basis  of  these 
features  may  be  difficult  to  run  down. 
They  are  none  the  less  real. 

Skin  hypersensitiveness  is  frequent. 
It  may  precede  or  accompany  an  attack, 
and  persist  after  the  pain  has  ceased. 
Epicritic  sensibility  is  mostly  implicated. 
Light  touch,  a  pin-prick  or  slight  degrees 
of  heat  or  cold  are  magnified.  Deep 
pressure  and  extremes  of  heat  and  cold 
are  usually  palliative. 

Anesthesia  is  not  infrequent  following 
an  attack  of  pain,  and  the  exact  topo- 
graphical distribution  of  the  sensory 
modifications  on  the  skin  throw  con- 
siderable light  on  the  possible  etiology 
of  the  neuralgic  pains  (Head). 

Paresthesiae  are  very  frequent,  and 
certain  distributions  seem  to  show  them 
more  than  others.  Thus,  in  the  cuta- 
neous branches  of  the  femoral  they  are 
not  infrequent.  Here  they  take  on  the 
character  of  a  "meralgia  paresthetica." 

Motor  disturbances,  either  as  cramp- 
like contractions  or  as  paralyses,  are 
not  infrequent  in  accompanying  con- 
ditions.    The   painful    contractions    of 


Fig.  212. — Cutaneous  reflex  zones  of  hyperalgesia,  showing  their  relations  with  the 
spinal  root  segments  and  their  vegetative  nervous-system  connections.  The  dotted 
areas  are  to  be  referred  to  the  internal  s\irfaces.     (After  Dejerine.) 


NEURALGIAS 


373 


tic  douloureux  and  the  oculomotor  paresis  of  ophthalmoplegic  migraine 
are  familiar  examples  of  this. 

\  asomotor  and  secretory  symptoms  are  frequent.  The  blood- 
vessels are  frequently  contracted  in  the  early  stages  of  a  neuralgic 
attack,  with  resulting  blanching  and  cooling  of  the  skin.  Following 
this  a  period  of  warmth,  of  redness,  of  free  perspiration  may  result 
from  the  secondary  dilatation  of  the  vessels.  In  many  cases  of  trigem- 
inal neuralgia  other  secretions  may  be  modified.  Crying,  coryza  or 
salivation  are  not  infrequent,  while  in  widespread  neuralgic  attacks  an 
increase  in  the  amount  of  urine  and  of  milk  secreted  is  frequently  found. 


Temporal 


Vertical  {.D8) 


Orbital  (DS 

Nasofrontal  (C  3,U) 
Temporofrontal  (D5 

Maxillary 


Nasolabial 


cipital{DlO) 


Mandibular 


Tig. 


213. — Cutaneous  reflex  zones  of  hyperalgesia  of  the  head,  neck  and  shoulders  in 
their  relations  to  vegetative  nerve  (somatic)  disturbances.     (After  Dejerine.) 


Premature  graying  of  the  hair,  loss  of  hair,  thickening  of  the  skin, 
erythemata,  eczema,  pemphigus,  herpes,  thickening  of  the  bones  and 
occasionally,  muscle  atrophy  are  among  the  rarer  trophic  by-products. 

During  an  attack,  irregularities  of  the  pulse  are  not  unusual;  slowing 
is  the  rule.     The  pupils  are  frequently  dilated. 

The  general  physical  and  psychical  reactions  are  extremely  impor- 
tant.   Loss  of  sleep  and  anorexia  cause  the  patient  to  lose    strength 


374  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

and  flesh;  and  anxiety,  irritability  and  petulance  are  almost  inevitable. 
Mental  depression,  sufficient  to  lead  the  patient  to  make  suicidal 
attempts,  is  not  infrequent,  especially  in  severe  cases  of  trigeminal 
and  sciatica  neuralgia.     The  contracting  of  a  drug  habit  is  not  unusual. 

Course. — This  depends  naturally  upon  the  underlying  condition. 
Many  are  acute  and  transitory,  persist  three  or  four  days  and  never 
reappear.  Such  are  the  herpetic  types.  ]Many  reflex  neuralgias  run 
an  acute  recoverable  course,  but  show  a  marked  tendency  to  recur- 
rence. The  neuralgias  which  accompany  the  chronic  cachexias  of 
nephritis,  carcinoma,  brain  or  spinal-cord  disease,  usually  progress  in 
a  markedly  chronic  manner.  In  those  hereditarily  disposed  individuals 
the  tendency  to  chronicity  with  longer  and  shorter  periods  is  proverbial. 

Earlier  French  writers  attempted  to  distinguish  benign  and  severe 
forms.  Most  neuralgias  in  which  the  causative  factor  is  undiscoverable 
(the  so-called  idiopathic  or  primary  neuralgias)  run  a  benign  course, 
while  the  neuritic  t\'pes  are  less  amenable  to  treatment. 

The  subdivision  of  neuralgic  neuroses,  subacute  neuritic  neuralgia, 
and  chronic  neuritic  neuralgia  offer  a  grouping  referable  to  a  course 
which  has  only  clinical  convenience  to  warrant  it. 

In  the  first  t^'pe  one  finds  the  disorder  more  or  less  limited  to  the 
neuropath.  The  attacks  come  without  appreciable  cause,  or  follow  a 
nervous  shock.  Exposure  to  cold,  or  dietary  indiscretions  are  fre- 
quently claimed  as  causes,  but  are  not.  The  pain  comes  on  with 
great  suddenness  and  usually  goes  without  great  violence;  it  comes  and 
goes  apparently  without  rhyme  or  reason,  and  is  not  accompanied  by 
painful  nerve  trunk  nor  trophic  disturbances.  It  recovers  at  times,  to 
recur  at  intervals  of  a  year  or  years. 

In  the  subacute,  neuritic  neuralgic  t^']De,  exposure  to  cold  or  pressure, 
especially  in  arthritic  patients,  determines  an  attack.  The  attack 
develops  gradually;  the  pain,  at  first  mild  and  intermittent,  gets 
worse  and  worse  and  more  continuous.  Finally,  after  a  day  or  more, 
the  paroxysms  become  extreme,  the  intervals  being  marked  by  dull 
pain;  h\'persensitive  Valleix's  points  are  characteristic  findings. 

When  a  mixed  nerve  is  involved,  muscular  atrophy  or  other  trophic 
signs  appear,  signalizing  the  occurrence  of  a  neuritic  process.  Local 
edema  and  herpes  zoster  are  frequent  accompaniments.  This  type 
usually  commences  to  recover  in  from  two  to  three  weeks,  and  an 
ultimate  recovery  is  to  be  ex]3ected.  Recurrences  occur,  however, 
and  a  leap  to  the  third  type  of  chronic  neuritic  neuralgia  is  made. 
This  form  is  frequent  in  the  aged.  The  history  is  usually  that  of 
several  subacute  attacks  with  increasing  tendency  to  chronicity. 
Here  the  trophic  disturbances  in  muscle  and  in  skin  are  more  marked. 
The  paroxysms  run  a  remittent  course. 

Diagnosis. — Enough  has  been  said  to  emphasize  the  need  for  a 
searching  analysis  of  the  causative  factors  of  every  neuralgia.  They 
are  many,  and  presumably  the  most  widespread  diagnostic  error  is  the 
overlooking  of  an  early  tabes  dorsalis  in  young  to  middle-aged  adults. 

Children  are  not  prone  to  neuralgias — in  the  narrower  sense — and 


NEURALGIAS  375 

a  neuralgic  affection  in  childhood  calls  for  close  scrutiny.     It  is  usually 
somatic,  but  may  be  psychogenic. 

Since  unilateral  pain  of  special  localized  type,  occiu-ring  in  irregular 
attacks,  is  almost  the  sole  criterion  of  neuralgia,  it  is  very  frequent 
that  organic  disease  of  a  viscus  will  show  precisely  similar  accom- 
panying features.  In  the  majority  of  cases  the  underlying  somatic 
lesion  may  be  detected — occasionally  it  remains  difficult  to  locate. 
Xot  infrequently  the  diagnosis  of  a  persistent  neuralgia  may  be  cleared 
up  by  the  finding  of  malarial  organisms  in  the  blood,  or  more  rarely  the 
presence  of  a  marked  eosinophilia  will  call  attention  to  trichina  as  the 
cause  of  an  obstinate  neuralgia;  or  the  eggs  of  an  intestinal  parasite 
in  the  feces  (uncinaria)  may  direct  attention  to  an  anemia  which 
underlies  a  severe  neuralgia.  S^'philitic  neuralgias,  either  toxic  or 
vascular,  are  by  no  means  infrequent. 

The  diagnosis  of  myalgia  from  true  neuralgia  is  not  often  difficult, 
but  occasionally,  especially  in  the  intercostal  and  lumbar  regions, 
the  diagnosis  becomes  uncertain.  These  neuralgic-like  myalgias  are 
usually  isolated  in  their  location,  are  not,  as  a  rule,  accompanied  by 
acute  exacerbations,  nor  are  the  regions  usually  painful  on  pressure. 
Motion,  on  the  contrary,  usually  aggravates  myalgias. 

Neuritis  of  a  mild  grade  offers  an  especially  difficult  problem.  As 
already  stated,  mild  neuritis  shows  itself  as  a  neiu-algia.  The  question 
to  be  solved  concerns  the  likelihood  of  a  more  severe  degree  of  neuritis. 
In  this  case  the  usual  signs  of  neuritis  are  painful,  swollen  nerve 
trunks,  trophic  disturbances,  more  continuous  pain,  Lasegue's  phe- 
nomenon, weak,  flabby  muscle  fibers  and  electrical  changes.  Xew 
growths  pressing  upon  or  involving  the  nerve  trunks  within  or  with- 
out the  spinal  canal,  in  the  early  stages  particularly,  begin  as  pure 
neuralgic  syndromes.  Minute  analysis  of  the  sensory  phenomena  will 
usually  clear  up  the  diagnosis  early,  although  at  times  it  may  be 
impossible  in  the  earliest  stages. 

A  neuralgic  affection  may  be  one  of  the  earliest  signs  of  a  multiple 
sclerosis.  Oppenheim  has  found  a  severe  tic  douloureux  to  have  been 
the  earliest  sign  of  this  disorder.  Syringomyelia  may  begin  as  a 
localized  neuralgia.  ]Minute  hemorrhagic  lesions  of  the  spinal  cord 
of  traumatic  origin  give  rise  to  neuralgias. 

In  the  diagnosis  of  hysterical  neuralgia,  great  caution  should  be 
exercised.  Hysterical  neuralgias  partaking  of  the  nature  of  a  pseudo- 
neuralgia  are  extremely  diffuse,  and  react  very  rapidly  and  markedly 
to  suggestive  influences.  Hysterical  neuralgias  are  almost  invariably 
accompanied  by  other  conversion  signs.  (See  chapters  on  Psycho- 
neuroses.) 

Neurasthenic  pains  need  to  be  differentiated,  if  not  almost  entirely 
ruled  out,  as  a  common  diagnostic  pitfall.  The  many  mixed  forms  of 
neurasthenic,  hypochondriacal  and  hysterical  neuralgic  pains  should 
bear  their  characteristic  sidelights.  The  diagnosis  of  these  types  of 
neuralgia  should  not  be  lightly  made,  for  it  is  not  to  be  forgotten  that 
tliese  syndromes  of  themselves  may  be  the  reaction  on  the  part  of  the 


376  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

nervous  system  to  some  more  fundamental  somatic  lesion.  Thus 
patients  suffering  from  severe  so-called  neurasthenia  with  cachexia, 
and  severe  intercostal  neuralgic  pains  may  have  an  undiscovered 
carcinoma  of  the  stomach,  mediastinum,  etc. 

In  tabes  dorsalis  the  neuralgic  pains  have  a  wide  range,  are  rarely 
localized  in  a  peripheral  nerve  distribution  and  are  apt  to  be  radicular 
in  their  distribution.  Pain  on  pressure  of  the  nerve  trunk  is  usually 
absent.  The  objective  findings  in  the  pupils  and  cerebrospinal  fluid 
establishes  the  diagnosis. 

The  pains,  cramps  and  muscular  weakness  of  intermittent  claudica- 
tion sometimes  give  rise  to  severe  neuralgias.  Aortic  aneurism  gives 
rise  to  reflex  neuralgic  pains,  w^hich  are  usually  very  severe,  burning 
or  boring  in  character.  Aneurisms  in  other  regions  are  to  be  carefully 
excluded. 

In  reflex  neuralgias  the  use  of  cocain  or  other  local  anesthetic  may 
determine,  by  exclusion,  the  site  of  the  original  lesion.  An  orthoform 
suppository  pressed  well  against  the  prostate  has  been  known  to  relieve 
a  severe  sciatic  neuralgia.  Tumors  of  the  pelvis  frequently  give  rise 
to  sciatic  and  crural  neuralgias,  and  persistent  neuralgic  pains  of  the 
knee  are  often  a  reflex  from  hip-joint  disorder. 

Neuralgic  pains  are  a  frequent  indication  of  disturbed  nerve  fiber 
metabolism,  with  a  hyper-  or  a  hypothyroid  affection  behind  it. 

Periostitis  and  osteitis,  often  resulting  from  members  of  the  less 
virulent  streptococcus  groups,  M.  viridans,  etc.,  are  not  infrequent 
causes  for  neuralgic  pains  which  are  often  localized. 

For  the  precise  localization  of  the  areas  involved  consult  Figs.  27, 
28,  29,  30,  31  and  32,  where  both  the  peripheral  and  radicular  nerve 
distribution  are  figured. 

Prognosis." — This  is  conditioned  by  the  pathological  process  that  is 
responsible.  The  more  chronic  of  the  neuralgias,  which  in  years  gone 
by  tended  to  bring  about  chronic  invalidism  or  inveterate  drug  habits, 
have  ceased  to  have  such  a  sinister  import  by  means  of  a  better  under- 
standing of  the  underlying  conditions,  and  by  a  much  more  resource- 
ful therapy.  The  younger  and  stronger  the  individual,  and  the  less 
the  tendency  to  hereditary  disposition,  the  better  the  prognosis  in 
those  neuralgias  which  apparently  are  idiopathic,  as  well  as  those  due 
to  alcohol,  lead,  or  other  toxic  agent.  In  the  more  chronic  forms  which 
are  not  due  to  removable  condition  the  prognosis  is  bad.  With 
increasing  insight,  however,  into  the  many  intricate  disturbances  of 
nerve  metabolism  many  of  the  intractable  forms  may  be  conquered. 

Treatment. — The  chief  indications  are  to  quiet  the  pain  and  ascer- 
tain the  cause.  A  painstaking  study  of  the  history  and  an  exhaustive 
physical  examination  are  necessary  in  all  cases.  The  therapy  will 
therefore  vary  widely  if  the  cause  be  ascertained;  quinin  will  cure 
one  patient,  a  surgical  operation  may  be  called  for  in  another.  General 
rules,  therefore,  are  largely  illusory.  One  should  never  treat  a  neuralgia 
per  se,  as  it  is  solely  a  general  result  of  many  causes. 

Taking  up  the  general  therapeutic  indications,  the  analgesics  which 


NEURALGIAS  377 

have  proved  useful  may  be  discussed  first.  Phenacetin,  acetanilid, 
antipyrin,  aspirin,  p^'ramido^,  lactophenin  and  phenocoll  are  among 
those  that  have  been  valuable.  New  ones  are  constantly  being  added, 
and  among  them  some  are  certain  to  be  of  value.  The  salicylic  acid 
group  combinations  are  at  times  useful,  especially  in  the  milder  cases 
and  in  patients  with  arthritic  tendencies.  In  influenza  and  tonsillitis 
neuralgias  the  salicylates  are  valuable.  Combinations  of  these  with 
soporifics,  such  as  chloral,  paraldehyde,  sulphonal,  trional  or  veronal, 
are  helpful  in  procuring  sleep,  and  thus  prevent  the  reduction  of  the 
patient's  resistance. 

If  any  of  the  opium  group  be  necessary  it  is  better  to  give  such  in 
sufficient  doses.  Usually  smaller  doses  may  be  given  when  combined 
with  the  analgesics  mentioned.  Aspirin,  gr.  vij  (0.5  gram),  codeine, 
gr.  ^  (0.02  gram),  and  trional,  gr.  vij  (0..5  gram),  for  instance,  is  a  useful 
combination  to  be  taken  at  night.  Other  combinations  are  equally 
efi'ective.  For  the  chronic  neuralgic  pains  morphin  is  to  be  avoided  as 
long  as  possible.  This  does  not  apply  to  a  very  old  patient,  or  one  in 
whom  the  neuralgia  is  simply  the  expression  of  some  chronic  incurable 
disorder — carcinoma  for  example.  The  gradually  acquired  immunity, 
with  the  need  for  larger  doses,  and  the  pernicious  effects  of  a  habit 
apply-to  all  the  members  of  the  opium  group. 

All  analgesics  are  purely  provisionally  used.  They  are  meant  to 
give  the  patient  ease  while  looking  for  the  real  cause  of  the  pains. 

If  one's  inquiry  is  satisfied  by  the  relief  of  pain  the  use  of  analgesics 
alone  is  bad  therapy.  Other  drugs  are  quinin,  which  in  combination 
witli  the  salicylates  is  specially  valuable;  arsenic  which  is  serviceable 
in  the  neuralgias  due  to  anemia,  especially  in  combination  with  iron, 
Atropin  and  ^conitin  were  used  widely  before  the  days  of  the  anti- 
pyretic analgesics.  Their  definitely  poisonous  qualities  have  driven 
them  into  the  background.  The  unreliability  of  cannabis  indica  has 
done  the  same  for  this  otherwise  useful  analgesic.  The  iodides  are 
called  for  in  the  s^'philitic  neuralgias  and  are  useful  in  many  neuritic 
neuralgias. 

Counter-irritation  is  of  great  service  in  many  cases  of  severe  neu- 
ralgia, especially  after  the  acute  onset  is  over.  The  Paquelin  cautery 
is  the  best  means;  mustard  paste,  cantharides,  turpentine,  chloro- 
form, ether  and  acupuncture  all  have  their  place.  Local  freezing  may 
be  carried  out  by  ethyl  chloride,  methyl  chloride,  ether  or  other  volatile 
substances.  Menthol,  or  other  similar  derivatives  may  be  used  to 
advantage  for  mild  neuralgic  pains. 

Direct  applications  of  local  analgesics,  either  to  the  nerve  trunk  or 
within  the  spinal  canal,  are  valuable  in  many  deep-seated  neuralgias, 
especially  of  medullary  origin.  Cocain,  tropococain,  eucain  are  all 
useful  given  by  the  Coming  or  Quincke  method. 

Local  applications  of  heat  are  grateful  and  valuable.  Hot-water 
bags,  hot  sand,  electrical  pads,  etc.,  may  be  utilized.  General  or 
local  hot-water  baths  or  hot-air  baths  (baking)  are  at  times  desirable. 

General  hygienic  treatment  is  imperative.    A  generous  diet,  full 


378  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

sleep,  healthful  occupation  and  freedom  from  mental  worry  are 
essential.  Cod-liver  oil,  nitrogenous  diet,  with  iron,  arsenic,  strych- 
nin, calcium  salts,  are  indicated.  Faddy  dietaries  should  be  avoided. 
Even  in  arthritic  neuralgias  it  is  doubtful  if  meat  does  any  particular 
harm  when  not  taken  to  excess.  Alcoholic  beverages  are  to  be 
restricted. 

An  alkaline  therapy  often  helps  many  fugacious,  persistent  neuralgic 
pains.     Fruits  containing  the  citrates  seem  to  give  relief. 

Preparations  of  the  internal  secretions,  particularly  thyroid  and 
pituitary  clear  up  some  intractable  neuralgias  of  unknown  origin. 
They  may  be  given  in  doses  of  from  y ^^  to  |  grains  twice  or  thrice  daily. 

Climatic  changes  are  rarely  advisable.  Low-lying,  damp  and  humid 
atmospheric  conditions  seem  least  desirable  for  certain  patients.  The 
general  stimulus  that  comes  from  a  dryer,  higher  atmosphere,  even  if 
colder,  works  to  the  general  advantage,  even  if  not  directly  valuable 
for  the  relief  of  pain. 

Electrotherapy  when  well  managed  and  properly  selected  is  of  great 
value  in  some  neuralgias.  It  cannot  be  said  that  it  is  clearly  recognized 
just  what  forms  of  current  are  best  utilized  in  what  types  of  neu- 
ralgia, hence  most  efforts  must  follow  the  method  of  trial  and  error. 
In  general,  however,  Leduc's  modifications  of  d'Arsonval's  rapidly 
interrupted  current  offer  the  readiest  and  most  widely  applicable  form 
of  electrical  current  for  the  relief  of  neuralgic  pain.  It  is  doubtful 
if  any  other  form  of  electrical  application  is  known  at  the  present  time 
that  is  as  valuable  as  this.  It  is,  in  fact,  a  type  of  electrical  anesthesia, 
solely  palliative,  but  very  grateful.  Newer  applications  are  being 
brought  out,  and  other  forms  may  replace  the  Leduc  currents,  but 
at  present  these  seem  to  give  the  most  reliable  results. 

Faradic  currents,  as  heretofore  employed,  act  for  the  most  part 
simply  as  counter-irritants,  and  seem  to  possess  little  superiority  over 
the  actual  cautery.  Galvanism  with  mild  currents  is  useful  for  many 
topalgias. 

Psychotherapy  is  the  only  rational  treatment  for  the  psychogenic 
neuralgias.     These  make  up  at  least  25  per  cent,  of  the  neuralgias. 

Surgical  intervention  is  called  for  in  all  cases  in  which  pressure  is 
demonstrable  and  the  cause  removable.  Tumors  and  new  growths, 
involving  or  pressing  upon  nerve  structures,  if  removable  should  be 
taken  away.  Surgical  interference  may  be  of  radical  service  in  many 
of  the  reflex  neuralgias  of  obscure  origin,  probably  related  to  visceral 
ptoses.  Such  surgical  interference  is  justifiable  only  in  chronic  cases 
where  these  visceral  ptoses  have  resulted  from  long-continued  psychical 
causes.  Psychotherapy  is  preferable  in  the  early  stages.  Nerve 
stretching  needs  mention  mostly  to  be  condemned. 

SPECIAL  LOCALIZED   FORMS   OF   NEURALGIAS. 

While  any  sensory  nerve  in  the  body  may  become  painful,  there 
are  certain  regions  which  show  a  greater  tendency  to  involvement 


SPECIAL  LOCALIZED  FORMS  OF  NEURALGIAS  379 

than  others.  Bernhardt  has  collected  the  statistics  of  localized  dis- 
tribution in  some  685  cases  with  the  following  results:  sciatic,  303; 
trigeminal,  124;  brachial,  108;  intercostal,  45;  occipital,  42;  crural, 
25;  obturator,  2;  lumbo-abdominal,  12;  anterior  femoral,  11;  tarsalgia, 
4;  metatarsalgia,  4;  Achilles,  3;  and  coccygeal,  2.  In  616  cases  col- 
lected by  one  of  us  (J.)  during  four  years  (1902  to  1906)  the  distribu- 
tion was  as  follow^s:  trigeminal,  315;  sciatic,  194;  brachial,  31;  occipital, 
28;  intercostal,  19;  lumbo-abdominal,  19;  peroneal,  2;  crural,  2;  ulnar, 
1 ;  coccygeal,  1 ;  and  plantar,  1 . 

Trigeminal  Neuralgia. — Simple  neuralgias  of  the  branches  of  the 
fifth  nerve  are  among  the  commonest  of  all  the  neuralgias.  Fother- 
giU's  studies  on  A  Painful  Affection  of  the  Face,  published  in  1773,  is  a 
classic.  The  inferior  and  superior  branches  preponderate  in  frequency 
of  involvement.  IVIost  frequently  these  neuralgic  pains  are  due  to 
some  affection  of  one  of  the  branches.  Inflamed  teeth  play  a  pre- 
dominant role.  Affections  of  the  ears;  the  eyes,  iritis,  cyclitis,  iridocy- 
clitis; the  skin  of  the  face  or  head;  inflamm.ation  within  the  accessory 
sinuses  of  the  nose,  forehead,  antrum,  m.astoid,  all  of  these  may 
produce  diffuse  neuralgic  pains,  at  times  clearly  separable  from  a 
neuritic  neuralgia  of  the  fifth,  at  other  times  not. 

Cold  and  wet  are  important  agents  in  facial  neuralgia.  In  certain 
countries,  notably  England  and  the  north  of  Germany,  trigeminal 
neuralgias  from  this  cause  are  extremely  common;  they  seem  to  be 
much  less  frequent  in  the  United  States,  and  notably  so  in  southern 
countries. 

Neuralgia  of  the  superior  branch  is  seen  more  commonly  by  physi- 
cians, although  the  dental  branches  are  involved  much  more  fre- 
quently. These  patients  go  to  dentists  and  therefore  do  not  enter  into 
medical  statistics.  This  is  a  reason  why  it  is  incorrectly  stated  by 
most  writers  that  the  superior  branches  of  the  fifth  are  most  often 
involved.  For  the  most  part  the  milder  types,  of  neuralgia  are  induced 
by  irritation  of  some  of  the  terminal  filaments,  while  in  the  neuritic 
form,  tic  douloureux,  which  is  the  more  classic,  a  lesion  of  the  Gas- 
serian  ganglion  is  usually  present.  Mild  cases  of  tic  douloureux  may 
be  indistinguishable  clinically  from  other  tN'pes  of  neuralgic  pain. 

Tic  Douloureux. — Enough  has  been  said  on  neuralgia  in  general  to 
indicate  the  character  of  the  simpler  form  of  neuralgia  of  the  fifth. 
One  type,  however,  by  reason  of  its  severit}'  and  its  fairly  definite 
pathological  anatomy,  needs  more  extended  consideration.  Avicenna 
knew  tic  douloureux  and  described  it  with  great  accuracy.  It  would 
be  desirable  to  restrict  the  term  tic  douloureux  to  a  definite  and,  if 
possible,  limited  tj^je  of  neuritis  of  the  fifth  nerve,  particularly  to  the 
form  due  to  changes  of  a  chronic  degenerative  nature  occiu-ring  in 
the  Gasserian  ganglion.  This  is  not  yet  possible,  and  clinically  the 
neuritic,  and  peripheral  neuralgic  cases  are  either  not  at  all  separable 
from  the  ganglion  cases,  or  with  considerable  difficulty. 

Tic  douloureux  usually  aft'ects  one  side  of  the  face.  In  the  majority 
of  cases  some  selection  occurs  among  the  branches,  one  or  two  being 


380  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

involved,  rarely  all  three;  the  ophthalmic  branch  the  oftenest,  the 
inferior  maxillary  the  least  often  implicated. 

The  more  classical  tix  douloureux  neuralgias  are  characterized  by 
the  extreme  severity  of  the  pain,  usually  preceded  by  paresthetic 
phenomena,  and  frequently  accompanied  by  sympathetic  or  irradiating 
pains  in  other  branches  than  the  one  chiefly  involved,  or  in  other 
nerves.  The  pain  may  be  paroxysmal  or  continuous,  with  marked 
exacerbations.  Patients  compare  them  to  the  piercing  pains  of  a  sharp 
knife  or  the  burning  of  a  red-hot  wire.  The  patient  remains  for  a 
shorter  or  longer  period,  a  few  minutes  to  several  hours,  under  the 
grip  of  the  pain,  unable  to  move  a  muscle  of  the  face  or  fearful  of 
stirring,  lest  a  spasm  more  fearful  than  the  others  should  occur;  even 
the  air-pressure  of  a  suddenly  closed  door  may  bring  on  an  exacerba- 
tion.   The  longer  attacks  are  rarely  as  vicious  as  the  shorter  ones. 

HjTDersensitive  Valleix's  points  are  relatively  constant.  In  ophthal- 
mic involvement  the  sore  points  are  found  above  the  supraorbital 
notch,  at  the  external  angle  of  the  upper  lid,  the  upper,  outer  aspect 
of  the  nose  and  the  globe  of  the  eye ;  in  the  superior  maxillary  branch 
the  inferior  orbital  notch  is  the  chief  point  of  pain;  the  malar  bone, 
and  opposite  the  last  upper  molar  are  other  less  frequently  found 
points,  while  the  outer  angle  of  the  mouth,  and  the  roof  of  the  mouth 
are  rarely  their  site.  In  the  inferior  maxillary  distribution  the  points 
are  chiefly  just  in  front  of  the  auditory  canal,  the  side  of  the  tongue, 
the  border  of  the  chin,  and  Trousseau's  points  over  the  first  and  second 
cervical  vertebrae. 

Vasomotor  and  secretory  disturbances  are  usual.  The  skin  is,  as  a 
rule,  hot  and  swollen,  occasionally  pale  and  frigid;  tears,  nasal  secre- 
tions, and  saliva  flow  in  abundance.  The  eyelids  may  be  swollea, 
the  conjunctiva  reddened  to  the  point  of  ulceration  at  times;  within 
the  nose  and  mouth  extravasations  occur,  and  ulcers  are  not  uncom- 
mon. Herpetic  attacks  are  also  not  infrequent,  and  in  some  of  the 
attacks  grave  injury  to  the  eye  structures  may  take  place.  Glaucoma 
is  one  of  the  severe  complications.  Other  trophic  disturbances  are 
skin  eruptions,  acne,  erysipelatous  reddening,  graying  of  hair,  and 
blackening  of  the  tongue.  In  long-continued  cases  hemiatrophy  may 
occur.  Changes  in  the  sense  of  taste,  of  touch,  of  hearing,  are  at  times 
present.  Photophobia  is  frequent,  while  diminution  in  the  visual 
fields  and  accommodation  cramps  have  been  noted. 

Severe  mental  disturbance,  amountmg  at  times  to  hallucinatory 
confusion  may  be  present.  Suicidal  attempts  are  to  be  guarded  against 
in  these  excruciating  cases. 

The  motor  disturbances  consist  of  convulsive  movements  of  the 
facial  muscles  (convulsive  tics,  spasmodic  epileptiform  neuralgia. 
Trousseau),  sudden  forced  closing  of  the  eyelids,  drawing  of  the  mouth 
to  one  side,  or  sudden  turning  of  the  head.  At  times  the  convulsive 
movements  extend  to  the  arms.  Paralytic  phenomena  in  the  third 
nerve  have  been  noted.  The  general  psychical  disturbances  noted  are 
prone  to  occur  in  this  type. 


TIC  DOULOUREUX  381 

Course. — In  the  majority  of  cases  the  attacks  appear  in  series  and 
attain  a  periodicity  which  comes  to  be  dreaded  by  the  sufferer.  The 
free  intervals  usually  become  shorter  and  shorter;  but  many  patients 
may  have  only  one  attack  a  year,  especially  in  cold  weather,  or  even 
at  longer  intervals.  A  single  attack  may  last  a  few  days,  or  in  the 
severe  forms  several  weeks,  the  patient  not  being  free  from  pain  day 
or  night,  save  under  the  influence  of  morphin.  Some  patients  have  a 
few  attacks  in  a  lifetime,  others  are  not  free  from  the  disease  for  years. 
The  severer  convulsive  forms  are  prone  to  occur  late  in  life. 

Diagnosw: — Ordinarily  the  classical  form  of  tic  douloureux  is  recog- 
nized without  difficulty.  Patients  have  all  their  teeth  extracted, 
however,  under  the  mistaken  diagnosis  of  a  dental  disease,  while 
some  intractable  trigeminal  neuralgias  have  been  cured  by  proper 
attention  to  diseased  teeth.  Aneurism  of  the  carotid,  tumors  pressing 
upon  the  nerve  or  upon  the  Gasserian  ganglion,  may  be  difficult  to 
determine  as  the  exciting  cause.  These,  however,  are  usually  accom- 
panied by  accessory  symptoms,  palsies,  eye-ground  changes,  aneuris- 
mal  murmurs,  pain  within  the  head,  cerebellar  syndromes,  ear  pains, 
etc.  The  otalgias  (tympanic  neuralgias)  usually  considered  in  this 
connection  are  probably  due  to  cephalic  ganglion  disorder,  although 
frequently  referred  to  by  some  authors  as  neuralgias  of  the  seventh 
nerve. ^ 

Multiple  sclerosis  has  started  as  a  trigeminal  neuralgia. 

Treatment. — It  is  as  essential  to  endeavor  to  find  and  treat  the 
cause  for  a  facial  neuralgia  as  for  neuralgia  in  general.  The  various 
remedies  given  under  the  heading  of  neuralgia  may  be  tried,  and  as 
malarial  neuralgias  are  very  frequently  trigeminal,  energetic  quinin 
therapy  may  be  given;  the  absence  of  blood  findings  is  not  contra- 
indicative,  especially  in  malarial  countries.  Gelsemimn,  the  tincture 
in  10-minim  doses,  gradually  ascending,  aconite  in  doses  of  -g-g-^ 
grain,  cannabis  indica  (fresh),  in  doses  of  from  |  to  ^  grain,  are 
reputed  as  especially  valuable  in  the  facial  cases.  Any  of  the  analgesic 
antipjTetics  may  suit  individual  cases,  and  avoid  the  use  of  morphin, 
which  alone  is  reliable  in  many  severe  cases.  Local  applications  of 
cocain  to  the  conjunctiva,  nasal  mucous  membranes,  buccal  surfaces 
are  sufficient  to  repress  some  mild  attacks. 

Injection  methods  have  been  tried  for  years.  In  the  beginning  the 
peripheral  branches  were  injected  by  various  analgesic  drugs,  in  early 
days  chloroform,  and  in  later  times  particularly  cocain  and  its  allies 
or  derivatives.  The  effects  were  valuable,  but  temporary.  Osmic 
acid  was  used  later,  but  regeneration  took  place.  Pitres  and  Vaillard, 
in  1887,  and  Schlosser,^  in  1900,  took  up  a  series  of  experiments  with 
alcohol,  and  the  latter  has  perfected  a  method  of  injecting  alcohol 
within  the  substance  of  the  (jasserian  ganglion,  which  has  given  excel- 
lent results.  The  chief  features  in  the  deep  alcohol  injection  method 
is  the  introduction  of  a  long,  dull,  cutting  needle  into  the  foramen 

'  Kidd:    Rev.  of  Neur.  and  Psych.,  1913.     Mills:    .Jour.  Xerv.  and  Ment.  Dis.,  1911. 
2  Munchen.  nied.  Wchuschr.,  April  .30,  1897. 


382  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

ovale  and  there  injecting  in  situ  the  branches  of  the  trigeminus. 
Special  methods  have  been  devised.  Narcosis  is  not  necessary;  80 
per  cent,  alcohol  is  used.  In  three  or  four  hours  following  the  injec- 
tion the  pain  is  relieved,  and  two  or  three  more  injections  are  given 
within  a  week  to  complete  the  treatment.  Immediately  following 
the  injections,  which  should  be  done  only  after  extended  practice  on 
the  cadaver,  there  is  a  marked  anesthesia  on  one  side  of  the  anterior 
part  of  the  head,  including  the  nostril,  palate,  and  one-half  of  the 
tongue;  a  slight  paralysis  of  the  muscles  of  mastication,  which  may 
persist  for  some  time,  but  usually  disappears  in  a  few  hours;  a  degen- 
erative process  is  set  up  in  the  nerve  trunk,  which  is  recoverable, 
and  general  sensibility  usually  returns,  but  the  pain  returns.  Relief 
extending  over  a  year  in  a  number  of  cases  is  reported  by  numerous 
observers.  Some  patients  have  been  relieved  for  four  or  five  years. 
Edema  of  the  posterior  eye  structures  and  hemorrhage  are  among  the 
discomforts  and  even  dangers  of  the  operation,  especially  in  the  use 
of  the  intra-orbital  methods  devised  by  French  operators.  Relapses 
are  apt  to  occur. 

Three  surgical  procedures  have  been  seriously  advocated.  The 
first  and  earliest  consisted  of  peripheral  section,  first  said  to  have 
been  done  by  J.  C.  Warren  of  Boston.  Section  of  the  fifth  may  be 
employed  to  advantage  in  those  cases  in  which  the  disease  is  undoubt- 
edly peripheral.  As  modified  by  more  recent  procedures,  the  older 
objection  that  regeneration  takes  place  is  partly  done  away  with. 

Rose,  MacEwen,  Horsley,  Hartley  and  Krause  perfected  the  opera- 
tion of  excision  of  the  ganglion,  and  the  modified  Hartley-Krause 
operation  by  the  temporal  route  has  been  largely  the  method  of 
choice.  C\ishing's  more  recent  modifications  are  of  lasting  value. 
The  operation  still  remains  one  of  much  difficulty  and  seriousness. 
Recurrences  are  known  even  with  this  method,  and  the  efficiency  of 
the  newer  devices  for  preventing  this  by  capping  the  ends  of  the 
divided  nerve  trunks  with  metallic  laminae  is  still  debatable. 

Van  Gehuchten,  in  1903,  suggested  tearing.  Spiller,  in  1898,  had 
suggested  the  surgical  expedient  of  cutting  the  sensory  root,  which 
he  claims  is  safer  than  the  operation  of  tearing,  a  procedure  tried  in 
1881.  The  method  of  division  of  the  sensory  root,  as  reported  by 
Frazier  and  Spiller,  promises  to  be  one  of  the  most  valuable  surgical 
procedures  thus  far  devised 

Psychogenic  cases,  which  are  numerous,  are  curable  by  psychoanalysis. 

Cervico-occipital  Neuralgia. — This  occurs  in  the  distribution  of  the 
sensory  nerves  of  the  cervical  plexus,  consisting  chiefly  of  the  occip- 
italis major,  the  occipitalis  minor,  auricularis  magnus,  cervicalis 
superior,  supraclavicularis  and  phrenic.  Neuralgia  in  this  general 
region  seems  to  be  rare.  In  Remak's  summary  of  15,000  cases  only 
50  were  in  the  cervico-occipital  regions.  Valleix  has  given  one  of  the 
most  complete  monographs  on  neuralgia  in  this  area  and  little  has 
been  added  to  his  description,  save  in  the  finding  of  rare  etiological 
factors. 


SPECIAL  LOCALIZED  FORMS  OF  NEURALGIAS  383 

Etiology. — The  several  causes  of  neuralgia  are  operative  here  and 
need  not  be  repeated.  Special  determining  features  seem  to  be  the 
carrying  of  heavy  weights  on  the  shoulders  (a  more  frequent  cause 
for  brachial  neuralgias),  arthritis  deformans  of  the  upper  cervical 
vertebrae,  caries,  s\'philis,  tuberculosis,  tumors,  cervical  pach;\Tnenin- 
gitis,  falls  and  blows  WTcnching  the  cervical  vertebrae,  enlargement 
of  the  cervical  lymphatics  and  aneurisms  of  the  vertebral  artery. 
Oppenheim  refers  to  the  great  frequency  of  hysterical  neuralgia  in 
this  region  and  psychogenic  neuralgias  of  the  back  of  the  neck  and 
occiput  are  extremely  common.  They  are  often  found  in  individuals 
who  are  either  under  great  strain  or  those  who  are  constantly  forcing 
themselves. 

The  pains  occupy  the  regions  mentioned,  being  particularly  local- 
ized in  the  neck,  below  the  occiput,  and  running  up  to  the  vertex, 
occasionally  behind  the  ears.  The  Valleix  point  found  most  fre- 
quently is  the  occipital  point  between  the  mastoid  apophysis  and  the 
first  cervical  vertebra;  points  between  the  sternomastoid  and  trapezius 
(cervical),  the  anterior  border  of  the  mastoid  and  the  middle  of  the 
ear  are  of  less  frequent  occurrence. 

The  pain  is  frequently  bilateral.  Dull  pain  on  pressure,  with  tender 
skin,  is  usual  as  a  paroxysmal  occurrence.  This  tends  to  make  the 
sufferer  hold  his  head  in  a  stiff  position,  which  in  time  may  cause  a 
characteristic  attitude.  This  tenderness  may  be  so  acute  that  ruffling 
of  the  hair  will  start  a  paroxysm.  Graying  of  the  hair,  loss  of  hair, 
with  other  trophic  signs  may  be  present.  Sudden  pulling  back  of  the 
head,  or  other  muscular  involvement,  is  an  occasional  SATnptom. 

Diaphragmatic  Neuralgia. — This  form  of  neuralgia,  also  known  as 
phrenic  neuralgia,  is  of  rare  occurrence.  Falot  and  Peter  have  written 
upon  it.  The  pain  is  usually  present  near  the  free  border  of  the  ribs, 
occasionally  as  high  as  the  chin  and  in  the  neck,  beneath  the  clavicle, 
and  in  the  scalenus  anticus  muscle.  Trousseau's  points  are  located 
over  the  second  to  the  fifth  cervical  vertebra.  The  pain  frequently 
runs  down  the  arm,  especially  in  certain  complex  cases  of  mixed 
brachial  neuralgia. 

Breathing  may  be  seriously  interfered  with,  the  breath  coming  fast 
and  short;  longer  excursions  of  the  diaphragm  are  impossible.  It 
is  a  common  experience  to  have  a  short,  sharp  stitch  in  the  side,  with 
inability  to  breathe  for  fear  of  pain.  This  is  the  t\i3e  of  distress 
encountered  in  phrenic  neuralgia.  In  the  majority  of  cases  the  pain 
is  in  the  left  side. 

Anemia,  afl'ections  of  the  mediastinum,  heart  and  pericardium  and 
aneurism  of  the  aorta  are  the  most  frequent  attending  features.  An 
intractable  phrenic  neuralgia  may  complicate  an  exophthalmic  goiter, 
or  be  present  in  carcinoma  of  the  neck  region. 

Idiopathic  or  pure  })hrenic  neuralgias  seem  to  be  unusual,  whereas 
temporary  or  more  ])ormanent  types  are  seen  as  symjjtoms  of  tJie 
affections  named.  In  the  latter  case  the  prognosis  depends  on  the 
initial  difficultv. 


384 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


Brachial  Neuralgia. — In  this  general  form  the  components  of  the 
brachial  plexus,  from  the  four  lower  cervical,  or  some  of  its  filaments, 
and  first  dorsal  roots,  are  those  involved.  The  chief  nerves  carrying 
sensations  from  the  skin  area  of  the  arms  and  shoulders  are  the  cir- 
cumflex, radial,  internal  cutaneous  and  musculocutaneous.  These 
enter,  for  the  most  part,  the  upper  and  middle  cords  of  the  plexus. 
In  the  majority  of  cases  the  pains  of  brachial  neuralgia  are  located  in 
the  upper  arm  and  about  the  shoulder,  i.  e.,  in  the  area  of  the  circumflex, 
radial,  musculocutaneous  and  internal  cutaneous  nerves. 


TO  SCALENI  & 
UONGUS  COLLI 


THIRD 

POSTERIOR 
THORACIC 


-FROM  FOURTH  CERVICA^ 


SUPRASCAPULAR 

EXTERNAL  ANTERIOR  THORACIC 

INT  ERNAL  ANTERIOR  THORACIC 


CIRCUMFLEX 


' MUSCULO  SPIRAL 


^''AL  curA^ro7,-?------A::     ^v>..  ^*       °<^s 


EOcis 


Fig.  214. — Plan  of  the  brachial  plexus.     (Gerrish.) 


Bernhardt's  statistics  show  that  men  are  more  frequently  affected 
than  women,  but  the  reverse  shows  true  in  the  figures  of  other  observers 
(Romberg,  Erb).  More  women  have  brachial  neuralgia  than  men 
and  in  most  instances  it  seems  that  excessive  sweeping  is  the  attributed 
cause.  In  piano-players,  neuralgias  in  this  area  are  frequent.  Perhaps 
these  should  be  relegated  to  the  occupation  neuroses  with  the  pains 
of  hair-dressing,  skirt-carrying,  telegraphy,  writing,  etc.  At  any  rate, 
arm  and  shoulder  pains  are  frequent,  in  their  mild  grades  at  least, 
and  very  variable.     (See  Fig.  221  and  Figs.  23,  24,  25,  26,  30  and  31.) 

The  usual  causative  factors  come  into  play  here.  The  neuropathic 
constitution  is  put  in  the  foreground  by  Oppenheim;  Bernhardt  lays 
considerable  stress  upon  the  importance  of  bone  injuries  with  callus 
formation  in  the  causation  of  many  arm  neuralgias.  Small  punctured 
wounds  about  the  forearm,  wrist  and  arm  are  responsible  for  many 


BRACHIAL  NEURALGIA 


385 


symptomatic  neuralgias,  as  Weir  ^Mitchell  has  so  well  shown.  More 
remote  causes  are  found  in  vertebral  disease,  tumor  formation,  aneu- 
risms, syringomyelia,  multiple  sclerosis  and  tabes.  The  frank  neuritic 
processes  in  their  beginnings  must  be  borne  in  mind,  and  cervical  rib 
should  not  be  overlooked. 

Symptoms.— Cervicobrachial  neuralgias  are  extremely  variable  in 
distribution,  extent  and  severity.  The  onset  is  usually  sudden,  espe- 
cially in  those  patients  in  whom  an  antecedent  history  of  exposure  to 
cold'  and  to  wet  is  obtainable  (motormen,  policemen,  etc.) ;  at  times 
the  beginning  is  preceded  by  twinges  and  slight  distress.    On  awaken- 


FiG.  215. — Painful  points  in  brachial  neuralgia- 

ing  in  the  morning  sharp  pain  is  felt  in  the  shoulder  and  arm.  The 
pains  in  brachial  neuralgia  are  less  apt  to  be  the  sharp,  shooting  variety 
so  dreaded  in  tic  douloureux,  but  sudden  accession  of  sharp  pains, 
varying  in  their  intensity,  are  frequent .  As  with  most  neuralgic  pains, 
movement  increases  them.  Toward  evening  the  i^ains  are  apt  to 
increase  and  the  patient,  although  obtaining  relief  b\'  lying  down 
rarely  sleeps  well.  Soreness  of  the  skin,  slight  swelling  and  general 
reduction  in  tone  are  the  usual  accompaniments.  With  increasing 
disuse  slight  atrophy  is  common,  and  swelling  is  usual.  The  tendon 
reflexes  are  frequently  more  irritable  and  active.  More  atrophy,  i)aresis 
25 


386  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

with  vasomotor-trophic  symptoms  and  altered  tendon  reflexes  indi- 
cate a  definite  neuritic  process.  Herpetic  eruptions  occur  with  non- 
infectious as  well  as  with  infectious  involvements  of  the  sensory  ganglia. 

Tender  points  are  very  variable.  They  are  most  frequently  in  the 
middle  of  the  back;  about  the  level  of  the  second  or  third  dorsal  there 
is  usually  a  sore  Trousseau  point.  Gowers  notes  that  the  inferior 
ulnar  point  in  front  of  the  wrist  is  the  commonest  sore  point.  Babinski 
has  called  particular  attention  to  a  radial  neuralgia  due  to  a  mild  or 
severe  neuritis  of  the  radial.  The  pains  occupy  the  posterior  portion 
of  the  arm  and  are  unusually  severe.  Neuritic  changes  are  not  infre- 
quent. The  chief  causes  seem  to  be  exposure  to  cold  and  disturbances 
of  the  reciprocal  action  of  the  ovaries  or  testicles  and  thyroids  and 
other  endocrinous  glands.  The  menopause  is  a  frequent  period  of 
onset. 

Diagnosis. — In  the  diagnosis  particular  care  is  needed  in  excluding 
affections  of  the  spinal  cord,  meninges,  and  vertebrae,  as  well  as  angina 
pectoris  and  pseudo-angina.  Disease  of  the  joints  and  bones  should 
be  excluded  at  the  outset,  although  it  may  be  very  difficult  in  some 
cases  of  periostitis.  In  tumors  and  other  somatic  affections  of  the 
cord  the  painful  points  are  usually  absent,  but  the  earliest  and  only 
symptom  of  spinal-cord  tumor,  intramedullary  or  extramedullary, 
may  be  a  brachial  neuralgia.  In  tabes  the  pains  are  apt  to  be  bilateral. 
The  exhaustion  neuralgias  are  also  apt  to  be  bilateral.  The  general 
indefinite  features  of  a  myalgia,  plus  the  muscular,  rather  than  the 
nerve  soreness,  are  usually  sufficient  to  exclude  it. 

The  occupation  neuroses  involving  the  arm  and  shoulder  are  many. 
The  history  of  protracted  exercise  of  certain  groups  of  muscles  is 
usually  sufficient  to  identify  the  proper  cause  for  the  neuralgic  pains. 
Occupation  neuralgias,  like  neuritic  neuralgias,  are  neuralgias  none  the 
less,  the  sole  diagnostic  question  arising  as  to  the  cause,  and  through 
this  the  proper  mode  of  therapeutic  attack  and  the  probable  outcome. 
Alcoholic  neuritis  in  its  mild  grade  offers  particular  embarrassments. 
Lead-poisoning  neuralgias  are  to  be  borne  especially  in  mind,  while 
diabetes  is  of  prime  importance.  Brachial  psychalgia  is  a  possibility, 
but  the  diagnosis  must  be  made  with  extreme  cautioi  after  a  rigid 
exclusion  particularly  of  organic  factors.  Psychogenic  and  neurasthenic 
neuralgias  occur  in  this  distribution  as  well. 

Treatment. — ^Rest  is  a  necessity,  and  is  primarily  insured  by  means 
of  a  sling.  The  diagnosis  of  a  cause  being  assured,  treatment  should 
be  begun  to  remove  it,  either  by  medical  or  surgical  means.  Nerve 
sutures  for  injury  has  come  to  occupy  an  important  place,  and  is 
usually  attended  with  good  results,  even  after  long  periods  of  loss  of 
function.  In  the  early  stages  active  mechanical  treatment  is  to  be 
avoided.  Hot  applications  are  useful  in  most  acute  neuralgias.  In  the 
later  phases  massage,  particularly  the  Nageli  movements,  are  valuable. 
Galvanism,  3  to  6  milliamperes,  is  well  adapted  to  these  neuralgias, 
but  usually  much  better  results  are  obtained  by  the  Leduc  rapidly 
alternating  currents.     Salicylates   (especially  in  analgesic  combina- 


INTERCOSTAL  NEURALGIA  387 

tions),  iodides,  qiiinin,  arsenic  and  large  doses  of  strychnin  are  of 
value  at  times.  The  internal  secretions  are  curative  for  some. 
Psychoanalysis  is  to  be  used  in  psychogenic  cases. 

Intercostal  Neuralgia. — The  twelve  dorsal  nerves  constitute  the 
plexus  involved,  although  the  upper  series,  especially  of  the  left  side, 
are  most  frequently  concerned.  Bernhardt  says  that  the  site  of 
election  is  mostly  from  the  fifth  to  the  ninth.  Since  the  dorsal  nerves 
divide  into  internal  and  external  branches  the  site  of  the  neuralgia 
may  be  on  the  surface  or  within  (pleurodynia,  etc.).  The  two  upper 
nerves  send  branches  to  the  internal  surface  of  the  arm,  and  pain  is 
occasionally  felt  there.  The  abdominal  involvements  are  rarer  and 
may  extend  down  to  the  genitals.     (See  Figs.  22  to  30.) 

Women  more  often  show  this  form  of  neuralgia  than  men,  and  the 
disorder  is  much  more  common  in  cold  weather. 

The  pains  are  usually  less  severe  than  in  other  regions,  although 
their  sharp,  sticking  character  impairs  chest  movements,  especially 
since  all  movement  tends  to  aggravate  them.  Tender  points  are  found 
at  the  site  of  the  nerve  exits  near  the  spine.  Skin  h^•peresthesia  is 
extreme  at  times.  Herpetic  neuritic  neuralgias  are  relatively  common 
in  this  distribution. 

Among  the  causes  to  be  diagnosed  may  be  costal  caries,  affections 
of  the  spinal  cord  and  meninges,  disorders  of  the  pleura,  particularly 
carcinoma  and  tuberculosis,  aortic  aneurism,  dilatation  of  the  stomach, 
carcinoma  of  the  liver,  angina  pectoris,  pericarditis,  local  trauma, 
fractures,  etc. 

Mammary  neuralgia  or  mastodynia,  which  is  frequent  in  the  later 
stages  of  nursing,  and  in  some  women  at  the  menstrual  epoch,  is  a 
special  form.  The  pain  is  usually  deep  within  the  gland,  and  may  be 
accompanied  by  a  slightly  increased  secretion.  The  whole  skin  may  be 
sensitive,  especially  the  nipple,  when  the  superficial  nerves  are  mostly 
involved.  Local  glandular  induration  occasionally  occurs.  This  has 
led  to  the  mistaken  diagnosis  of  carcinoma,  but  a  neuralgia  may  occur 
due  to  a  carcinoma  of  the  breast.  Tabes  may  give  rise  to  an  intercostal 
neuralgia. 

Treatment.^ — Local  applications  are  useful,  especially  the  eth>l 
chloride  spray.  Blisters  are  efficacious.  Support  by  bandaging 
affords  marked  relief.  General  measures  already  described  call  for 
no  further  mention. 

Lumbar  Plexus  Neuralgias. — These  are  most  conveniently  arranged 
as  (1)  lunibo-abdominal;  (2)  ilioscrotal  or  testicular,  (3)  crural  (sciat- 
ica), (4)  femoral,  and  (o)  obturator,  involving  in  each  case  certain 
of  the  branches  of  this  plexus.  Mixed  and  indeterminate  forms  are  not 
infrequent. 

The  Sciaticas  (Sciatic  Neuralgias,  Sciatic  Xeuritides). — It  has 
already  been  indicated  that  it  is  largely  indifferent  whether  one 
regards  this  as  a  neuralgia  or  a  neuritis,  since  transition  forms  are 
very  frequent.  It  consists  of  pain  in  the  distribution  of  the  nerves  of 
the  sacral  plexus,  the  sciatic  and  its  branches. 


388 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


History. — Cotugni,  in  1764,  gave  so  clear  a  description  of  sciatica 
that  the  malady  is  often  given  his  name.  Valleix,  in  1841,  described 
the  painful  points  with  great  minuteness.  Lasegue,  in  1864,^  described 
his  well-known  symptoms  of  neuritis  of  the  sciatic,  since  which  time 
many  monographs  have  appeared,  the  most  important  of  which  are 
those  of  Briihl,  Lago,  Vulpian  and  Bernhardt. 

Etiology. — Similar  causes  are  at  work  here  as  in  the  other  neuralgias 
and  neuritides.  It  is  unnecessary  to  amplify  these  causative  factors. 
Any  of  the  general  causes  found  on  previous  pages  may  produce 
a  sciatica,  but  special  emphasis  should  be  laid  upon  two  or  three. 
Trauma  is  responsible  in  many  cases  for  the  development  of  sciatica. 
Syphilitic  osteo-arthritis  and  syphilitic  meningitis  of  mild  grade  are 


ILIOHYPOGASTRIC 
ILIO-INGUINAL 


GENITOFEMORAL 


EXTERNAL 
CUTANEOUS 


TO    PSOAS    AND 
ILIACUS 


FEMORAL  (anterior 

crural) 


Fig.  216. — Diagram  of  the  lumbar  plexus. 

responsible  for  the  development  of  intractable  sciaticas.  Certain 
French  authors  claim  as  high  as  90  per  cent,  of  all  sciaticas  to  be  due 
to  this  syphilitic  factor.  In  this  respect  then  sciatica  stands  in  sharp 
contrast  to  the  neuralgic  neuritides  of  the  upper  extremities. 

Gout  is  an  infrequent  causative  factor,  while  diabetes  is  more 
common,  especially  for  double-sided  sciaticas.  Double  sciaticas  may 
also  be  the  expression  of  a  tumor  of  the  pelvis,  of  pressure  due  to  a 
gravid  uterus,  to  venous  stasis,  spinal-cord  tumor,  or  new  growths 
of  the  Cauda.  Occasionally  prostatic  enlargement  of  tuberculous 
or  gonorrheal  origin  gives  rise  to  sciatic  pains.  Exposure  to  cold, 
with  prolonged  standing  is  frequently  met  with  in  the  histories,  and 
occasionally  in  those  who  sit  a  great  deal.     Prolonged  walking  or 


1  Arch.  gen.  de  med.,  1864.     Oppenheim. 


SCIATIC  NEURALGIAS 


389 


marching  may  occasion  an   attack,   and   bicycling  predisposes  to 
mechanical  injury  of  the  nerve. 

It  is  a  comparatively  common  affection,  particularly  in  men,  being 
one  of  the  most  frequent  neuralgias  met  with  in  dispensary  practice. 
Chronic  constipation  as  a  cause  should  not  be  overlooked.  The 
etiological  factor  in  some  cases  is  impossible  to  find.  This  is  a  result 
of  insufficient  methods  of  examination. 

.'''      FOURTH  LUMBAR 


FTH  LUMBAR 


RST  SACRAL 


SECOND  SACRAL 


THIRD  SACRAL 


FOURTH  SACRAL 


FIFTH  SACRAL 

N.TO  COCCYGEUS 


COCCYGEAL 


Fig.  217. — Plan  of  sacral  plexus  with  the  pudendal  plexus.     (Gerrish.) 


INFER 
GLUT 


Symptoms. — There  is  no  one  sciatica,  there  are  many,  and  it  is 
advisable  at  the  outset  to  separate  those  cases  in  which  the  principal 
sjTnptoms  are  pain  and  inability  to  use  the  limb  from  those  in  which 
there  is  added  nerve  tenderness,  with  motor,  sensory  and  trophic 
phenomena. 

The  pains  rarely  commence  abruptly,  but  beginning  more  or  less 
gradually  from  a  sense  of  soreness  to  uneasiness  with  occasional 
twinges,  gradually  develop  into  well-marked  severe  pains,  usually  at 
first  more  intense  just  beneath  the  sciatic  notch,  gradually  extending 
from  above  downward  to  the  entire  distribution  of  the  sciatic  and 
some  of  the  branches.  There  is  usually  considerable  variation  in  the 
character  of  the  pain  during  an  attack.  Some  patients  suffer  for  some 
time  simply  from  boring,  dull  pains,  while  others  have  excruciatingly 
sharp,  stabbing  twinges  that  make  the  slightest  movements  impossible. 


390  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Rarely  continuous,  the  pains  come  in  attacks,  sparing  almost  no  region 
of  the  distribution  of  the  plexus  either  en  masse  or  picking  out  special 
branches. 

The  pain  in  the  proximal  portions  of  the  leg  is  usually  deep-seated, 
but  becomes  more  superficial  distally.  In  some  patients  an  extensive 
series  of  involuntary  adaptive  positions  take  place  in  order  to  seek  the 
most  comfortable  position,  not  only  of  the  thigh  and  leg,  but  of  the 
pelvis,  or  even  the  vertebral  column. 

Painful  pressure  points  are  fairly  constant.  The  most  important 
of  these  are  situated  at  the  sacro-iliac  joint,  the  sciatic  notch,  or  the 
gluteal  point  on  the  gluteal  fold  over  the  nerve,  and  the  peroneal 
point  at  the  head  of  the  peroneus.  In  some  cases,  often  mild,  pressure 
points  are  lacking.  Lasegue's  phenomenon  is  of  considerable  diagnostic 
significance.  It  is  brought  out  either  in  the  lying  or  sitting  position. 
The  patient's  foot  is  grasped  with  one  hand,  the  other  placed  upon  the 
knee,  and  keeping  the  leg  stiffly  extended  the  thigh  is  flexed  upon  the 
pelvis,  when  a  severe  pain  develops  beneath  the  knee  or  higher  up  in 
the  course  of  the  nerve.  In  the  sitting  position  the  pain  is  more  apt  to 
be  beneath  the  knee,  since  the  extension  on  the  pelvis  cannot  be  made 
so  extreme. 

With  the  general  extension  of  the  neuritic  process — as  in  many 
alcoholic  cases — the  entire  nerve  may  be  sensitive  to  pressure. 

Minor^  has  described  some  interesting  findings  on  having  the 
patients  arise  from  a  prone  posture.  Patients  with  well-marked 
sciatica  can  rarely  get  up — without  excessive  pain — with  the  arms 
crossed.  They  put  their  two  hands  behind  them,  push  the  hips  back- 
ward between  the  arms,  flex  the  knees  slowly  beneath  the  buttocks, 
then  gradually  with  one  hand  on  the  hip,  and  then  on  the  knee,  the 
other  balancing  in  the  air,  gradually  come  to  a  standing  position.  The 
procedure  is  not  invariable,  but  is  useful  in  gaining  some  idea  as  to 
simulation,  and  as  to  the  diagnosis  of  a  lumbago.  The  untrained 
simulator  gets  up  in  a  variety  of  ways,  the  lumbago  patient  usually 
rises  on  all  fours,  the  arms  in  front,  somewhat  as  does  the  pseudo- 
hypertrophic dystrophy  patient. 

In  those  groups  of  patients  with  more  serious  neuritic  involvements 
there  are  added  the  signs  of  muscular  atrophy,  of  circumscribed  pa- 
ralyses, of  fibrillary  contractions,  of  contractures,  of  sensory  disorders, 
and  of  trophic  disturbances. 

The  atrophies  may  be  true  atrophies,  but  are  usually  position 
or  disuse  atrophies.  They  are  demonstrated  by  palpation,  or  by 
measurements. 

Moreover,  the  atrophies  may  be  segmentary,  or  radicular,  in  either 
of  which  instances  the  localizing  diagnosis  is  aided.  The  electrical 
examination  of  the  muscles  in  sciatic  neuritis  is  usually  contradictory. 
In  positive  cases  with  partial  R.  D.  the  nature  of  the  process  becomes 
clearer. 

»  Deutsch.  med.  Wchnschr.,  1898. 


SCIATIC  NEURALGIAS  391 

In  the  beginning  of  the  process  the  patellar  reflex  is  frequently 
increased  on  the  aftected  side,  while  if  marked  neuritis  be  present  it 
may  be  diminished  or  absent.  The  Achilles  tendon  reflex  shows  a 
similar  reaction.  Oppenheim  has  called  attention  to  a  mild  degree  of 
flabbiness  or  diminution  in  the  size  of  the  xA.chilles  tendon  on  the 
aftected  side.  A  pseudo-Babinski  is  described,  largely  a  plantar 
flexion  of  the  smaller  toes,  with  immobility  of  the  great  toe. 

Sensibility  is  often  unaffected,  but  careful  testing,  following  Head's 
methods,  may  show  a  hj^^esthesia  to  touch,  pain  and  temperature. 
Occasionally  the  disturbance  of  sensibility  shows  a  marked  radicular 
distribution,  which  speaks  for  a  more  or  less  localized  process  in  the 
plexus,  frequently  of  a  sjqjhilitic,  meningomyelitic,  or  osteo-arthritic 
nature. 

Trophic  and  vasomotor  phenomena  are  not  infrequent,  consisting 
either  of  acroparesthesise,  erji;hemas,  local  cyanoses,  diminished  or 
increased  perspiration,  changes  in  the  growth  and  character  of  the 
hair  or  even  the  nails.  True  herpes  is  rare.  Glycosuria,  polyuria, 
azoturia  are  rare  complications. 

Course. — In  the  early  attacks  in  healthy  adults,  the  prognosis  is 
good.  The  patients  usuall}^  recover  in  from  six  to  eight  weeks  under 
proper  treatment.  A  failure  to  respond  should  excite  suspicion  as  to 
the  diagnosis,  especially  with  reference  to  tabes,  to  a  spinal  growth  or  a 
radiculitis.  Recurrent  cases  usually  develop  a  tormenting  chronicity, 
which  exhausts  one's  therapeutic  resources,  and  occasionally  drives 
the  patient  into  a  mental  state  which  suicide  or  chronic  morphinism 
alone  terminates.  Fortunately  such  cases  are  becoming  rarer  with 
better  methods  of  diagnosis  and  enlarged  therapeutic  resources. 

Clinical  Forms. — Certain  variants — largely  based  on  etiological 
concepts — may  be  met  with.  Fournier's  gonorrheal  sciatica  with  an 
acute  onset,  slight  temperature,  with  prostatic  and  articular  com- 
plications is  one.  Brissaud  has  described  a  spasmodic  type  with 
increased  tendon  reflexes,  contractures  in  the  peri-articular  muscles 
of  the  hip,  and  trepidation  or  pseudoclonus.  Quenu's  varicose  sciatica, 
which  has  certain  analogies  to  the  intermittent  claudication  of  arterio- 
sclerotic type,  is  characterized  by  deep-seated  pain,  and  a  very  pro- 
tracted onset  and  chronic  course.  Psychogenic  sciaticas  may  always 
be  expected,  but  they  are  extremely  rare,  except  under  war  condi- 
tions. 

Diagnosis. — The  increased  knowledge  afforded  by  lumbar  puncture, 
x-rays,  and  finer  modes  of  testing  for  sensory  disturbances  is  dismem- 
bering the  old  sciatica  group  fairly  rapidly.  Of  the  more  common 
diagnostic  errors,  tabes  dorsalis  and  lumbago  call  for  special  mention. 

Tabes  lacks  the  pressure  points,  the  Lasegue  phenomenon,  and 
usually  shows  the  lost  knee-jerks,  lost  Achilles-jerk,  and  possibly 
the  Argyll-Robertson  phenomenon.    The  pains  are  usually  bilateral. 

Lumbago  is  usually  much  relieved  by  the  recumbent  posture,  and 
is  increased  by  the  movements  of  the  trunk;  the  site  of  the  pain  is, 
as  a  rule,  higher. 


392  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

Lumbosacral  radiculitis  calls  for  special  mention  since  many  of 
the  classical  chronic  sciaticas  fall  under  this  disorder. 

Muscular  rheumatism  (myositis)  lacks  the  pressure  points,  Lasegue's 
sign,  and  the  pains  are  more  diffuse. 

Spinal-cord  tumors  in  their  initial  symptomatology  cause  sciatic 
pains,  usually  bilateral,  occasionally  unilateral,  but  careful  sensory 
examination  soon  shows  anomalies;  special  localizing  signs,  pareses  and 
trophic  symptoms  point  to  a  severe  lesion  of  the  cord. 

Hip-joint  disease  lacks  the  classical  situation  of  the  pains.  Arthritis 
deformans  cases  with  sciatic  pains  show  diminished  power  of  abduction 
and  adduction  and  the  joints  are  painful. 

Intermittent  claudication  occasionally  gives  rise  to  diagnostic 
difficulties.  Its  arteriosclerotic  nature  is  revealed  by  palpation  of  the 
bloodvessels,  and  by  .r-ray  examinations  which  show  the  tortuous 
modified  vessels. 

Achillodynia,  Morton's  tarsalgia,  relaxation  of  the  sacro-iliac  joint, 
and  flat-foot  occasionally  cause  sciatica-like  syndromes. 

Therapy. — Rest  and  quiet  are  the  first  essentials.  Then  an  etiologi- 
cal therapy  becomes  imperative.  Mercury  for  syphilitic  cases,  quinin 
for  malarial  cases,  surgical  intervention  for  pressure  cases,  diet  for 
diabetic  cases,  rest  for  sacro-iliac  cases,  orthopedic  measures  for  flat- 
foot,  etc. 

While  one  is  waiting  to  obtain  a  clear  notion  as  to  etiology,  general 
treatment  may  be  necessary.  Such  treatment  should  always  be 
regarded  as  provisional,  not  final. 

For  the  treatment  of  the  pain,  analgesics  are  imperative.  These 
are  numerous,  and  different  patients  will  respond  to  different  members 
of  the  group.  Even  during  an  attack  it  may  be  found  that  one  anal- 
gesic has  lost  its  value,  and  another  must  be  substituted.  It  is  of 
value  to  bear  the  chemical  structure  of  the  various  analgesics  in  mind 
in  one's  therapeutic  endeavors.  Antipyrin,  or  its  related  products 
aspirin,  salipyrin,  pjTamidon;  the  amido-phenol  series,  with  phenace- 
tin,  lactophenin,  exalgin,  apolysin,  citrophen,  phenosal,  phenocol,  and 
salocol,  as  representatives  are  often  of  signal  service,  but  require 
considerable  testing.  Acetanilid  itself,  with  its  compounds,  is  in  wide 
use.  These  analgesics  can  be  used  in  combination,  when  smaller  doses 
of  the  two  or  three  in  use  are  more  efficient  than  large  doses  which 
often  have  marked  toxic  action,  either  on  the  blood  cells  (acetanilid, 
amido-phenol  series)  or  on  the  vasomotors  (antipyrin  derivatives). 

In  the  presence  of  anemia,  iron  and  arsenic  should  be  added. 

Counter-irritation  is  especially  useful.  It  is  best  practised  by 
means  of  the  actual  cautery  (Paquelin)  but  mustard  plasters,  canthar- 
ides  plaster,  deep  but  careful  massage,  etc.,  are  useful  adjuvants. 
Applications  should  be  made  along  the  nerve  trunks. 

Hydrotherapy  is  often  extremely  valuable  but  must  be  employed 
with  reason.  A  too  energetic  hydrotherapy  with  massage  often 
aggravates  a  sciatic  pain,  especially  in  the  initial  stages  when  rest 
is  so  imperative.    Later  hot  packs,  mud  baths,  spray  douches,  with 


SPECIAL  LOCALIZED  FORMS  OF  NEURALGIAS  393 

mild  massage  are  indicated.  In  many  patients  the  treatments  carried 
out  in  bath  resorts  is  especially  indicated.  Hot-air  treatment  is  not 
well  borne  in  the  initial  stages,  but  later  is  grateful  and  of  therapeutic 
value. 

Direct  nerve  injections  of  substances  having  a  degenerative  action 
on  nerve  fibers,  osmic  acid,  carbolic  acid,  etc.,  are  to  be  condemned. 
Infiltration  methods,  using  water  or  cocaine,  or  allied  substances,  or 
various  mixtures  have  more  to  recommend  them.  Schlosser  has 
reported  excellent  results  but  has  also  had  permanent  palsies  follow 
his  injections. 

At  times  it  may  be  deemed  necessary,  by  reason  of  the  severe  pain, 
to  practise  injections  of  stovaine,  cocaine  or  allied  substances  into  the 
region  of  the  cauda  or  into  the  spinal  cord  (Corning).  Such  injections 
are  useful,  but  their  action  is  temporary  as  a  rule.  Nerve  stretching 
is  to  be  condemned. 

The  opium  derivatives  should  be  used  only  as  a  last  resort. 

Electrotherapy. — The  older  methods  of  galvanization  and  faradiza- 
tion are  useful  in  a  few  cases,  but  on  the  whole  are  unsatisfactory. 
Sinusoidal  currents  are  more  valuable,  while  the  Leduc  rapidly  alter- 
nating currents  are  almost  alwaj's  of  some  service  in  relieving  pain 
but  not  in  curing.  High-frequency  currents  with  the  use  of  the  ultra- 
violet rays  at  times  give  extremely  satisfactory  results  from  the  same 
standpoint. 

Lumbo-abdominal. — These  occupy  the  lower  half  of  the  trunk,  and 
are  extremely  variable.  The  chief  nerves  involved  are  the  iliohypo- 
gastric and  its  branches,  the  inguinal,  and  genitocrural.  Strict  locali- 
zation to  one  trunk  is  rare,  and  men  are  more  frequently  affected  than 
women.  The  chief  causes,  in  addition  to  those  of  general  moment, 
are  local  inflammatory  conditions  or  new  growths  involving  the  plexus 
or  some  of  its  branches.  The  pains  are  usually  unilateral,  occasionally 
bilateral,  involve  the  region  of  the  back  below  the  ribs,  the  gluteal 
region,  the  abdominal  and  inguinal  areas,  the  scrotum,  or  the  labia. 
The  chief  Valleix's  points  are  over  the  lumbar  vertebrae,  the  hip  or 
iliac  point,  hypogastric  point,  and  the  scrotal  point.  Lumbo-abdom- 
inal pains  are  usually  accompanied  by  intercostal  pains  above  or 
thigh  pains  below. 

Testicular  Neuralgia. — Astley  Cooper  termed  this  neuralgia  the 
"  irritable  testicle."  The  pains  are  usually  unilateral  and  pass  into  the 
testicle  which  may  be  swollen  and  tender  to  the  touch.  The  pain  not 
infrequently  passes  into  the  leg  and  back,  and  the  patient  may  have 
an  attack  of  vomiting.  Bernhardt  notes  that  the  pain  may  be  so 
intense  as  to  cause  the  patient  to  seek  castration.  The  affection  is  an 
obstinate  one,  and  is  not  helped,  as  a  rule,  by  removal  of  the  testicle. 
Diagnosis  involves  a  rigid  exclusion  of  somatic  disorder  of  the  testicle 
although  many  affections  (gonorrhea,  tuberculosis,  chronic  prostatitis 
etc.)  are  not  infrequently  accompanied  by  persistent  neuralgic  pains. 

Crural  Neuralgia. — The  crural  or  femoral  nerve  is  here  implicated. 
The  pain  extends  in  the  upper  front  and  inner  side  of  the  thigh,  to 


394  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

the  knee,  and  farther  through  the  saphenous  distribution  to  the  ankle 
and  inner  aspect  of  foot,  extending  as  far  as  the  big  toe.  It  is  almost 
entirely  confined  to  men,  and  shows  considerable  variability  as  to  the 
branch  involved.  It  not  infrequently  accompanies  a  sciatica.  Special 
etiological  features  are  found  in  fecal  impaction,  or  even  chronic  con- 
stipation, disease  of  the  hip  or  knee  bones,  enlargement  of  the  inguinal 
glands,  aneurism  of  the  iliac  artery.  Charcot  called  attention  to  the 
frequent  association  of  crural  neuralgia  and  diabetes.  Spinal  arthritis 
is  an  obscure  cause. 

Movements  of  the  thigh  usually  are  painful  and  the  patient  comes  to 
bend  his  body  forward  in  a  strained  position.  The  painful  points  of 
greatest  frequency  are  just  below  Poupart's  ligament,  just  within  the 
inner  condyle,  over  the  malleolus,  inner  side  of  the  instep,  and  one 
over  the  great  toe.  Neuro-atrophic  changes  usually  occur  in  the 
quadriceps,  but  the  patellar  reflex  is  rarely  affected,  save  when  a 
definite  neuritis  is  present.  Herpes,  reddening,  h;yT3eresthesia  are  not 
infrequent.  In  the  diagnosis,  disease  of  the  inguinal  vessels  is  to  be 
looked  for,  as  well  as  intrapelvic  disorders,  new  growths,  etc.  Crural 
neuralgias  have  a  fairly  good  prognosis. 

Femoral  Neuralgia. — Here  the  cutaneous  femoris  lateralis,  arising 
higher  up  in  the  pelvis,  is  involved.  The  pain  is  felt  in  the  upper  and 
outer  aspects  of  the  thigh,  extending  to  the  knee.  A  painful  point  over 
the  anterior  spinous  process  of  the  ilium  is  usual.  Paresthesia  in  the 
distribution  of  this  nerve  has  been  extensively  studied  (meralgia 
paresthetica) .  The  relation  of  the  pressure  of  corsets  in  the  causation 
of  this  type  of  neuralgia  has  been  pointed  out  by  Freud,  and  much 
sitting  in  adipose  individuals  is  frequently  associated  with  this 
neuralgia.     The  prognosis  is  favorable. 

Obturator  Neuralgia. — Lesions  of  this  nerve  are  fairly  constant  as  a 
result  of  the  pressure  of  the  intestinal  loops  of  a  hernia.  The  pain  is 
located  in  the  inner  side  of  the  thigh,  and  is  accompanied  by  a  feeling 
of  stiffness,  creepy,  crawly  feelings  of  the  skin,  and  inability  to  bring 
the  thigh  toward  the  middle  line  of  the  body. 

Neuralgias  of  the  Pudendal  Plexus. — A  large  number  of  neuralgias 
of  the  genital  plexus  are  recorded.  The  median  hemorrhoidal  branches, 
distributed  to  the  rectum,  bladder,  and  vagina,  the  inferior  branches 
to  the  anus,  and  the  pudendal  nerve  supplying  the  testicular  sac,  the 
labia,  penis,  urethra,  and  clitoris,  are  the  chief  nerves  involved.  The 
general  terms,  spermatic  neuralgia,  anal  neuralgia,  perineal  neuralgia, 
rectal  neuralgia,  vesical  neuralgia  or  cystalgia,  urethralgia,  prostalgia, 
penis  neuralgia,  irritable  uterus,  ovarian  neuralgia,  are  utilized  to 
describe  these  different  affections.  These  neuralgias  are  very  rare, 
but  often  very  obstinate.  Spermatic  neuralgias  are  among  the  most 
frequent,  and  are  not  infrequently  accompanied  by  painful  priapism, 
perhaps  ejaculation. 

Since  the  advent  of  bicycle  riding  neuralgias  of  this  general  region 
have  been  on  the  increase.  The  ovarian  neuralgias  are  complex,  and 
more  often  come  within  the  domain  of  the  gynecologist,  as  structural 


HERPES  ZOSTER  395 

defects  are  often  the  underlying  causes.  Localized  herpetic  eruptions 
accompany  neuralgias  of  this  plexus.  Lesions  of  the  cauda  equina 
are  to  be  carefully  excluded  in  neuralgias  of  this  region. 

Neuralgias  of  Coccygeal  Plexus. — Coccygodynia,  painful  coccyx,  is 
a  not  infrequent  disorder  in  women,  especially  in  multiparse  and  in 
the  badly  constipated.  Trauma  and  caries  are  frequent  causes.  The 
psychogenic  coccyx  is  not  unusual  and  referred  coccygeal  pains  are 
common.  The  pain  is  so  intense  at  times  that  defecation  is  rendered 
impossible;  the  patient  cannot  sit,  and  a  grave  neurasthenic  condi- 
tion supervenes.  The  medicolegal  significance  of  coccygod\Tiia  is  real, 
appearing  frequently  as  a  local  s^'mptom  of  a  general  traumatic 
neurosis.  Surgeons  frequently  lay  considerable  stress  on  a  freely  mov- 
able coccyx  in  accident  litigation.  A  just  estimate  of  the  true  bearing 
of  an  injiu-y  to  the  coccyx  can  only  be  arrived  at  by  a  careful  survey 
of  all  of  the  factors  of  the  particular  case. 

Local  treatment  is  seldom  efficacious  save  in  the  truly  neiu-algic 
tj'pes.    Resection  is  rarely  a  justifiable  procedure. 

HERPES  ZOSTER:  SHINGLES:  ZONA.     RADICULOGANGLIONIC 
SYNDROME  (ACUTE  POSTERIOR  POLIOMYEUTIS). 

In  a  broad  sense  zoster  consists  of  a  special  t^pe  of  painful  erj-the- 
matous  eruption  with  formation  of  vesicles  occupying  the  radicular 
distribution  of  the  segment  involved,  due  to  disease  of  the  posterior 
roots  and  the  sensory  ganglion.  In  this  sense  it  may  be  due  to  an  acute 
or  chronic  meningitis,  tabes,  Pott's  disease,  carcinoma  of  the  vertebrae, 
acute  infectious  diseases,  intoxications  or  other  lesions  implicating 
the  posterior  roots  and  the  ganglion  (s\Tiiptomatic  zoster). 

In  a  narrower  sense  it  may  be  conceived  of  as  a  specific  infectious 
disease  affecting  the  ganglion  cells  in  the  posterior  spinal  ganglia 
and  the  adjacent  fibrillary  structures  (essential  zoster  or  posterior 
poliomyelitis) . 

History. — Zona  was  first  well  described  by  Rayer  in  1835,  although 
notes  on  its  occurrence  date  from  Hippocratic  times.  Baerensprung, 
in  1861,  gave  the  first  important  monograph,  and  pointed  out  the 
implication  of  the  ganglia  as  an  essential  feature  of  the  disease,  while 
Head  and  Campbell  (1900)  called  particular  attention  to  the  specific 
infectious  type.  Rosenow  and  OftedaP  have  isolated  streptococci  from 
the  ganglia. - 

Etiology. — Nearly  all  of  the  general  causes  which  give  rise  to  a 
neuralgia  or  a  neuritis  may  by  an  extension  or  an  intensification  of  the 
pathological  process  involve  the  posterior  ganglia  and  thus  develop  a 
herpes.  In  poisoning  by  arsenic  and  carbon  monoxide  these  ganglia 
seem  to  be  specially  afi'ected,  and  the  acute  gastro-intestinal  aft'ections, 
pneumonia,  and  tuberculosis  are  not  infrequently  contributory  factors. 

*  Jour.  Am.  Med.  Assn.,  June  12,  1915. 

'E.  Salomon:  Ztschr.  f.  d.  ges.  Neurol,  u.  Path,  ref.,  vii,  34.5,  for  recent  work  on  this 
syndrome. 


396  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 


1 


Trousseau  first  called  attention  to  the  zoster,  which  was  a  specific 
infection,  which  type  has  been  so  extensively  studied  by  Head  and 
Campbell.    Epidemics  of  zoster  point  to  the  truth  of  this  position. 

Symptoms. — Neuralgic  pains  and  a  skin  eruption  constitute  the 
main  symptoms.  The  disorder  shows  a  slightly  different  order  of 
development  according  to  the  etiological  factors.  In  the  pure  or 
essential  zosters  (acute  posterior  poliomyelitis)  there  is  usually  a 
feeling  of  malaise,  a  slight  temperature,  and  gastro-intestinal  disturb- 
ances, then  the  patient  has  neuralgic  pains  which  may  be  mild  and 
superficial,  burning  or  pricking,  or  deep  and  extremely  severe,  and 
in  from  three  to  four  days  an  eruption  develops.  There  is  marked 
hyperesthesia  of  the  skin  along  the  affected  segment,  with  redness,  and 


Fig.  218. — Herpes  zoster.     Typical  thoracic  location.      (Knowles.) 

suddenly  or  gradually  there  appears  a  group  of  vesicles  varying  in  size 
from  a  few  millimeters  to  a  few  centimeters.  These  vesicles  are  rarely 
confluent,  and  the  fluid,  which  is  at  first  serous,  sometimes  tinged  with 
blood,  may  later  become  purulent.  The  vesicles  gradually  dry,  leaving 
a  scaly,  yellowish-brown  stained  scar  which  persists  for  a  long  period. 
Ulceration  or  gangrene  occasionally  occurs,  especially  in  diabetes. 
The  cycle  occupies  about  four  to  eight  days.  There  is  usually  some 
anesthesia  to  both  epicritic  and  protopathic  sensibility  after  the  acute 
stage  has  passed.    One  attack  seems  to  confer  immunity. 

In  the  symptomatic  zosters  of  the  infectious  type  there  is  rarely 
fever  or  gastro-intestinal  disturbance,  the  development  of  the  eruption 
is  usually  irregular,  and  it  often  shows  a  chronic  character.    Symp- 


RADICULITIS  397 

tomatic  zona  may  involve  both  sides,  whereas  the  infectious  (influenza, 
etc.)  t\^e  is  nearly  always  one-sided. 

In  the  dorsal  t\'pes  only  is  the  girdle  distribution  maintained  (inter- 
costal herpes  zoster),  whereas  involvement  of  the  Gasserian,  cervical, 
lumbar  or  sacral  roots  gives  rise  to  irregular  eruption  appearances  by 
reason  of  the  segmental  complexities  of  these  regions. 

Ophthalmic  zoster  is  an  especially  severe  type,  occurring  in  individ- 
uals above  middle  life,  and  often  accompanied  by  alcoholic  and  arterio- 
sclerotic factors.  It  may  develop  apparently  like  an  erysipelas  of  the 
face,  with  severe  neuralgia,  and  then  a  widespread  vesicular  eruption, 
even  involving  the  mucous  surface,  develops.  Ocular  complications, 
conjunctivitis,  keratitis,  iritis,  of  a  severe  nature,  are  not  infrequent. 
Facial  palsy  may  be  present  in  zoster  of  the  cephalic  ganglia;  thus  the 
geniculate.  A  symptomatic  ophthalmic  zoster  due  to  lesions  in  the 
region  of  the  pons  occurs. 

Pathology. — In  essential  zona  there  is  an  acute,  often  hemorrhagic 
inflammation  in  the  sensory  ganglia.  These  are  swollen,  the  capsule 
notably  thickened,  ^^ith  marked  infiltration  of  leukoc5i;es.  The 
ganglion  cells  are  in  part  destroyed  or  damaged,  and  the  contiguous 
fibrillary  structures,  both  central  and  peripheral,  are  also  involved 
in  the  inflammatory  reaction.  The  inflammation  is  usually  limited 
to  a  few  ganglia.  In  the  spinal  cord  secondary  degenerations  have 
been  observed,  and  occasionally  there  is  an  extension  of  the  general 
process  to  the  cord.  In  certain  cases  of  what  appears  to  be  essential 
zona  the  ganglia  have  been  free,  the  only  lesions  found  being  those  of 
a  neuritis.  There  is  an  inconstant  IjTnphocytosis  of  the  cerebro- 
spinal fluid  in  the  infectious  zones,^ 

Treatment. — For  the  sjTnptomatic  cases,  the  cause  must  be  found. 
Otherwise  the  treatment  is  purely  sjinptomatic.  Local  applications 
of  zinc  oxide  ointment  for  protection  and  the  use  of  a  mild  antiseptic 
to  prevent  suppuration  are  advisable.  For  the  pains  the  analgesic 
remedies,  already  spoken  of  in  the  treatment  of  neuralgia,  are  usbful. 
Gastro-intestinal  therapy  relieves  the  discomfort  and  itching  some- 
what, and  may  possibly  limit  the  accumulation  of  a  possible  secondary 
irritant. 

RADICULITIS. 

The  radicular  syndrome,  often  confused  with  neuralgia  and  neuritis, 
is  due  to  an  inflammatory  or  traumatic  lesion  of  the  spinal  nerve  roots, 
usually  of  the  brachial,  and  of  the  lumbosacral  plexuses. 

Attention  has  been  given  to  it  chiefly  by  French  neurologists, 
notably  Dejerine  (1905)  and  his  pupils.^ 

Symptoms. — These  are  neuralgic  pains,  which  are  usually  severe, 
yet  very  variable.  They  usually  occur  in  crises,  and  are  sticking 
and  lancinating  in  character,  at  times  extremely  intense;  not  infre- 

'  Schallcr:     The  Cerehrospinal  Fluid  in  Herpes  Zoster,  Calif.  State  Jour.  Med.,  1918. 
'Dejerine  et  Thomas:     Maladies  de  la  moclle  epini6re,  1909.     Revue  neurol.,  1917. 
Mayer,  E.:  Jour.  Am.  Med.  Assn.,  August  3,  1918. 


398 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


quently  resembling  the  pains  of  tabes.  The  pains  have  a  tendency 
to  remit  and  then  to  recur  at  shorter  intervals,  leaving  a  certain 
soreness  behind.  The  nerve  trunks  are  usually  not  markedly  painful. 
There  is  usually  a  marked  hyperesthesia  over  the  radicular  segment. 
It  does  not  follows  the  peripheral  distribution  as  in  a  non-radicular 
neuralgia.  This  hj'peresthesia  is  usually  followed  by  an  anesthesia 
to  both  epicritic  and  protopathic  sensibility,  and  occasionally  bony 
sensibility  is  involved  as  well  if  the  inflammatory  reaction  is  intense. 
Deep  sensibility  may  be  so  involved  as  to  cause  astereognosis.  Par- 
esthesise  and  acroparesthesise  are  common.^ 


Figs.  219  and  220. — Topography  of  the  sensory  disturbance  in  a  syphilitic  radiculitis 
(type  Klumpke).     The  Cg  and  Z>i,  distribution  are  involved.     (Dejerine.) 

It  is  essential  that  these  features,  which  may  be  found  in  other 
affections,  be  radicular  in  their  distribution.  They  are  not  segmentary, 
i.  e.,  involving  the  hand,  the  forearm,  or  the  arm;  nor  do  they  follow 
the  peripheral  nerve  distribution. .  They  are  distributed  in  long  bands 
down  the  arm  or  the  leg,  corresponding  to  the  root  segments  involved. 
(See  Figs.  227,  231,  and  also  Figs.  23  to  31,  and  Plates  X  and  XL) 

It  is  a  striking  fact  that  sneezing  or  coughing  may  bring  on  a  par- 
oxysm of  pain  in  the  cervicodorsal  plexus,  and  coughing  and  straining 
at  stool  may  bring  on  pain  in  a  sciatic  radiculitis. 

In  the  upper  extremity  it  is  rare  to  find  a  pure  radiculitis,  i.  e.,  one 
without  some  motor  involvement,  while  for  the  lower  limb  the  great 
majority  of  the  classical  cases  of  sciatica  are  due  to  a  radiculitis. 


1  Rousellier,  Paris  These,  1907. 


RADICULITIS 


399 


In  the  mixed  cases,  involvement  of  the  anterior  roots  causes  muscular 
atrophies,  likewise  radicular  and  not  peripheral  in  their  distribution. 
The  atrophy  is  rarely  accompanied  by  fibrillary  twitchings,  or  by 
spasmodic  contractions. 

Ataxias  may  be  met  with.    Lasegue's  sign  is  commonly  found. 

The  knee-jerks  are  primarily  exaggerated,  later  diminished  or  lost 
in  sacral  forms. 

Trophic  disturbances,  particularly  in  the  joints,  and  vasomotor 
signs  (cyanosis)  are  found  in  long-standing  cases.  In  rare  instances 
the  anterior  roots  are  involved  primarily. 


Figs.  221  and  222. — Scheme  of  root  (radicular)  segment  distribution. 
(Compare  with  Figs.  27  to  32.) 


(Flatau.) 


Diagnosis. — Lumbar  puncture  may  reveal  a  lymphocytosis.  As 
noted,  radiculitis  of  the  lumbosacral  plexus  has  for  the  most  part  been 
grouped  with  the  sciatic  neuralgias;  both  under  the  so-called  true  and 
the  svTnptomatic  neuralgias. 

The  presence  of  sensory  changes,  using  the  procedures  of  Head 
and  Dejerine,  the  radicular  distribution  of  the  hyperesthesije,  the 
anesthesite  and  the  atrophies  are  sufficient  to  determine  a  diagnostic 
picture.  The  crural  nerve  is  more  often  iuNoived  in  the  radicular 
process  than  in  the  peripheral  sciaticas.' 

'  Dejerine:    Semiologi6  du  systeme  nervcux,  1914. 


400  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

The  presence  of  ataxia  in  the  lower  limbs,  occasionally  in  the  upper, 
Romberg's  sign,  and  the  frequent  loss  of  the  knee-jerks  often  leads  to 
the  mistaken  diagnosis  of  tabes.  From  one  point  of  view  tabes  begins 
as  a  radiculitis,  and  the  diagnostic  difficulty  centers  about  the  etio- 
logical element.  Since  so  much  of  radiculitis  of  the  lower  extremity 
is  due  to  syphilis,  the  ordinary  lumbar  puncture  and  Wassermann  tests 
are  essential  to  make  the  diagnosis  positive.  In  tabes  one  must  also 
take  into  consideration  the  involvements  of  the  cranial  nerves,  Argyll- 
Robertson  pupil,  etc. 


Fig.  223. — Syphilitic  meningomyelitis  with  radicuHtis. 

Cervical  radiculitis  naturally  travels  under  the  guise  of  a  cervico- 
brachial  neuralgia.  Here  the  greater  implication  of  the  sensory  system 
is  enough  to  make  a  diagnosis.  In  mild  early  cases  the  diagnosis  of  a 
radiculitis  is  impossible.  Muscular  atrophy  is  a  frequent  complication 
of  cervical  radiculitis.  Furthermore,  pupillary  phenomena  (Klumpke) 
are  more  common  in  this  type. 

Brachial  radiculitis  gives  rise  at  times  to  a  typical  Aran-Duchenne 
atrophy — the  biceps,  anterior  brachial,  supinator  longus  and  deltoid  — 
the  fifth  and  sixth  cervical  roots  being  chiefly  involved.    The  Klumpke 


NEURITIS  401 

oculopupillary  phenomena,  i.  e.,  diminution  in  size  of  the  palpebral 
fissure,  slight  retrogression  of  the  eyeball  and  miosis  are  present  if 
the  last  cervical  and  first  dorsal  roots  are  involved. 

Disseminated  types  are  described  by  Dejerine^  with  practically 
all  the  signs  of  a  tabes.     Cervical  rib  may  cause  a  radiculitis. 

Occasionally  cerebral  tumors  cause  the  s\Tnptoms  of  a  radiculitis 
with  alterations  in  the  posterior  columns  and  radicular  lesions  (Collier, 
1899;  Nageotte,  1909;  RajTnond,  1907).    Both  limbs  may  be  involved. 

Acroparesthesia  was  described  originally  by  Gamberini  in  1844, 
later  by  Nothnagel  (1881),  by  Putnam  (1882),  named  by  Schultze, 
and  then  shown  by  Pick  to  be  due,  so  far  as  the  so-called  chronic 
organic  cases  were  concerned,  to  a  radicular  or  intraspinal  involve- 
ment, principally  distributed  in  the  lower  half  of  the  cervical  region. 
Other  areas  may  be  involved  and  vegetative  level  acroparesthesise 
(hypothyroidisms)  as  well  as  psychogenic  acroparesthesife  also  are 
frequent. 

Its  s\Tnptoms  are,  in  the  periodic  cases,  pains  or  burning,  or  cold 
sensations,  usually  in  the  fingers,  accompanied  by  blanching  and 
coldness  of  the  skin  with  the  sensation  of  engorgement  and  extreme 
heaviness  of  the  hand  or  the  fingers.^ 

Treatment.^ — Spontaneous  radiculitis  seems  to  be  preeminently 
syphilitic.  Hence  an  antisA'philitic  treatment  is  indicated  in  every 
case  of  suspected  radiculitis.  Other  forms  of  meningitis  may  produce 
it,  however.  The  prognosis  is  good  in  the  sj^hilitic  forms,  but  less 
so  for  the  others.  Many  patients  with  cervical  and  brachial  radicu- 
litis recover  spontaneously  after  from  six  to  ten  months.  Treatment 
seems  to  alleviate  but  not  cure.  The  general  treatment  for  a  neuritic 
neuralgia  is  indicated.  Violent-ray  exposure  should  be  tried  in  the 
intractable  cases. 

NEURITIS. 

Neuritis  is  a  generalized  inflammation  of  the  peripheral  nerves, 
involving  in  varying  degrees  of  completeness  the  motor,  sensory  and 
vegetative  fibers.  In  old-standing  cases  the  spinal  portions  of  the 
neurons  are  implicated. 

The  inflammatory  changes  may  proceed  either  from  the  perineurium, 
or  from  the  endoneurium  and  involve  the  axis-cylinders.  These 
may  be  poisoned  and  degenerate  (parenchymatous  inflammation). 
The  pathological  nature  of  the  lesion  bears  little  relation  to  the  general 
symptomatology,  and  pathogenically  considered  there  is  considerable 
interplay  of  the  various  processes. 

Etiology.— A  vast  vareity  of  causes  may  bring  about  a  neuritis, 
seen  either  as  the  result  of  acute  toxic  parenchymatous  changes,  acute 
degeneration  due  to  actual  pressure  or  injury,  or  some  acute  or  chronic 
inflammatory  changes  following  a  variety  of  noxa.  The  most  important 
of  these  causes  are  as  follows : 

•  Rev.  Neurol.,  1904,  p.  524. 

'^  Putnam:    Jour.  Nerv.  and  Meiit.  Dis.,  Septenil)er,  1916. 
26 


402  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Infections. — Neuritis  may  result  from  the  toxins  of  microorganisms, 
as  those  of  diphtheria,  tuberculosis,  syphilis,  influenza,^  smallpox, 
dysentery,  typhoid  fever,  pneumonia,  streptococcus,  occasionally 
measles,  scarlet  fever,  parotitis,  whooping-cough,  etc.  Practically 
there  is  no  infectious  disease  that  has  not  produced  a  toxic,  usually 
parenchymatous,  neuritis.  A  second  group  of  infections,  such  as 
leprosy,  beriberi,  malaria,  rabies,  bring  about  neuritis,  but  here  the 
mechanism  is  different. 

Intoxications. — Next  in  general  importance  are  the  intoxications, 
either  exogenous  or  endogenous.  Alcohol  plays  the  chief  role,  while 
arsenic,  lead,  carbon  monoxide,  sulphur,  and  anilin  compounds  are 
rarer  exciting  causes.  Of  the  auto-intoxications  diabetes  is  per- 
haps the  most  important,  leukemia,  and  also  anemia. 

Trauma. — A  third  important  cause  for  the  neuritic  process  is  trauma 
to  the  nerve,  either  as  the  result  of  accident  or  injury,  or  from  the 
pressure  of  new  growths,  dislocations,  false  positions  or  other  anomalies 
of  structure  combined  with  the  prolonged  fixed  positions  required  in 
certain  occupations  (professional  neuritis). 

Less  important  causes,  numerically  speaking,  are  extensions  of 
inflammatory  processes  (ascending  neuritis),  inflammations  about 
joints,  inflammations  of  adjacent  organs,  etc.  The  war  neuritides  form 
an  immense  group  for  which  special  monographs,  which  are  numerous, 
should  be  consulted  .^ 

Symptoms.^ — The  syirptomatology  of  neuritis  varies  enormously,  not 
only  so  far  as  the  etiological  factors  which  determine  general  trends 
of  reaction  are  concerned,  but  also  as  to  location,  onset,  etc. 

Only  the  symptoms  of  the  generalized  process  will  be  considered 
here,  reserving  for  the  sections  on  Pareses  or  Paralyses,  both  of  the 
plexuses  and  of  the  peripheral  nerves,  a  more  detailed  description  of 
the  various  isolated  types. 

It  has  already  been  indicated  that  the  conceptions  neuralgia,  radicu- 
litis and  neuritis  are  very  flexible — it  is  only  for  the  sake  of  description 
that  one  draws  more  or  less  arbitrary  lines  between  them. 

There  is  a  generalized  tA^^e  of  neuritis  which  of  and  by  itself  con- 
stitutes a  fairly  definite  syndrome.  This  is  so-called  multiple  neuritis, 
or  polyneuritis.    It  is  largely  due  to  toxemias,  organic  or  inorganic. 

Under  the  general  caption  of  peripheral  neuritis,  one  considers  a 
large  number  of  peripheral  palsies,  while  as  localized  neuritis  one  has 
to  consider  a  number  of  the  professional  neuritides. 

Polyneuritis,  Multiple  Neuritis. — This  is  a  general,  widely  distrib- 
uted, diffuse,  parenchymatous  neuritis  in  which  the  entire  peripheral 
neuron  is  involved.  Primary  or  secondary  degenerations  of  the 
cerebral  neurons  take  place. 

Etiology. — Multiple  neuritis  is  almost  invariably  due  to  some 
toxemia.     Such  toxemias  may  be  due  to  (1)  alcohol,  lead,    arsenic 

1  Jelliffe:    Loc.  cit. ;    New  York  Med.  Jour.,  November,  1918. 

^Tinel:  Nerve  Wounds,  1918.  Athanassio-Benisty,  1918:  Revue  Neurologique,  Neu- 
rologie  de  Guerre,  1915,  1916,  1917,  1918.     Stewart:  Nerve  Wounds  of  War,  1918. 


MULTIPLE  NEURITIS  403 

zinc,  carbon  monoxide,  bisulphide  of  carbon,  sulphuric  acid,  and 
some  of  the  rarer  metals:  mercury,  copper,  phosphorus,  etc.,  (2)  or 
to  the  toxins  of  acute  or  chronic  infectious  diseases,  such  as  smallpox, 
typhoid  fever,  grippe,  measles,  scarlet  fever,  diphtheria,  pneumonia, 
dysenteries,  streptococcemias,  leprosy,  malaria,  tuberculosis,  parasitic 
worms  and  s^'philis,  or  the  inflammation  may  result  from  (3)  auto- 
intoxications such  as  diabetes,  leukemias,  anemias,  beriberi,  etc.  Acute 
chilling  of  the  body  is  held  to  be  responsible  for  certain  cases,  particu- 
larly in  the  presence  of  some  infectious  diseases,  or  acute  toxemias, 
notably  in  alcoholic  cases,  cases  of  rabies,  influenza,  etc. 

Occurrence. — No  general  laws  can  be  made  with  reference  to  occur- 
rence because  of  the  wide  range  of  etiological  factors. 

Symptoms. — Clinically  considered,  one  meets  with  subacute  and 
acute  cases.  In  the  subacute  cases,  which  are  in  general  milder,  the 
patient  usually  begins  to  have  a  rapid  progressive  enfeeblement  of  the 
muscles,  as  a  rule,  of  the  lower  extremities.  There  is  rarely  any  fever 
at  the  onset,  and  the  loss  of  power  gradually  extends  from  the  periph- 
eral segments  toward  the  trimk.  Thus,  the  extensors  of  the  leg  and 
of  the  foot  first  show  weakness,  and  later  those  of  the  thigh  and  hip. 
At  the  same  time,  or  shortly  foflowing,  the  upper  extremities  may 
be  involved,  in  accordance  with  the  same  general  law,  the  muscles 
of  the  hand,  wrist  and  forearm  usually  being  primarily  involved. 
There  are  occasional  exceptions  to  this  general  law  of  progression,  but 
they  are  comparatively  rare. 

It  is  further  characteristic  that  the  weakness  and  paralysis  are  more 
or  less  symmetricaUy  distributed.  Although  one  leg  or  one  arm  may 
show  a  greater  amount  of  weakness  than  the  other  there  is  almost 
invariably  quadrilateral  involvement.  In  the  milder  cases  quantita- 
tive ^'ariations  in  the  severity  occur,  and  in  the  mild  subacute  cases 
the  cranial  nerves  are  less  often  diseased.  Still  the  muscles  of  the 
abdomen,  the  diaphragm,  the  face,  eyeballs  or  tongue  may  all  suffer. 

In  the  more  severe  cases,  the  implication  of  the  pneumogastric  is 
shown  by  tachycardia,  dyspnea  and  feebleness  of  the  pulse. 

The  superficial  reflexes  may  first  be  exaggerated,  but  later  become 
lost,  as  a  rule,  and  the  tendon  reflexes  usually  exhibit  the  same  phenom- 
ena. Disturbances  of  sensibility  are  usually  more  marked.  Initial 
pain  is  more  or  less  universal,  but  the  sensations  of  actual  pain  are 
often  preceded  by  tingling  or  creeping  sensations,  and  the  skin,  muscles, 
ner^'e  trunks  and  joints  may  all  show  h^^eresthesijF.  The  Lasegue 
phenomenon  is  universally  present. 

Careful  testing  of  epicritic  sensibility  may  show  no  loss,  although, 
as  a  rule,  the  sense  of  localization  to  light  touch  and  the  ability  to 
distinguish  between  two  points  of  a  compass  soon  become  some- 
what diminished.  A  certain  amount  of  loss  of  epicritic  temperature 
sense  may  also  be  met  with.  In  the  milder  cases  the  atrophy  gradually 
disap])ears,  and  there  is  no  tendency  to  the  development  of  contrac- 
tures, but  in  other  cases  contractures  may  follow,  and  the  limbs 
become  fixed  and  immobile. 


404  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

In  acute  generalized  polyneuritis  the  attack  begins  very  abruptly, 
usually  with  high  temperature  and  chill,  headache,  malaise,  suppression 
of  urine,  albuminuria,  and  the  general  signs  of  an  acute  illness.  Par- 
alyses develop  very  rapidly,  usually  involving  the  lower  extremities 
first,  and  gradually  ascending  the  trunk  and  the  arms,  closely  resem- 
bling the  ascending  type  of  acute  anterior  poliomyelitis  (Landry). 
There  is  great  tenderness  on  pressure  over  the  nerve  trunk,  sharp, 
shooting  pains,  marked  hyperesthesia  of  the  muscles  and  the  Lasegue 
phenomenon.  The  reflexes  are  rapidly  abolished,  the  patient  loses 
epicritic  sensibility,  is  unable  to  localize  touch,  cannot  distinguish 
points  of  the  compass,  but  rarely  loses  sense  of  pain  or  of  deep  pressure. 
The  sphincters  are  not  usually  involved,  except  in  extremis.  Atrophies, 
contractures,  trophic  disturbances  of  the  skin,  such  as  glossy  skin, 
pemphigus-like  eruptions,  perforating  ulcers,  fragile  nails,  thick  and 
fragile  hairs,  etc.,  develop.  Then  ocular  palsies  are  met  with,  occa- 
sionally facial  palsy,  tinnitus  frequently  results  from  cochlear  disturb- 
ance, and  neuritic  vertigo  from  vestibular  disorder  is  found.  Pupillary 
inequalities  are  frequent  in  the  severe  cases.  Sluggish  light  and 
accommodation  reflexes  are  fairly  constant  and  occasionally  a  true 
Argyll-Robertson  pupil  is  found.  Loss  of  accommodation  with 
retained  light  reflexes  is  met  with  occasionally. .  Amaurosis,  complete 
or  partial,  is  not  infrequent. 

The  cranial  nerve  nuclei  are  not  infrequently  involved.  Li  the  fatal 
cases  the  implication  of  the  pneumogastric  causes  death.  The  symp- 
toms are  those  of  asphyxiation,  or  with  cardiac  irregularity  and 
paralysis  of  that  organ. 

Course. — Diagnosis. — Treatment.^ — Inasmuch  as  multiple  neuritis 
varies  not  only  with  reference  to  its  symptomatology  and  course,  but 
offers  special  diagnostic  problems  according  to  the  etiological  factors, 
and  since  the  treatment  must  depend  upon  a  due  consideration  of  the 
etiological  factors,  it  is  best  to  discuss  these  problems  under  special 
heads. 

Alcoholic  Multiple  Neuritis. — This  is  the  most  common  of  all  the 
types  of  multiple  neuritis.  Any  form  of  alcohol-containing  drink  can 
cause  it,  including  heavy  beers.  Cologne,  ether  and  other  substances  of 
the  marsh-gas  series  are  among  the  etiological  curiosities  as  causing 
neuritis. 

It  is  usually  subacute  in  development,  although  occasionally, 
especially  if  the  patient  has  been  subjected  to  severe  cold,  the  disease 
may  begin  in  a  very  acute  manner,  and  present  the  picture  of  a  Landry 
syndrome.  In  a  few  instances  an  apoplectiform  onset  has  been  noted. 
Intercurrent  infectious  disease  in  an  alcoholic  may  constitute  the  point 
of  departure  for  a  polyneuritis;  this  is  especially  true  of  influenza. 

These  patients  usually  complain  of  tingling  sensations;  of  formica- 
tion over  the  hands  or  down  the  legs,  with  occasional  twinges  of 
pain,  particularly  in  the  legs.  The  skin  is  usually  hyperesthetic 
throughout  the  entire  body,  and  the  muscles  become  very  sore.  Such 
symptoms  may  be  present  for  weeks  or  even  months.     The  patient 


ALCOHOLIC  NEURITIS 


405 


commences  to  notice  difficulty  in  walking,  inability  to  go  up  and  down 
stairs  and  occasional  falling  when  called  upon  for  certain  muscular 
exertions. 

p]xamination  of  the  muscular  power  at  this  stage  shows  marked 
weakness,  particularly  in  the  extensors  of  the  feet,  and  of  the  hands. 
In  walking  there  is  a  tendency  to  "foot-drop,"  and  the  patients  lift 
the  legs  a  little  higher,  and  may  show  a  characteristic  flopping  step 
superficially  resembling  that  of  the  tabetic. 

In  this  stage,  before  patients  are  confined  to  bed  bv  reason  of  the 
muscular  weakness,  an  exaggeration  of  the  tendon  reflexes  is  occasion- 
ally found,  although  diminution  and  loss  become  the  rule.  Atrophy, 
flabbiness  of  the  muscles,  and  the  trophic  signs  may  then  appear. 

A  number  of  these  patients  are  able  to  be  about,  and  are  often 
mistaken  for  cases  of  beginning  tabes,  especially  as  there  is  very 
frequently  a  certain  amount  of  ataxia,  definitely  marked  in  the  lower 
extremities,  less  so  in  the  upper.    The  sphincters  are,  as  a  rule,  intact. 

In  some  severe  cases,  in  addition  to  the  physical  signs,  a  very  definite 
psychosis  develops.  This  is  treated  under  the  head  of  polyneuritic 
psychosis — chronic  alcoholic  delirium.  Korsakov's  psychosis.  (See 
chapter  on  Toxic  Psychoses.) 


Fig.  224. — Alcoholic  neuritis  in  chronic  stage  with  contractures. 

Course. — Alcoholic  polyneuritis  shows  an  innumerable  number  of 
variants,  but  in  the  main  it  runs  a  subacute  course.  The  patients 
go  through  the  usual  sjTnptoms  of  chronic  alcoholism,  with  tremor, 
sleeplessness,  gastric  disturbance  and  malnutrition.  In  the  severe 
cases  which  develop  marked  mental  signs — Korsakov's  syndrome — 
they  usually  go  through  one  or  more  periods  of  acute  delirium  (delirium 
tremens) . 

Then  the  neuritic  symptoms  commence  to  appear,  usually  with 
formication  or  other  paresthesiae  in  the  arms  and  legs.  Sudden  twinges 
of  pain,  particularly  in  the  lower  limbs,  are  frequent,  and  an  unusual 


406  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

sense  of  muscular  fatigue  develops.  Dynamometer  readings  show  this 
loss  of  power  and  excessive  fatigability  early  in  the  disorder.  The 
average  case,  if  drinking  continues,  and  the  malnutrition  and  insomnia 
are  not  overcome,  takes  three  or  four  to  eight  weeks  for  the  develop- 
ment of  definite  palsies.  The  patients  note  great  difficulty  in  going 
up  and  down  stairs,  with  much  palpitation  and  shortness  of  breath. 
Then  on  some  sudden  exertion  they  fall,  or  their  legs  shut  up  like  a 
jack-knife  under  them.  Thev  totter  in  their  walk,  or  show  a  steppage 
or  ataxic  gait.  The  disease  may  be  arrested  at  this  stage,  and  recovery 
takes  place  with  proper  care.  In  advancing  cases,  locomotion  becomes 
impossible.  Foot-drop,  wTist-drop,  and  ptosis  may  develop,  and  the 
atrophies,  contractures,  and  trophic  disturbances  come  on  rapidly. 
The  pains  are  of  increased  severity  and  frequency,  and  are  apt  to 
be  excruciating.  The  hyperesthesia,  which  has  been  excessive,  may 
now  be  gradually  and  irregularly  supplanted  by  hypesthesia  or  anes- 
thesia, h;ypalgesia,  or  analgesia,  and  extension  to  the  cranial  nerve 
nuclei  may  be  looked  for.  Sensitive  nerve  trunks  and  Lasegue's 
phenomenon  are  invariably  present. 

The  patient  who  has  advanced  to  the  stage  of  paralysis,  atrophies, 
and  trophic  changes  is  usually  confined  to  bed  for  several  months, 
and  then  commences  to  make  a  slow  and  irregular  recovery.  It  may 
be  complete,  but  there  is  apt  to  be  some  local,  persistent  impairment 
which  may  require  treatment  for  years,  especially  if  fibrotendinous 
contractures  have  developed.  In  the  Korsakov  cases  certain  grades 
of  residual  mental  impairment  are  extremely  common. 

No  two  cases  of  alcoholic  polyneuritis  are  alike.  There  is  a  general 
tendency  for  the  disorder  to  involve  all  four  extremities  in  the  pro- 
nounced cases,  but  the  lower  extremities  are  more  severely  implicated. 
Hyperesthesise  and  paresthesias  are  frequent.  Special  predilection  is 
shown  for  the  extensors  of  the  foot  and  wrist.  Pains  are  extremely 
severe  and  are  universal.  Blindness  (amaurosis)  is  frequent;  with 
methyl  (wood)  alcohol  it  usually  comes  on  in  advance  of  any  other 
neuritic  symptoms. 

Treatment. — Treatment  of  alcoholic  neuritis  involves  the  absolute 
withdrawal  of  alcohol  in  any  form,  complete  rest,  forced  feeding, 
particularly  with  foods  rich  in  fat — milk,  eggs,  butter.  Pain  is  best 
relieved  by  hot  applications;  continuous  warm  baths  are  very  grateful 
if  there  is  marked  hyperesthesia  of  skin,  muscles,  and  nerve  trunks; 
the  temperature  should  not  exceed  96°  to  97°  F.,  if  the  bath  is  to  be 
continued  for  any  great  length  of  time.  Active  catharsis  and  diapho- 
resis are  essential  in  the  early  stages.  After  the  stage  of  acute  hyper- 
esthesia is  passed  the  forced  feeding  should  be  continued  and  the  use 
of  strychnin  and  electricity  commenced.  Great  care  should  be  taken 
in  the  selection  of  the  hypnotics  used  to  give  sleep,  since  so  many  of 
them  contain  alcohol,  and  a  few  are  directly  poisonous  to  the  nerve 
trunks.  Bromids,  hyoscine,  or  occasionally  the  opium  derivatives  may 
be  employed.  If  an  alcohol  hypnotic  seems  necessary  paraldehyde 
and  chloral  are  indicated. 


ARSENICAL  NEURITIS  407 

Glycerophosphites  with  calcium  are  valuable,  preferably  not  given 
in  an  alcoholic  medium.  Massage  and  muscular  movements  of  various 
types  are  indicated  in  the  chronic  stages,  and  most  cases  will  repay  con- 
stant working  upon  them.  In  the  presence  of  contractures,  surgical 
intervention  may  be  necessary,  but  should  be  deferred  until  persistent 
massage  and  muscular  therapy  have  been  exhausted. 

Lead  Neuritis. — Lead  Palsy. — In  neuritis  from  lead  poisoning  which 
occurs  from  the  gradual  intake  of  lead  in  some  form — drugs,  hair 
dyes,  cosmetics,  contaminated  water,  certain  occupations  (plumbers, 
workers  in  type  foundries,  printers,  glaze  workers,  potters,  etc.)  the 
picture  is  very  different  from  that  seen  in  alcoholism,  although  the 
terminal  stages  may  be  similar.  The  histological  alterations  are 
practically  identical.    Many  cases  are  complicated  with  alcoholism. 

Symptoms. ^ — There  are  the  initial  gastro-intestinal  SAHiptoms  of 
lead  poisoning,  furred  tongue,  constipation,  attacks  of  colic,  headaches, 
anemia,  painful  joints,  and  perhaps  the  signs  of  a  nephritis.  The  gum 
lead  line  is  frequently  present.  After  a  few  months,  or  even  a  year  or 
so  of  exposure,  the  neuritis  develops,  often  after  an  attack  of  colic. 
It  usually  attacks  the  upper  extremities,  by  preference,  although  there 
is  always  some  slight  involvement  of  the  lower  limbs.  Definite  lower 
limb  palsies  are  rare,  and  usually  occur  only  in  children.  The  pareses 
predominate  particularly  in  the  extensors  of  the  index  finger  and  thumb 
— the  sensory  symptoms,  hyperesthesise,  pains,  nerve  tenderness, 
Lasegue's  phenomenon,  paresthesise,  are  usually  much  less  than  in 
alcoholic  neuritis.  The  supinator  longus  is  very  frequently  spared. 
The  paralyses  are  usually  sjTnmetrical,  but  may  be  quite  irregular; 
the  proximal  trunk  muscles  may  be  involved — the  distal  ones  free. 
This  occasionally  happens  in  alcoholic  neuritis  as  well.  Reaction 
of  degeneration  appears  in  the  paretic  muscles.  Anesthesia,  atrophies, 
trophic  disturbances,  and  contractures  are  met  with,  but  may  be 
considered  exceptional.  Oculomotor  palsies  also  occur,  and  optic 
nerve  atrophy  is  not  infrequent.  Other  cranial  nerves,  those  of  the 
larynx,  pharynx,  and  face  are  also  implicated,  though  rarely.  I^ead 
encephalopathies  resembling  those  of  alcohol  are  known. 

Course  and  Treatment. — Ihe  course  of  lead  poljiieuritis  is  essentially 
chronic,  lasting  from  several  months  to  a  year.  The  prognosis  is 
usually  favorable.  The  treatment  is  the  same  as  for  neuritis  in  general, 
with  the  addition  of  excessive  diuresis,  and  the  use  of  such  remedies, 
as  may  hasten  lead  elimination,  such  as  potassium  iodid. 

Arsenical  Neuritis. — Dejerine,^  in  1883,  first  insisted  on  the  essential 
similarity  of  neuritis  of  alcoholic  and  arsenical  origin,  which  view- 
point has  been  amply  verified  in  the  extensive  studies  following  a 
severe  epidemic  of  arsenical  polyneuritis  in  England,  in  1899-1900.^ 
The  most  frequent  source  of  arsenical  poisoning  has  been  shown  to 
come  from  impure  glucose  products;  the  sulphuric  acid  used  to  con- 
vert the  starch  containing  arsenic.     Other  sources  are  wall-papers, 

'  Comptes  rend.,  October,  1883,  vol.  xcviii.  No.  17. 
2  Lancet,  1900,  i,  1610. 


408  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

certain  manufactures  (dyestuffs),  artificial  flowers,  cosmetics,  beauty 
pastes  and  powders,  hair  dyes,  and  arsenic  used  in  medicines.  The 
pathological  alterations  (a  parenchymatous  neuritis)  do  not  differ 
from  those  seen  in  alcoholic  neuritis. 

Symptoms.^ — Acute  polyneuritis  from  arsenical  poisoning  is  rare.  It 
sets  in  shortly  after  the  gastro- intestinal  symptoms  have  ceased. 

In  the  chronic  cases  the  general  symptoms  of  chronic  arsenic 
intoxication  are  first  observed.  These  are  the  anorexias,  congestions 
of  the  upper  respiratory  tract  (nasal  catarrh,  cough)  or  more  frank 
diarrheas  of  gastro-intestinal  irritation.  The  neuritis  develops  simul- 
taneously with  the  symptoms  of  chronic  intoxication. 

As  with  alcoholic  neuritis,  sensory  symptoms,  paresthesige,  hyperes- 
thesise,  numbness,  shooting  pains,  sweating,  develop  first.  A  pig- 
mented condition  of  the  skin,  most  marked  about  normally  pigmented 
areas,  is  found  in  the  majority  of  the  cases.  The  pigmentation  may 
become  very  general  and  very  dark,  and  is  associated  with  herpetic, 
eczematous,  or  scaly,  papular  eruptions.  Certain  of  the  newer  prep- 
arations of  arsenic  which  have  been  extensively  advocated  for  the 
treatment  of  syphilis,  notably  atoxyl  and  arsacetin,  are  reported  to 
have  caused  optic  nerve  atrophy  with  blindness. 

The  signs  of  sore  nerve  trunks,  Lasegue's  phenomenon,  loss  of  motor 
power  in  both  extremities,  are  present  in  arsenical  cases.  Ataxias 
occur,  and  cases  of  arsenical  neuritis  have  been  confounded  with  tabes. 
The  prognosis  is  usually  good,  but  the  blindness  has  been  permanent. 

Other  Intoxications. — Carbon  monoxide,  diabetes,  and  illuminating 
gas  poisoning,  if  severe  and  not  lethal,  frequently  develop  a  severe  grade 
of  multiple  neuritis  not  differing  in  any  marked  degree  from  alcoholic 
polyneuritis.  The  gases  found  in  natural  gas,  and  in  many  artificial 
gases  contain  the  same  chemical  radicals  as  alcohol,  and  the  toxic 
action  is  identical.  In  the  very  severe  cases,  polio-encephalitis  develops 
with  multiple  softenings,  not  entirely  confined  to  the  thalamus  or 
corpora  striata. 

Carbon  bisulphide,  which  is  extensively  used  in  rubber  industries, 
may  give  rise  to  a  multiple  neuritis.  The  toxic  ion  is  not  definitely 
known.  Similar  poisoning  results  from  sulphonal  and  trional,  two 
sulphuric  acid-alcohol  hj'pnotics.  A  number  of  the  nitrobenzol  series 
can  produce  localized  or  general  neuritis. 

Phosphorus,  mercury,  copper,  and  silver  can  produce  poisoning  with 
the  development  of  multiple  neuritis. 

Phosphorus  metabolism  defect  (vitamines)  seems  to  underlie  the 
beriberi  cases. ^ 

Infectious  Disease  Types. — Mild  or  severe  general  neuritis  has 
occasionally  followed  nearly  every  infectious  disease. 

Diphtheria.— Here  the  neuritis  is  rarely  generalized,  and  the  cranial 
nerves  bear  the  chief  brunt  of  the  poison.  In  mild  forms  the  soft 
palate  and  pharyngeal  muscles  are  first  or  alone  implicated.    Oculo- 

'  Jelliffe:   Jour.  Nerv.  and  Ment.  Dis.,  June,  1919. 


PLEXUS  PALSIES  409 

motor  palsies  are  also  frequent.  Some  degree  of  facial  palsy  is  also 
met  with.  In  the  severe  t^'pes  the  larynx,  tongue  and  the  pneumo- 
gastric  inner\'ations  become  invaded.^ 

Generalized  forms  occur,  differing  in  no  essential  particular  from  the 
t^pes  already  described. 

Diphtheric  palsies  may  come  on  soon  in  the  disease  or  they  may 
follow  a  month  or  six  weeks  after  the  subsidence  of  the  disease  proper. 
For  those  patients  who  develop  polyneuritis  the  diagnosis  is  usually 
grave.  The  cranial  nerve  types  are  usually  less  severe,  although 
occasionally  one  finds  pneumogastric  palsies  which  are  fatal. 

Influenza. — The  toxins  of  the  influenza  organism  seem  to  have  a 
special  attraction  for  sensory  nerve  structures.  Neuralgias,  localized 
neuritides,  are  extremely  common,  and  pohTieuritis  not  a  rarity.  The 
poljTieuritis  is  of  a  parenchymatous  t^'pe,  is  usually  mild,  quite  irregu- 
lar, and  differs  in  no  essential  manner  from  other  t^pes  described. 
Its  course  is  rarely  over  a  few  months  and  the  prognosis  is  good. 

PohTieuritis  of  a  mild  parenchymatous  type,  occurs  as  a  sequel  of 
t}T)hoid  fever,  smallpox,  erj'sipelas,  pneumonia,  pleurisy,  acute  articu- 
lar rheumatism,  parotitis,  gonorrhea,  dysentery,  measles,  Pasteur 
rabies  treatment,  whooping-cough,  and  puerperal  septicemia. 

In  chronic  tuberculosis  mild  grades  of  neuritis  are  frequent,  and 
severe  polyneuritis  is  occasionally  met  with  in  the  marantic  type. 
Sj'philis  rarely  causes  a  polyneuritis,  but  it  is  known.  ^Malaria  is 
also  a  rare  cause.    Leprosy  causes  a  specialized  form. 

PLEXUS  PALSIES. 

In  peace  times  plexus  palsies  were  rare.  They  occurred  more  often 
in  the  upper  extremity.  Brachial  plexus  palsy,  as  Erb's  birth  palsy, 
is  the  type.  Lumbar  plexus  palsies  rarel\'  occur  alone  uncomplicated 
by  cord  lesions,  since  they  are  usually  produced  by  compression, 
resulting  from  tumor,  fracture.  Pott's  disease,  etc.  Sacral  plexus 
palsies,  however,  are  not  infrequent.  They  make  up  the  classical 
Cauda  equina  lesions,  arising  from  the  pressure  of  a  tumor,  from  hemor- 
rhage, fracture  of  the  sacrum,  bony  disease  of  the  lumbar  vertebrae, 
pelvic  tumor,  abscess,  etc.  War  injuries  have  caused  many  plexus 
palsies. 

Brachial  Plexus  Palsies. — These  most  frequently  arise  from  pulling 
or  wrenching  of  the  arms  from  accidents.  Dislocation  of  the  shoulder- 
joint  can  cause  a  plexus  injury.  Cervdcal  rib  is  a  rare  cause,  aneurism 
of  the  subclavian  also.  Gunshot  wounds  occasion  them.  (See  Fig. 
225.) 

The  plexus  is  made  up  of  the  lower  four  cervical  (fifth,  sixth,  seventh 
and  eighth)  and  the  upper  dorsal  roots.  Extraspinal  as  well  as  intra- 
spinal lesions  go  to  make  up  the  symptom  picture.  This  picture  is 
rarely  complete,  but  as  the  fibers  making  up  the  plexus  have  a  fairly 

1  Archanibault :    Ciliarj'  Ganglion  Involvements,  Jour.  Nerv.  and  Meut.  Dis.,  1917. 


410 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


definite  arrangement  so  far  as  muscular  distribution  is  concerned,  a 
study  of  the  muscles  involved  gives  a  key  to  the  roots  injured. 

The  fifth  and  sixth  cervical  roots  contain  the  fibers  going  to  the 
deltoid,  biceps,  brachialis  anticus,  supinator  longus,  supra-  and  infra- 
scapularis,  the  clavicular  fascia  of  the  pectoralis  major,  and  the 
serratus  magnus.  The  seventh  cervical  root  contains  the  fibers  dis- 
tributed to  the  triceps,  the  sternal  portion  of  the  pectoralis  major, 
to  the  dorsalis  magnus,  to  the  extensors  of  the  wrist,  and  also  some 
filaments  to  the  median  and  ulnar  nerves. 

The  eighth  cervical  and  the  first  dorsal  form  the  brachial,  internal 
cutaneous,  ulnar,  median  and  parts  of  the  radial  nerves  with  their 
muscular  innervations,  as  seen  in  the  illustrations.  (See  Figs.  22  to 
30,  also  Fig.  215.) 

The  clinical  pictures  seen  are  those  of  a  total  brachial  plexus  palsy, 
a  superior  and  an  inferior  type. 


Fig.  225. 


-Sensory  disturbances  in  seven  cases  of  gunshot  wounds  of  the  brachial 
plexus  in  the  "World  War."     (Edinger.) 


Total  Brachial  Palsy. — Here  all  of  the  muscles  of  the  hand  are  par- 
alyzed, those  of  the  forearm,  the  arm  and  the  shoulder.  The  arm 
hangs  limp  like  a  flail.  In  the  early  stages  the  skin  is  cyanosed,  there 
are  severe  pains  (in  the  accident  cases),  and  a  suppression  of  the 
secretion  of  sweat.  Atrophy  comes  on  quickly  and  is  extreme,  with 
loss  of  electrical  responses.    Trophic  disturbances  are  usually  present. 

Sensory  disturbances  are  present.  There  is  loss  of  all  forms  of 
sensibility,  including  the  sense  of  position  in  the  hands  and  the  fore- 
arm, excepting  the  upper  part  of  the  shoulder.  Civ,  and  a  triangular 
zone  in  the  inner  side  of  the  arm  Dii  Dili. 

Furthermore,  since  cervical  sympathetic  fibers  are  represented  in 
the  communicating  branch  of  the  first  dorsal,  one  finds  oculopupil- 
lary  signs,  first  demonstrated  by  IMadame  Dejerine-Klumpke  (1885). 


PLEXUS  PALSIES 


411 


They  consist  of  a  contraction  of  the  pupil  on  the  paralyzed  side,  a 
diminution  in  the  size  of  the  palpebral  fissure  (pseudoptosis)  and  a 
retraction  of  the  globe  of  the  eye  within  the  orbit  (enophthalmus) . 


Ficjs.  226,  227  and  228. — Distribution  of  radicular  sensorj-  disturbances  in  case  of 
traumatic  brachial  palsy  of  the  left  side.  There  was  total  paralysis,  atrophy  and  loss  of 
tendon  reflexes,  edema  and  cyanosis  of  left  arm.  Abolition  of  all  types  of  sensibility 
of  the  distribution  of  the  Cc  to  Di.  Myosis  and  enophthalmus  in  left  eye.  (Dejerine- 
Klumpke.) 


im.  2:^9.— Erb's  birth  pa 


(Frauenthal.) 


412 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


Furthermore,  lesions  here  frequently  cause  changes  in  the  sweat 
secretion.  These  are  sharply  delimited  to  the  head,  neck,  face,  to  the 
level  of  the  third  rib  in  front  and  the  spine  of  the  scapula  behind, 
including  the  upper  extremity,  all  confined  to  the  side  injured. 

Inferior  Root  Type. — This  has  also  been  designated  the  Klumpke 
pais}'.  It  follows  a  less  complete  lesion  of  the  plexus,  involving  the 
eighth  cervical  and  the  first  dorsal.  It  may  result  from  direct  injury  of 
strain,  gunshot  wound,  birth  palsy  (P^rb's  palsy),  from  cervical  rib,  or 
from  sj'philitic  deposits.  The  small  muscles  of  the  hand  are  involved. 
The  atrophy  is  rapid.  There  is  edema  of  the  skin,  cyanosis,  perhaps 
trophic  changes  in  the  nails.  The  anesthesia  is  less  extensive,  being 
limited  to  the  distribution  of  the  radial  and  internal  cutaneous  nerves. 
The  oculopupillary  signs  are  evident. 


CVD 


C  VI.  VD 


Figs.  230  and  231. — ^Topography  of  tactile,  pain  and  thermal  sensory  disturbances  in 
a  superior  brachial  palsy  type  due  to  injury  to  the  shoulder.  The  fifth,  sixth  and 
seventh  cer\dcal  roots  are  involved.     (Dejerine.) 


Superior  Brachial  Plexus  Palsy. — Here  the  upper  branches,  fifth  and 
sixth  cervical,  are  involved.  Duchenne  described  the  earlier  cases 
minutely.  The  sensory  disturbances  are  more  extensive  than  those 
observed  in  the  inferior  type.  The  pupillary  symptoms  are  absent. 
The  chief  muscles  involved  are  the  deltoid  (circumflex),  biceps  and 
brachialis  (musculocutaneous,  supinator  longus,  .musculospiral) . 
Flexion  of  the  arm  is  impossible.  Paralysis  of  the  clavicular  head  of 
the  pectoralis  major,  supra-  and  infraspinati,  subscapularis  and  teres 
major  occurs.  Lesions  high  up  may  implicate  the  serratus,  rhomboids 
and  levator  anguli  scapuli.  There  results  scapular  atrophy,  displace- 
ments of  the  shoulder  blade  (winged  scapukie)  and  loss  of  compensatory 
shoulder  movements  for  the  deltoid  and  serratus  loss. 


PLEXUS  PALSIES 


413 


Mixed  Types. — These  are  more  frequent.  In  the  recent  great 
World  War  a  vast  medley  of  plexus  palsies  have  been    observed.^ 

The  more  common  form  of  so-called  Erb's  palsy  belongs  here.  !Many 
of  these  are  bilateral,  the  two  arms,  however,  being  dissimilarly 
involved.  The  study  of  the  muscles  involved  in  the  atrophy,  the 
electrical  changes,  the  sensory  changes  to  light  touch,  heat,  cold,  pain, 
bony  sensibility,  and  to  position,  the  presence  of  trophic  disturbances 
(protopathic  system  changes),  and  the  oculopupillary  phenomena 
determine  the  roots  involved.    (See  Figs.  2.3  to  31.) 


Fig.   2.32. — -Double  cer\-ical  rib,  causing  mixed  type  of  brachial  palsy.    (Goodhart.) 

There  are  a  number  of  bony  conditions  which  can  determine  brachial 
plexus  palsies.  Tuberculous,  syphilitic,  carcinomatous,  sarcomatous, 
arthritic  infiltrations  about  the  vertebral  canal  impinging  upon  the 
cords  of  the  plexus  can  give  rise  to  palsies  of  this  type.  Similar  changes 
not  infrequently  also  cause  pictures  which  are  often  confused  with 
brachial  neuritis.  Some  of  the  severe  arm  pains  with  mild  atrophies 
are  forms  of  brachial  radiculitis  (q.  v.),  either  inflammatory  or  trau- 
matic in  origin.  In  the  course  of  a  rheumatoid  arthritis  one  not 
infrequently  encounters  these  radicular  disturbances  which  are 
undoubtedlv  referable  to  a  vertebral  arthritis. 


'Tinel:  Nerve  Injuries,  M.  Wood  &  Co.,  1917.  .Vthanasio  Benisty,  D.  Appleton, 
1917,  are  two  invaluable  works  on  war  injuries  of  the  peripheral  nerves.  Also  see  Purves 
Stewart  on  Peripheral  Nerve  Injuries,  1919,  2d  ed. 


414  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Cervical  Rib. — Symptoms  usually  appear  in  early  adult  life  and 
are  due  in  most  eases  to  pressure  upon  the  lowest  trunk  or  nerve 
entering  into  the  plexus.  In  many  cases  they  consist  of  wasting  of  the 
intrinsic  muscles  of  the  hand,  most  marked  and  starting  in  those  of 
the  thenar  eminence;  if  of  long  standing  the  reaction  of  degeneration 
may  supervene.  In  other  cases  the  principle  complaint  is  pain  along 
the  ulnar  border  of  the  forearm  and  hand,  or  a  general  weakness  or 
heaviness  of  the  whole  limb  noticed  at  the  end  of  the  day.  Sensory 
changes  are  unusual  and  when  present  rarely  exceed  epicritic  loss. 

This  condition  should  be  thought  of  in  all  cases  of  "brachial  neu- 
ralgia," or  of  wasting  of  the  thenar  muscles.  The  ribs  can  usually 
be  felt  but  occasionally  can  only  be  demonstrated  by  .r-ray  exami- 
nation. 

Although  a  cervical  lib  be  present  it  does  not  necessarily  mean  that 
it  is  the  cause  of  the  symptoms.  Several  cases  have  been  observed 
in  which  a  cervical  rib  was  removed  from  a  patient  suffering  from 
syringomyelia  to  which  the  symptoms  were  due.  Thomas  Murphy 
has  recorded  a  case  in  which  the  symptoms  were  due  to  the  pressure 
of  the  first  rib.  A  similar  case  is  reported  by  Sherren.  (See  p.  413, 
Fig.  232.) 

Treatment  consists  in  removal  of  the  rib  together  with  the  periosteum 
covering  it,  followed  by  careful  after-treatment. 

Course. — General  statements  regarding  the  course  are  misleading. 
The  majority  of  Erb's  palsies  due  to  obstetrical  accidents  recover, 
especially  when  only  a  few  roots  of  the  plexus  are  involved.  Total 
separation  from  the  cord,  as  in  severe  dislocations,  falls,  etc.;  with 
complete  plexus  palsy,  usually  means  an  incurable  affection,  not 
remediable  by  surgery. 

The  underlying  etiological  factor  determines  the  course  in  a  number 
of  others.  Palsies  caused  by  cervical  rib,  or  subclavian  aneurism  do 
not  get  well  spontaneously,  nor  does  iodid  help  them.  Proper  surgery, 
as  indicated  by  the  .r-ray  findings,  may  be  of  service. 

Diagnosis. — The  rarer  nuclear  and  neuritic  atrophies  and  dystrophies 
have  been  mistaken  for  these  radicular  palsies,  but  the  study  of  the 
sensory  changes  should  at  once  establish  the  differences.  Spinal 
gliosis  of  the  upper  arm  type  will  show  dissociation  symptoms,  indicat- 
ing the  intraspinal  nature  of  the  lesion.  Such  a  dissociation  is  a 
retention  of  tactile  sensibility  with  loss  of  pain  and  thermal  sensi- 
bility. Various  neuritides,  lead,  diabetes,  have  a  peripheral  rather 
than  a  root  distribution.  Certain  exceptional  cases  of  tabes,  with 
atrophy,  can  be  separated,  but  may  require  a  cerebrospinal  fluid 
examination.  Syphilitic  cervical  spondylitis  cases  at  times  show  these 
symptoms. 

Treatment. — There  is  little  treatment  for  the  severe,  tearing  lesions 
of  the  plexus.  Whether  they  can  be  looped  up  with  other  roots  to 
advantage  has  to  be  answered  in  the  future. 

The  cause  of  the  palsies,  other  than  tearing,  can  often  be  removed. 
Thus  surgical  treatment  of  neck  glands,  subclavian  aneurisms,  cervical 


PLEXUS  PALSIES  415 

rib,  spondylitis,  and  osteo-arthritis  is  efficacious.  The  treatment  of  a 
cervicodorsal  Pott's  by  proper  fixation,  and  the  antisyphiUtic  treat- 
ment of  a  luetic  spondylitis  are  satisfactory.  Rare  cases,  often  bilat- 
eral, due  to  profound  secondary  anemia,  are  often  very  refractory. 
True  radicular  neuritis  is  also  stubborn. 

The  pains  are  best  relieved  by  analgesics;  antipyrin,  aspirin,  pyram- 
idon,  or  other  combinations  are  valuable.  Heat  is  usually  not  well 
borne,  and  massage  is  contra-indicated  in  the  early  stages.  Osteopathic 
manipulation  is  a  dangerous  procedure  in  the  early  stages.  In  certain 
osteo-arthritic  cases  it  proves  valuable  later. 

Simple  counter-irritation  over  the  site  of  the  plexus,  above  the 
clavicle,  is  invaluable  in  many  mild  neuritic  attacks;  while  for  the 
severer  attacks  high-frequency  currents,  violent  light  therapy,  Leduc 
current  at  times  cause  great  relief. 

Dietetic  and  general  management  in  the  neuritic  cases  is  not  to  be 
overlooked.  They  need  fats  in  ample  proportion.  This  is  best  supplied 
through  taking  large  quantities  of  milk. 

Lumbosacral  Syndromes. — The  lumbosacral  plexus  constituents  may 
be  injured  not  only  in  their  pelvic  course,  as  is  seen  in  the  typical 
parturient  sjTidrome,  but  as  a  result  of  war  missiles,  and  particularly 
in  the  great  ravages  of  1914-1918  many  injuries  to  the  roots  in  their 
long  intraspinal  course  have  been  observed.  (See  Tinel,  Athanassio 
Benisty,  Roussy  et  Lhermitte) .  Here  in  the  cauda  equina  they  form  a 
long  compact  bundle  which  may  be  injured  in  a  great  variety  of 
ways.  Spinal  fractures,  bullet  and  shell  splinters,  hematomyelias  cause 
injuries  of  the  several  roots  and  produce  various  syndromes,  which 
do  not  have  to  be  differentiated  into  trunk  and  root  types.  The 
peripheral  nerves  contain  fibers  from  different  roots,  hence  root  lesions 
will  give  rise  to  a  variety  of  dissociated  peripheral  palsies.  The  palsies 
are  apt  to  be  incomplete,  unless  the  lesion  is  extensive,  since  the  nerves 
usually  are  supplied  from  several  roots.  The  sensory  changes  appear 
as  radicular  strips  of  anesthesia  running  along  the  axis  of  the  limbs, 
when  the  sacral  and  lower  lumbar  roots  are  involved,  the  anesthesise 
are  distributed  obliquely  from  injuries  of  the  upper  lumbar  roots  and 
transversely  from  dorsal  root  lesions. 

Lumber  Root  Sjmdromes. — The  first  and  second  lumbar  roots  when 
injured  produce  slight  lesions  only  as  a  rule.  They  supply  few  fibers 
to  the  quadratus  lumborum,  psoas,  and  the  lower  parts  of  the  trans- 
versus  and  anterior  muscles  of  the  thigh.  These  muscles  are  only 
weakened.  The  sensory  disturbances  are  found  on  the  outer  surface 
of  the  buttock  and  the  base  of  the  thigh  spreading  over  the  upper  two- 
thirds  of  the  inner  aspect  of  the  groin  to  the  inguinal  fold. 

The  fibers  of  the  third,  fourth  and  fifth  lumbar  when  involved  on 
the  contrary  produce  widespread  disturbances.  Here  all  of  the  ante- 
rior and  internal  thigh  muscles  are  implicated,  also  the  triceps,  pec- 
tineus  and  sartorius  and  if  the  obturator  is  caught  in  the  injury  the 
adductors  and  gracilis  are  paralyzed. 
The  sensory  loss  extends  as  an  oblique  band  from  the  middle  of  the 


416  AFFECTIONS  OF   THE  PERIPHERAL  NERVES 

thigh  above  downward,  mternal  surface  to  the  middle  of  the  leg  and 
down  the  inner  border  to  and  including  the  big  toe.  The  patellar 
reflex  is  lost.  There  is  marked  atrophy  of  the  thigh  which  seems 
shrunken  in  just  above  the  knee  as  if  squeezed.  A  number  of  muscles 
are  involved. 


Figs.  233  and  234. — Distribution  of  anesthesia  in  sacral  roots,  sciatic  chiefly. 

Sacral  Root  Syndromes. — ^The  sciatic  distribution  corresponds  to 
the  fibers  of  the  first  and  second  sacral  roots,  excepting  the  tibialis 
anticus  which  is  supplied  from  the  fourth  and  fifth  lumbar,  which  also 
supplies  a  number  of  the  muscles  of  the  leg  and  the  foot,  but  in  the 
leg  the  gastrocnemius,  outer  head,  and  peronei  are  supplied  by  the 
sacral  fibers  and  in  the  foot  the  adductor  hallucis  and  the  interossei 
are  exclusively  sacral  distributions.  Extremely  complicated  pictures 
therefore  result  from  the  cauda  lesions  which  have  to  be  worked  out 
on  the  basis  of  the  charts  (Figs.  2.33  and  234).  The  sensory  distribu- 
tions of  the  first  and  second  sacral  roots  occupy  a  wide  tract  from  the 


PERIPHERAL  PALSIES 


417 


buttock  to  the  outer  posterior  aspect  of  the  knee,  where  it  takes  a  further 
obhque  course  to  the  front  of  the  leg  and  dorsum  of  the  foot.  The 
Achilles  reflex  is  lost.  If  the  fourth  or  fifth  sacral  roots  are  included 
or  alone  injured,  incontinence  of  urine  and  feces,  with  failure  to  feel 
the  same,  are  added  as  isolated  symptoms. 

Pains  are  usually  verj-  marked  and  persistent.  They  occur  in 
paroxysms,  and  are  usually  extreme.  The  chief  path  is  that  of  the 
sciatic,  but  they  may  be  located  almost  an;v'w^here  about  the  thigh. 
They  may  be  bilateral  or  unilateral  and  shift  considerably. 

Treatment. — This  must  vary  largely  upon  the  tA^pe  of  lesion. 


Fig.  235. — Sensorj^  disturbances  in  five  cases  of  sacral  plexus  injury  due  to  gunshot 
wounds.  In  the  first  case  there  was  a  total  palsy  in  the  peroneus  and  tibialis  nerve 
distribution;  in  the  second  the  peroneus  alone  was  involved;  in  the  third  the  peroneus, 
tibialis,  ilio-inguinalis  and  obturator;  in  the  fourth  the  peroneus  and  tibialis;  in  the 
fifth  the  crural  nerve  distribution  alone  suffered.  (Edinger.)  Compare  Figs.  252, 
253,  254. 

PERIPHERAL  PALSIES. 

Numerous  forms  of  peripheral  nerve  palsy  are  found,  varying 
according  to  the  particular  fibers  involved.  The  branches  that  arise 
from  the  brachial  plexus  have  already  been  described.  Any  of  these 
may  be  injured  or  inflamed  with  a  resulting  partial  or  complete  palsy. 
The  various  neuritides  will  not  be  reexamined  and  the  following  sections 
will  deal  with  those  peripheral  palsies  largely  due  to  defect  or  injury. 

Defects,  Congenital  and  Acquired. — IMuscular  defects  in  the  upper 
and  lower  extremities  are  by  no  means  uncommon.  They  have 
been  described  for  some  centuries.  Their  precise  formulation  began 
with  Ziemmsen's  work  in  1857.  The  later  literature  is  summed  up  by 
Bing.i  Lorenz,2  and  by  Hirschfeld.^  The  causes  for  these  muscular 
defects  are  extremely  complicated. 

Their  occurrence  is  very  manifold.  Bing  was  the  first  to  collect  the 
entire  group,  and  Abromeit,  in  1909,  completed  the  study  which  shows 
that  an  absence  of  any  muscle  of  the  body  may  be  expected.  One  in 
10,000  shows  such  defects. 

In  the  majority  of  the  cases  the  defect  is  unilateral,  and  they  are 
more  frequent  in  men. 

Al)romeit  collected  reports  of  180  cases  of  defect  of  the  pectoral 
nniscles,  the  sternocostal  ])()rti()n  being  most  att'ected.'    This  seems 

'  Virchows  Arch.,  1902.  p.  170.  ^  Die  Muskel  Krkrankungcn,  Notlinajiel,  1904. 

'  Kongenitale  Muskcldefektc,  Lewandowsky's  Handbuch,  1911. 
*  See  Jjcwandowsky,  ii,  2.5.3. 
27 


418  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

the  most  frequent  of  such  anomaUes.  The  trapezius  was  absent 
in  33  cases,  the  serratus  magnus  in  22,  quadratus  26,  omohyoid  16, 
semimembranosis  7,  rectus  muscles  11,  pyramidahs,  often  found 
absent  postmortem,  diaphragm  3  times,  deltoid  5,  sternocleidomastoid 
8,  etc.    The  smaller  muscles  of  the  hand  are  not  infrequently  absent. 

Certain  combinations  of  muscles  may  be  absent,  constituting  a 
group  complex.  Atrophy  or  loss  of  other  parts  may  be  combined  and 
gross  anomalies  of  structure  may  be  combined  with  marked  muscular 
defects;  various  monsters,  syndactylia,  phocomelus,  etc.  The  hered- 
itary nature  of  sjTidactylous  anomalies  has  given  occasion  for  some 
important  studies. 

The  symptoms  involve  the  physiological  loss  of  the  special  muscle 
function  or  the  resultant  of  function  from  the  muscles  present  in  a 
combination. 

These  patients  rarely  show  as  marked  loss  of  efficiency,  especially 
with  one-sided  defect,  as  do  those  who  acquire  a  defect.  Acquired 
defects  are  usually  bilateral.  They  usually  involve  a  group  of  muscles; 
the  disease  rarely  causes  a  total  loss  of  muscle  substance,  and  anomalies 
of  accompanying  structures  are  missing. 

Fibrillary  twitchings  are  often  diagnostic  of  the  acquired  muscular 
defects. 

The  prognosis  and  treatment  require  little  discussion.  Gymnastic 
exercise  of  the  residual  muscular  combinations  directed  to  the  acquisi- 
tion of  greater  efficiency  by  skilful  application  of  mechanical  principles 
is  always  an  individual  goal  that  cannot  be  more  than  mentioned 
here. 

Peripheral  Palsies  Due  to  War  Injuries. — This  section  discusses 
peripheral  palsies  due  to  nerve  injuries  rather  than  to  those  due  to 
general  neuritic  processes.  An  injury  may  affect  a  nerve  either  in  its 
continuity,  or  in  one  of  its  terminal  branches,  thus  giving  rise  to 
different  symptoms,  and  requiring  a  diverse  therapy. 

Injury  in  continuity  may  result  from  penetrating  wounds,  traction, 
pressure,  blows  or  by  operation.  They  may  give  rise  to  complete  or 
incomplete  division . 

Sjrmptoms. — The  recent  World  War  has  amplified  the  existing 
mass  of  information  relative  to  peripheral  nerve  injuries.  The  large 
mass  of  observations  cannot  even  be  summarized  here.  A  score  of 
monographs  filled  with  statistical,  clinical,  pathological  and  surgical 
data  have  appeared  (1915-1919)  some  of  which  are  referred  to  in  the 
footnotes  here.^  These  are  but  forerunners  of  a  probable  host  which 
will  come  from  the  laboratories  and  clinical  records  of  the  war.  Tinel 
in  the  first  of  the  important  monographic  researches  of  the  war  sum- 
marizes the  statistical  incidence  of  the  main  nerve  injuries  as  occurring 
amx^ng  the  French  soldiers.    In  a  series  of  639  cases  the  relative  fre- 

1  Tinel,  I.e.;  Athanassio  Benisty,  I.e.;  Frazier,  I.e.;  Elsberg,  I.e.;  Revue  Neurologique ; 
Neurclogie  de  Guerre;  Review  of  Neurology  and Psyehiatry ;  War  Neurology;  Burrow: 
1000  Capes  of  Peripheral  Nerve  Injury.  Dustin:  Ambulance  de  "I'Ocean,"  December, 
1917,  July,  1918.     Important  for  Study  of  Fasciculation  of  Nerve  Fibers. 


PERIPHERAL  PALSIES  419 

quency  of  involvement  was  as  follows:  Upper  limb:  408,  distributed 
as  musculospiral  146;  ulnar  84;  median  67;  musculocutaneous  11;  cir- 
cumflex 25;  brachial  plexus  27;  combined  lesions  48.  Lower  limb:  231; 
anterior  crural  11;  long  saphenous  14;  sciatic  (trunk)  78;  internal 
popliteal  29,  external  popliteal  37;  posterior  tibial  18;  muscalocu- 
taneous  7;  anterior  tibial  4;  short  saphenous  9;  obturator  1;  external 
cutaneous  3;  ilioinguinal  1;  lumbosacral  plexus  19.  Burrows'  for 
Erglish  army  injuries  in  a  series  of  1000  cases,  showed  the  following 
relative  frequency:  Ulnar  527;  median  242;  musculospiral  204; 
radial  20;  posterior  interosseus  19;  musculocutaneous  24;  internal 
cutaneous  26;  brachial  plexus,  root  17;  outer  cord  17;  inner  cord  19; 
posterior  cord  24;  cervical  roots  12;  circumflex  2;  facial  2;  spinal 
accessory  2.  Lower  limbs:  sciatic  121;  external  popliteal  97;  internal 
popliteal  8;  posterior  tibial  8;  anterior  tibial  9;  musculocutaneous  2; 
external  saphenous  1;  internal  saphenous  2;  miscellany  29. 

In  view  of  the  recent  investigations  of  Head  and  Sherren  as  carried 
into  the  field  of  war  neurology  by  Dejerine  and  his  pupils,  it  seems 
possible  to  separate  the  peripheral  nerve  injury  s;yTidromes  into  four: 
(1)  The  interruption  syndrome;  (2)  Compression  syndrome;  (3)  Irri- 
tation syndrome;  and  (4)  Restoration  syndrome. 

(1)  The  interruption  syndrome,  when  complete,  causes  abolition  of  all 
of  the  functions.  Paralysis  of  the  involved  muscles  is  complete,  tonus 
is  lost,  the  limbs  assume  attitudes  caused  by  the  antagonist  predomi- 
nance. There  is  no  pain  on  pressure  of  the  muscular  masses  and  there 
is  muscle  anesthesia.  To  these  may  be  added :  Abolition  of  the  tendon, 
osseous  and  cutaneous  reflexes,  exaggeration  of  the  myotatic  reaction, 
amyotrophies,  deformities,  as  dorsal  tumor  of  the  tarsus  or  carpus, 
modified  electrical  reactions.  The  sensory  changes  need  some  elabora- 
tion and  the  principles  involved  applied  to  the  other  s;vTidromes  to  be 
described  later.  In  complete  division  Sherren  and  Head  have  shown 
that  the  sensory  peripheral  fibers  may  be  divided  into  three  systems: 

1.  Those  which  subserve  deep  sensibility  and  conduct  the  impulses 
produced  by  pressure.  The  fibers  of  this  system  run  mainly  with 
the  motor  nerves  and  are  not  destroyed  by  division  of  all  the  sensory 
nerves  of  the  skin. 

In  a  part  innervated  only  by  this  system  gradual  increase  in  pressure 
can  be  recognized  and  pain  experienced  when  such  pressure  becomes 
excessive.  The  patient  can  also  appreciate  the  extent  and  direction 
of  movements  produced  passively  in  all  the  joints  within  the  affected 
area. 

2.  Those  which  subserve  protopathic  sensibility.  This  system  of 
fibers  and  end-organs  responds  to  painful  cutaneous  stimuli  and  to 
the  extremes  of  heat  and  cold ;  it  also  endows  the  hairs  with  power  to 
react  the  painful  stimuli.  The  distribution  of  the  protopathic  fibers 
usually  overlaps  greatly  the  area  supplied  by  similar  fibers  from 
adjacent  nerves.  Head's  protopathic  fiber  system  undoubtedly  con- 
tains much  of  the  vegetative  fibers  innervating  the  skin  muscles,  vessels 
and  secretory  organs. 


420  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

3.  Those  which  subserve  epicritic  sensibility.  The  nerve  fibers  and 
end-organs  of  this  system  endow  the  part  with  the  power  of  responding 
to  hght  touch  with  a  well-locaUzed  sensation.  The  existence  of  this 
system  enables  one  to  discriminate  two  points  and  to  appreciate  the 
difference  between  cold  and  heat.  The  distribution  of  these  fibers  in 
large  peripheral  nerves,  such  as  the  median  and  ulnar,  has  very  little 
overlap  compared  with  the  greater  overlapping  of  the  protopathic 
supply. 

These  investigations  were  carried  further,  particularly  with  regard 
to  deep  sensibility  and  the  distribution  of  heat  and  cold  spots,  by 
Head  and  Rivers  after  voluntary  section  of  the  radial  and  external 
cutaneous  nerves  in  the  former's  arm.^ 

To  illustrate  these  changes  in  sensibility  after  division  of  a  mixed 
nerve  the  ulnar  is  an  excellent  example  (Fig.  237).  After  complete 
division  of  this  nerve  at  the  wrist,  if  no  tendons  have  been  divided 
at  the  same  time,  the  patient  is  able  to  appreciate  those  stimuli  com- 
monly called  tactile.  A  touch  with  anything  which  deforms  the  skin 
may  be  readily  appreciated  and  correctly  localized.  When  pricked 
with  a  pin  the  patient  knows  that  he  has  been  touched  but  fails  to 
perceive  the  sharpness  of  the  stimulus  (deep  sensibility).  But  if  ten- 
dons are  divided  at  the  same  time  or  the  section  involves  the  nerve 
above  the  point  at  which  its  muscular  branches  are  given  off,  deep 
touch  may  be  unperceived.  These  characteristics  are  of  the  utmost 
importance,  many  cases  of  nerve  injury  have  been  overlooked  from 
failure  to  recognize  these  facts. 

The  point  of  a  pin  and  all  temperatures  are  unappreciated  within 
an  area  which  varies  somewhat  in  each  case  (loss  of  protopathic 
sensibility).  Surrounding  this  area  and  corresponding  closely  to  the 
distribution  of  the  nerve  as  figured  in  Figs.  236  and  237  is  a  territory 
within  which  the  patient  is  unable  to  appreciate  light  touches  with 
cotton-wool  and  temperatures  between  about  22°  and  40°  C.  (minor 
degrees  of  temperature),  and  fails  to  discriminate  the  points  of  a 
pair  of  compasses  when  separated  to  many  times  the  distance  necessary 
over  the  corresponding  portion  of  the  sound  limb  or  the  unaffected 
portion  of  the  injured  one  (loss  of  epicritic  sensibility).  Within  this 
area  of  loss  of  sensibility  to  light  touch,  to  which  they  gave  the  name 
of  "intermediate  zone"  the  patient  is  able  to  appreciate  the  sharpness 
of  a  pin-prick  and  to  differentiate  temperatures  below  20°  C.  and 
above  45°  C,  naming  them  correctly. 

Division  of  a  peripheral  nerve  produces  a  well-defined  loss  of  epicritic 
sensibility,  a  smaller  loss  of  protopathic  sensibility  with,  as  a  rule,  ill- 
defined  limits.    In  many  cases  there  is  no  loss  of  deep  sensibility. 

Complete  division  of  certain  nerve  branches  produces  no  objective 
change  in  sensibility,  these  are  the  musculospiral  below  the  point  at 
which  its  external  cutaneous  branches  are  given  oft",  tlie  radial,  and 
certain  cervical  anterior  primary  divisions. 

In  incomplete  division  the  sensory  symptoms  may  be  nil.  The 
patient  is  conscious  of  an  area  altered  in  sensibility,  and  it  is  often 

'  Compare  Boring:  Quart.  Jour.  Exp.  Physiol.,  x,  1916,  p.  1. 


PERIPHERAL  PALSIES 


421 


possible  to  demonstrate  this  by  the  changed  sensibiHty  produced  at 
its  borders  when  a  piece  of  cotton-wool  or  the  point  of  a  needle  is 


Fig.  236. — To  illustrate  the  changes  in  sensibility  met  with  after  complete  di^^sion 
of  a  peripheral  nerve.  The  area  inclosed  Vjy  a  line  is  that  in  which  epicritic  sensibility 
is  lost.  The  shaded  area  is  that  of  loss  of  epicritic  and  protopathic  sensation.  The 
unshaded  portion  is  the  "intermediate  zone."     (Sherren.) 


Fig.  237. — Loss  of  sensibility  after  complete  division  of  ulnar  nerve.     (Sherren.) 


dragged  lightly  across  the  skin  from  sound  to  affected  portions  (line 
of  change).  If  the  area  of  changed  sensibility  is  well  marked,  response 
to  the  compass  test  will  be  defective. 


422  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

In  cases  of  greater  severity  the  loss  of  sensibility  to  cotton-wool 
may  be  absolute,  with  borders  as  well  defined  as  after  complete 
division. 

When  the  injury  is  more  severe,  impairment  or  loss  of  protopathic 
sensibility  results  apd  the  sensory  loss  may  resemble  exactly  that  seen 
after  complete  division. 

Sherren  has  shown  that,  contrary  to  the  usual  teaching,  the  motor 
loss  in  incomplete  division  is  not  more  than  the  sensory  loss.  Paralysis 
of  muscles  of  the  injured  nerve  distribution  may  result  after  eight  to 
fourteen  days.  The  usual  reaction  on  or  about  the  tenth  day  is  that 
the  muscles  do  not  respond  to  the  interrupted  current  but  do  react 
to  the  constant  current.  The  strength  of  the  current  needed  to  cause 
a  contraction  is  less  on  the  sound  side;  the  contraction  is  brisk  in 
comparison  to  the  R.  D.  and  polar  reversal  is  absent. 

Pain  is  a  frequent  after-result  in  incomplete  division;  there  may  also 
be  tender  and  glossy  skin  and  changes  in  the  nails. 

Stages  of  Recovery. — Following  complete  division,  in  from  six  to 
sixteen  weeks,  the  restoration  of  protopathic  sensibility  commences 
and  is  completed  in  from  four  to  twelve  months  after  suture  of  divided 
nerves.  Blisters  may  occur  early,  but  later  all  ulcers  heal;  blisters 
no  longer  appear.  Epicritic  touch  is  unchanged  throughout,  but  in 
from  twelve  to  fifteen  months  the  whole  area  is  sensitive  to  light  touch 
and  intermediate  degrees  of  temperature.  Improvement  in  the  power 
of  accurate  localization  is  the  third  stage,  and  is  tested  out  by  the 
compass.  The  motor  recovery  is  gradual,  the  electrical  reactions  of 
incomplete  division  first  appearing. 

It  seems  certain  that  no  regeneration  takes  place  in  the  periph- 
eral end  of  a  divided  nerve  without  union  with  the  central  nervous 
system. 

After  incomplete  division  of  a  mixed  nerve  the  loss  of  sensation  and 
motion  may  at  first  resemble  that  which  follows  complete  division, 
but  the  method  of  recovery  is  entirely  different. 

After  complete  division  of  a  nerve  and  suture,  sensibility  to  prick  is 
restored  before  the  commencement  of  recovery  of  sensibility  to  light 
touch.    Complete  sensory  recovery  often  occupies  several  years. 

But  after  incomplete  division  sensibility  to  light  touch  and  to  prick 
are  restored  together  and,  unless  nerve  fibers  have  been  anatomically 
divided  in  considerable  number,  the  power  of  appreciating  two  points 
(the  compass  test)  is  soon  regained.  This  is  an  extremely  important 
point,  for  upon  the  recovery  of  this  power  of  localizing  depends  the 
utility  of  the  part  for  fine  work.  It  is  important  to  recognize  that  in 
injuries  of  nerves  without  interruption  of  their  anatomical  continuity 
the  power  of  localization  returns  quickly,  unless  the  injury  has  been 
sufficient  to  cause  complete  division;  in  this  case  the  usual  three  stages 
are  present,  but  the  time  of  the  third  stage  is  much  shortened. 

Knowledge  of  this  method  of  sensory  recovery,  first  described  by 
Head  and  Sherren,  is  a  valuable  addition  to  our  powers  of  diagnosis. 
If  both  forms  of  sensibility  are  recovering  together,  it  is  certain  that 


PERIPHERAL  PALSIES 


423 


the  injury  has  not  been  severe  enough  to  produce  complete  interrup- 
tion of  conduction  in  the  injured  nerve,  with  degeneration  of  the  whole 
peripheral  end. 


Fig.  238. — End  of  first  stage  of  recovery.     (Sherren.) 


Fig. 


239. — Commencement  of  second  stage  of  recovery, 
area  regaining  sensibility  to  cotton- wool. 


The  dotted  line  marks  the 
(Sherren.) 


Motor  recovery  after  incomplete  division  follows  the  same  march 
as  after  complete  division;  the  muscles  nearest  the  seat  of  the  injury 
first  regain  voluntary  power  and  excitability'  to  the  interrupted  current. 

In  the  cases  in  which  the  reactions  tj^^ical  of  incomplete  division 


424 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


are  present,  voluntary  power  usually  returns  before  the  reestablish- 
ment  of  excitability  to  the  interrupted  current. 

Sensory  recovery  usually  begins  in  about  three  weeks,  and  is  com- 
plete in  about  six  months;  motor  recovery  in  from  a  few  days  to  ten 
weeks. 

These  times  of  motor  and  sensory  recovery  are  approximate  only, 
and  vary  with  the  severity  of  the  injury  and  its  distance  from  the 
periphery.  When  epicritic  sensibility  alone  is  lost  recovery  is  much 
more  rapid  than  when  both  forms  of  sensibility  are  affected.  When 
the  injury  affects  the  brachial  plexus,  considerably  longer  time  is 
necessary  for  the  commencement  and  progress  of  recovery. 


Fig.  240. — Showing  method  of  recovery  after  incomplete  division.     (Sherren.) 


To  sum  up :  After  incomplete  division  of  a  mixed  nerve,  both  forms 
of  sensibility  (epicritic  and  protopathic),  if  lost,  return  at  the  same 
time,  commencing  at  a  date  which  varies  with  the  distance  of  the 
injury  from  the  periphery  from  about  three  weeks  at  the  wrist  to  six 
months  in  the  plexus,  and  also  with  the  degree  of  the  injury.  Complete 
recovery,  as  a  rule,  rapidly  ensues.  Muscular  recovery  commences 
at  a  time  which  varies  in  the  same  way.  In  cases  in  which  the  muscles, 
though  paralyzed,  retain  their  irritability  to  the  interrupted  current, 
recovery  commences  in  three  or  four  weeks,  sometimes  earlier,  and 
soon  becomes  perfect.  This  degree  of  injury  is  seen  most  often  as  the 
result  of  compression  of  the  musculospiral  nerve,  producing  sleep, 
anesthetic  or  crutch  paralysis.  If  the  reactions  typical  of  incomplete 
division  are  present  a  much  longer  time  is  necessary. 

After  neurolysis,  or  when  the  nerve  has  been  relieved  from  any 
form  of  pressure,  recovery  follows  exactly  the  same  lines. 


PERIPHERAL  PALSIES  425 

Syndrome  of  Compression. — There  is  tenderness  of  the  muscles  and 
of  the  nerve  trunks,  and  in  general  many  of  the  signs  of  complete  inter- 
ruption. There  is  usually,  however,  retention  of  the  muscle  tonus,  the 
reaction  of  degeneration  is  less  pronounced,  slight  resistance  movements 
may  be  elicited,  deep  sensibility  is  less  involved.  The  causative  lesions 
are  about  the  nerve,  splinters,  callus,  fibroids,  bits  of  cloth,  aneurisms, 
abscesses,  hematomata,  etc. 

Surgical  intervention  is  called  for  to  free  the  nerve  and  remove  the 
object  pressing  upon  it.  After  this  the  nerve  is  isolated  and  placed 
amid  healthy  tissue,  preferably  muscle  planes  which  prevent  further 
pressure.  When  there  is  interstitial  sclerosis  it  is  useless  to  plough  it 
up  blindfold  (Dejerine).  Indeed,  it  is  better  to  leave  it  alone  entirely. 
In  treating  sclerosis  the  greatest  nicety  of  operative  technic  is  required. 

Syndrome  of  Irritation.— Dejerme  describes  this  as  characterized 
by  the  absence  of  complete  paralysis,  absence  of  the  dysesthetic  dis- 
turbances seen  in  complete  section,  already  described,  persistent 
hyperesthesia,  continuous  painful  phenomena  and  trophic  disturbances. 
These  latter  may  be  muscular,  osseous,  articular  or  cutaneous  (hyper- 
trichosis, etc.).  In  grave  irritation  the  pains  are  extremely  severe 
(Causalgia  of  Weir  Mitchell).  The  slightest  motion  is  painful  and 
wearing.  Local  applications  of  water,  moist  cloths  are  very  grateful. 
Sometimes  the  irritant  is  very  trifling,  bits  of  bone,  fragments  or 
splinters  of  foreign  bodies  hardly  touching  the  nerves.  These  cases 
tend  to  spontaneous  recovery. 

Syndrome  of  Restoration. — Sensory  symptoms  soon  appear.  These 
are  spontaneous  stabbing,  darting  pains  along  the  injured  nerve. 
These  continue  from  eight  to  fifteen  days  like  electric  shocks  and  tend 
to  subside  later.  There  is  pain  on  pressure  of  the  nerve  trunks. 
Shrinkmg  of  the  dysesthetic  areas  (see  Figs.  236-241).  Slow  return 
of  muscular  tissue,  of  muscle  tonus,  painful  muscles  disappear  and  a 
return  of  voluntary  contraction  takes  place.  The  electrical  reactions 
remain  as  before. 

Signs  of  restoration  contra-indicate  operation,  but  if  it  be  seen  that 
the  motor  loss  continues  after  the  sensory  restoration,  operation  is 
advisable. 

Treatment. — The  indications  in  cases  of  complete  division  in  all 
firearm  injuries  in  which  symptoms  of  repair  do  not  occur  is  to  operate 
whatever  may  be  the  lesion.  The  suigeon  should  freely  resect  all  the 
indurated  tissues  about  the  nerve  or  in  its  course  (fibroma,  neuro- 
fibroma, keloids),  and  suture  the  upper  and  lower  segments  end  to  end. 
In  large  gaps  a  graft  may  be  taken  from  a  functionally  less  useful 
sensory  nerve.  If  collateral  motor  branches  are  cut  during  the  opera- 
tion they  should  be  sutured  at  once.  One  should  insist  on  the  great 
tenderness  of  nerve  structures  and  insist  on  great  gentleness  in  manipu- 
lation on  the  part  of  the  surgeon. 

The  general  trend  in  recent  war  surger}'  of  the  nerves  is  to  greater 
and  greater  conservatism  in  operations  and  to  the  greater  consideration 
given  to  the  study  of  the  fasciculation  in  nerve  trunks,  with  careful  end- 


426  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

to-end  anastomoses  of  the  divided  fasciculi,  cut  well  away  from  neurom- 
atous stumps.  For  complete  discussion  of  these  factors  the  student  is 
referred  to  the  works  of  Dustin  and  others  referred  to. 

TineP  has  described  a  sign  which  he  considers  of  value  in  determining 
the  advisability  of  operation  on  peripheral  nerves,  particularly  of  the 
extremities.  The  value  of  this  is  affirmed  by  certain  observers  (Burrow, 
1.  c.)  denied  by  other  (Elsberg-).  Its  final  evaluation  remains  to  be  deter- 
mined. If  a  wounded  nerve  be  gently  percussed,  or  tapped,  or  gently 
rolled  under  the  finger,  distal  to  the  site  of  injury,  a  tingling  paresthesia 
is  apt  to  develop  over  its  surface  distribution  area.  As  regeneration 
along  a  nerve  trunk  takes  place  the  sign  is  elicitable  only  further  and 
further  distally  in  its  course.  Thus  in  early  ulnar  nerve  injury,  per- 
cussion of  the  ulnar  at  the  elbow  brings  out  the  tingling;  a  month  later 
if  recovery  is  taking  place,  pressure  on  the  trunk  in  the  forearm  may 
start  the  "pins  and  needles"  sensation;  still  later,  pressure  at  the  wrist 
may  elicit  the  sign.  The  test  can  be  made  quickly  and  hence  is  an 
advantage  in  busy  hospital  practice.  The  general  principle  is  to  delay 
nerve  suture,  so  long  as  the  tingle  may  be  brought  out  by  pressure  at 
progressively  more  and  more  distal  points. 

Injuries  to  Facial  Nerve. — Facial  paralysis  may  result  from  injury  to 
the  nerve  (a)  above  the  geniculate  ganglion,  (6)  between  the  ganglion 
and  the  point  at  which  the  chorda  tympani  is  given  off,  and  (c)  below 
this  point.  When  injured  at  (6)  taste  is  affected  over  the  anterior 
two-thirds  of  the  corresponding  side  of  the  tongue. 

Apart  from  the  so-called  "rheumatic"  affections  of  the  nerve  (Bell's 
palsy),  interference  with  the  function  of  the  nerve  in  the  middle  ear 
as  a  result  of  disease  or  operation  is  the  usual  cause.  The  nerve  may 
suffer  in  fractures  of  the  skull  primarily,  or  more  often  from  involve- 
ment in  callus.  Outside  the  skull  it  may  be  injured  during  operations 
in  the  parotid  region  or  in  the  removal  of  tuberculous  glands,  or 
from  forceps  pressure  during  childbirth;  in  most  of  these  cases  the 
"division"  is  incomplete  and  spontaneous  recovery  ensues. 

In  cases  of  incomplete  division  the  usual  treatment  directed  toward 
maintaining  the  nutrition  of  the  paralyzed  muscles  must  be  adopted. 
When  due  to  middle-ear  disease  it  is  an  indication  for  the  complete 
mastoid  operation. 

When  the  reaction  of  degeneration  is  present,  showing  that  com- 
plete division,  either  anatomical  or  physiological,  has  occurred,  the 
treatment  to  be  adopted  depends  on  the  cause.  If  it  follows  a  radical 
mastoid  operation,  the  sooner  operation  is  carried  out  after  the  wound 
has  healed  the  better;  in  cases  of  Bell's  palsy  it  is  justifiable  to  wait 
for  six  months.  If  the  nerve  is  divided  during  the  course  of  a  mastoid 
operation,  the  ends  should  be  adjusted  in  the  aqueduct,  when  restora- 
tion of  function  may  be  expected  unless  neuritis  is  set  up  as  the  result 
of  sepsis.     If  discovered  after  the  operation  the  electrical  reactions 

1  Presse  m6d.,  October  7,  1915,  p.  588. 

*  Transactions  American  Neurological  Association,  Journal  Nervous  and  Mental  Dis- 
eases, 1919. 


PERIPHERAL  PALSIES  427 

hould  be  tested  at  the  end  of  a  fortnight;  if  the  reaction  of  degenera- 
tion is  present,  the  wound  should  be  opened  up  and  an  attempt  made 
to  adjust  the  ends.  If  this  fails,  nerve  anastomosis  must  be  under- 
taken. It  must  be  remembered  that  the  injury  during  mastoid  opera- 
tion is  in  most  cases  incomplete,  and  that  spontaneous  recovery  follows 
the  usual  non-operative  treatment. 

Spinal  Accessory  Nerve. — ^The  external  or  spinal  portion  of  this 
nerve  is  not  infrequently  divided  during  the  course  of  operations  upon 
the  neck,  particularly  during  the  removal  of  tuberculous  glands.  In 
many  of  these  cases  the  branches  of  the  cervical  nerves  to  the  trapezius 
are  affected  at  the  same  time,  producing  its  complete  paralysis.  The 
extent  of  supply  of  the  spinal  accessory  and  the  cervical  nerves  to 
the  trapezius  varies;  as  a  rule  the  upper  portion  of  the  trapezius  is 
paralyzed  by  division  of  the  spinal  accessory  alone. 

The  Long  Thoracic  Nerve  (Nerve  of  Bell). — This  nerve  is  most 
often  injured  in  males  between  the  ages  of  twenty-five  and  forty, 
usually  on  the  right  side.  The  injury  is  generally  caused  by  prolonged 
pressure  in  the  supraclavicular  region,  it  occasionally  follows  violent 
muscular  efforts  and  direct  violence  applied  to  the  shoulder. 

Paralysis  of  the  serratus  ma  gnus  rarely  occurs  alone;  it  is  usually 
combined  with  paralysis  of  the  lower  trapezius. 

The  Circumflex  Nerve. — Injury  to  the  circumflex  nerve  is  by  no 
means  so  common  as  is  usually  supposed.  It  has  been  said  to  follow 
direct  blows  on  the  point  of  the  shoulder,  but  in  most  cases  the  injury 
is  to  the  anterior  primary  division  of  the  fifth  cervical  nerve,  and  careful 
examination  will  show  that  the  spinati  also  are  affected.  In  other  cases 
the  wasting  of  the  deltoid  in  common  with  the  muscles  around  the  joint 
on  which  the  circumflex  injury  was  diagnosed  has  been  found  to  be  the 
result  of  a  traumatic  arthritis. 

Carefal  examination  is  necessary  before  coming  to  a  decision  with 
regard  to  treatment;  testing  must  be  carried  out  for  all  forms  of  sen- 
sibility. If  there  is  no  loss  of  sensibility,  and  there  is  paralysis  of 
the  deltoid  with  the  reaction  of  degeneration,  it  is  extremely  improbable 
that  the  circumflex  nerve  is  injured.  If  the  signs  are  those  of  complete 
section  of  the  nerve,  the  age  of  the  patient  and  his  occupation  must  be 
taken  into  consideration;  in  some  cases  operation  can  be  avoided  by 
training  the  neighboring  muscles  to  take  the  place  of  the  deltoid. 

Ulnar  Nerve. — ^This  nerve  is  frequently  wounded  in  warfare,  327 
in  1000  of  Burrow's  series,  and  by  dislocation  or  fractures  of  the 
humerus,  and  at  the  elbow-joint.    It  is  frequently  wounded  at  the  wrist. 

If  the  injury  be  above  the  elbow  flexion  of  the  hand  becomes  difficult. 
The  little  finger  cannot  be  moved  and  the  middle  and  ring  fingers 
cannot  be  flexed  in  the  last  joint.  The  basal  phalanges  of  all  the 
fingers  cannot  be  flexed.    The  fingers  cannot  be  abducted  or  adducted. 

Injuries  lower  down  in  the  forearm  cause  only  a  loss  of  power  of  the 
interossei  and  muscles  of  the  thumb.    Main  en  griff e  develops. 

There  is  usually  a  definite  deficiency  in  the  prehensile  function  of  the 
thumb  and  first  finger.    This  may  be  used  as  an  indication  and  gauge 


428  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

of  ulnar  paralysis,  including  its  influence  in  causing  disability  in  the 
occupational  sense.  In  the  detection  of  a  weak  prehensile  function 
signifying  paresis  or  paralysis  of  the  ulnar  nerve,  the  patient  may  be 
made  to  grasp  some  thin  object,  such  as  a  folded  newspaper,  first 
between  the  thumb  and  forefinger  of  the  normal  hand,  then  between 
the  tliumb  and  forefinger  of  the  affected  hand,  while  the  examiner  exerts 
strong  traction  on  the  other  end  of  the  thin  object.  Where  the  ulnar 
nerve  and  the  prehensile  muscles  it  supplies  are  normal,  the  object  will 
be  strongly  grasped  with  the  th^umb  lying  flat  against  it,  opposite  the 
likewise  extended  forefinger.  On  the  paralyzed  side,  on  the  other  hand, 
the  thumb  will  be  found  flexed,  and,  no  matter  how  much  force  the 
patient  intends  to  apply,  will  be  in  contact  with  the  object  only  at  its 
extremity,  i.  e.,  with  its  pulp,  and  there  will  be  a  tendency  for  the  object 


Fig.  241. — Loss  of  sensibility  after  complete  division  of  ulnar  nerve. 

to  slip  from  his  grasp  if  some  degree  of  force  is  applied.  The  reason  for 
this  lies  in  that  forcible  prehension  is  accomplished  with  the  adductor 
of  the  thumb  and  the  internal  portion  of  the  short  flexor,  which  are  sup- 
plied (except  sometimes  the  deep  head  of  the  flexor)  by  the  ulnar 
nerve.  In  feeble,  delicately  adjusted  prehension,  on  the  other  hand, 
the  adductor  muscle  is  not  used,  but  almost  exclusively  the  flexors 
of  the  thumb  and  index  finger,  supplied  instead  by  the  median  nerve. 
Thus,  when  the  ulnar  is  paretic  or  paralyzed,  only  the  feeble,  delicate 
type  of  prehension  is  possible.^  It  may  be  of  service  to  recall  that  a 
first  dorsal  interosseus  muscle  may  remain  intact  with  complete  ulnar 
nerve  reaction.  Hence  its  early  recovery  is  not  a  necessary  index  of 
recovery  of  an  ulnar  nerve  injury. 
The  sensory  loss  is  complete  in  the  little  finger,  the  ulnar  border  of 

1  Froment:    Presse  med.,  October  21,  1915. 


MEDIAN  PALSY 


429 


the  hand  has  diminished  sensibiHty:  Epicritic  touch  and  heat  loss 
extend  to  the  ring  finger.  In  wrist  wounds  the  sensory  changes  are 
apt  to  be  minimaL 

Ulnar  palsies  most  closely  resemble  plexus  and  spinal  palsies  of  the 
eighth  cervical  and  first  dorsal  and  must  be  carefully  separated  from 
hem.    The  Klumpke  eye  findings  are  absent  in  the  ulnar  palsies. 

War  experience  seems  to  show  that  complete  section   injuries  are 

ecovered  from  after  suture  very  slowly  even  if  the  principles  of  fascicle 

nd-to-end  anastomosis  are  fairly  rigidly  carried  out.    ^Muscular  power 

^s  apt  to  be  lost  well  up  to  nine  months  or  more  and  loss  of  sensory 

iscrimination  is  apt  to  persist  as  long. 

Musculocutaneous. — This  nerve  supplies  the  muscles    which  flex 
the  forearm  on  the  arm.    Severe  lesions  cause  paresis  of  the  biceps, 
|;oracobrachialis,  brachialis  anticus.     There  is 
iilso  a  sensory  defect,  cutaneous  anesthesia  in 
the  area  indicated  on  the  figures  (Fig.  242.) 

Median  Nerve. — This  nerve  lies  deep  in  the 
muscles  of  the  arm  and  arises  by  two  branches : 
an  external  from  the  sixth  and  seventh  cervical 
roots  and  an  internal  from  the  eighth  cervical 
and  first  dorsal.  It  supplies  motor  fibers  chiefly 
to  the  forearm  muscles,  and  sensory  fibers  to  the 
haiKl.  The  chief  functions  are  pronation  of  the 
wrist,  flexion  of  the  hand  on  the  forearm,  flexion 
of  the  fingers,  by  the  deep  and  superficial  flexors, 
in  the  hand  the  thenar  muscles  except  the 
adductor  pollicis.  In  median  injuries  these 
•movements  then  are  lost.  To  the  patient  the 
loss  seems  greatest  in  the  fine  movements  of 
the  fingers  and  thumb,  buttoning,  holding  the 
pen,  constitute  the  greatest  loss.  The  two 
first  lurabricales  are  also  paralyzed  but  the  loss 
is  overcome  by  the  intact  interossei. 

Bullet  wounds,  fractures  and  dislocations  of 
the  humerus,  pressure  of  callus  and  from 
crutches,  all  may  cause  median  nerve  disorder. 
In  Burrow's  series  the  median  was  involved  242 
times  in  1000  peripheral  nerve  wounds.  A  type 
of  occupation  neuritis  in  workers  who  use  the 
small  muscles  extensi\ely  is  not  infrequent  in 
carpenters,  seamstresses,  cigar  and  cigarette 
makers,  milkers,  ironers,  etc. 

In  addition  to  the  motor  signs  there  are  usually  distinct  sensory  dis- 
turbances involving  anesthesia  of  the  i)almar  surface  of  the  hand  as 
far  as  the  middle  of  the  ring  finger  extending  up  to  the  wrist.  This 
anesthesia  is  most  marked  in  a  contractefl  area  of  this  zone  as  far  as  the 
middle  of  the  middle  finger,  the  rest  of  the  zone  shading  ofl'  to  the 
intact  sensory  distribution.     On  the  dorsum  there  are  also  slight 


Fig.  242.  —  Cutaneous 
anesthesia  in  complete 
section  of  the  musculo- 
cutaneous. 


430 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


disturbances  of  the  fingers  from  the  first  joint  outward.  There  is 
usually  a  marked  atrophy  of  the  thenar  muscles  and  some  flattening  of 
the  flexor  muscles  of  the  forearm.    Skin  disturbances,  ulcers,  elongation:, 


Fig.   243. — -Paralysis  of  the  median.     On  the  left  side  the  patient  cannot  bring 
the  thumb  in  as  on  the  right.     (Claude,  Dumas  et  Porack.) 


Fig.  244. — Combined  palsy  of  the  median  and  ulnar.     Flexion  of  the  hand  by  the  long 
abductor  and  the  short  extensor  of  the  thumb.     (Claude,  Dumas  et  Porack.) 


MUSCULOSPIRAL  PALSY 


431 


of  the  nails  occur  in  severe  bullet  and  tearing  wounds.  Severe  causalgias 
occur  in  median  nerve  injuries. 

The  general  trend  of  opinion  regarding  recovery  in  median  nerve 
injury  is  that  the  flexor  groups  of  the  forearm  recover  first  and  this  in 
from  seven  to  eight  months  after  proper  suture.  The  small  muscles  of 
the  hand  take  usually  from  twelve  to  twenty  months,  unless  intensive 
muscle  exercises  have  been  carried  on.  Vegetative  fibers  are  usually 
involved  and  the  causalgias  resulting  in  median  nerve  injury  are  often 
quite  intractable.  Their  treatment  by  alcohol  injections  is  usually 
unavailing.  The  most  frequent  factor  is  a  neuromatous  end  stump  or 
scar  tissue.  Complete  resection  and  fascicle  end-to-end  anastomosis  is 
advisable  in  cases  persisting  twenty  months. 

Combined  lesions  of  the  median  and  ulnar  which  are  frequent  in 
warfare  produce  very  characteristic  syndromes.    (See  Fig.  244.) 


Fig.  245. — Sensory  disturbances  in  median-nerve  injury:  a,  hyperesthesia  to  cotton- 
wool; b,  hyperesthesia  to  pin  prick;  c,  hyperesthesia  to  heat.  Oblique  shadings  indicate 
diminution  in  heat  discrimination;  horizontal  shadings,  loss  of  heat  sensation.  (J.  et  A. 
Dejerine  et  Mouzon.) 

Radial  or  Musculospiral  Nerve. — This  is  preeminently  the  nerve  of 
extension  of  the  arm  and  is  more  frequently  involved  than  any  other 
nerve  of  the  arm.  It  extends:  (1)  The  forearm  on  the  arm,  by  the 
triceps;  (2)  the  hand  upon  the  forearm  by  the  radial  and  posterior 
cubital;  (3)  the  fingers  on  the  hand  by  the  common  extensor  and  the 
extensors  of  the  thumb,  index  and  little  finger. 

The  chief  causative  lesions  are  war  wounds — 204  times  in  1000  cases 
in  Burrow's  summary,  146  in  639  of  Tinel's  series — fracture  of  the 
humerus,  "crutch"  pressure,  pressure  from  sleeping  with  arms  over  the 
back  of  a  chair,  "Saturday  night"  paralysis,  rarely  in  anesthesia  from 
overextension  of  the  arm.    It  is  partly  involved  in  lead  palsy. 

Symptoms. — These  will  depend  upon  the  site  of  the  lesion.  Woinids 
or  pressure  in  the  axilla  cause  a  complete  palsy. 

The  arm  hangs  with  the  forearm  drawn  up  semiflexed,  the  wrist 
dropped,  the  fingers  in  semiflexion.    Only  the  first  phalanges  are  par- 


432 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


alyzed,  however,  for  if  the  arm  be  supported  the  terminal  phalanges  are 
capable  of  extension  by  means  of  the  interossei  which  are  uninvolved. 

When  the  nerve  is  injured  on  the  outer  aspect  of  the  arm,  as  in  most 
bullet  wounds,  cuts,  etc.,  the  extensors  of  the  elbow  are  apt  to  escape, 
and  at  times  the  supinator,  but  wrist-drop  is  the  characteristic  position. 
Abduction  and  extension  of  the  thumb  are  interfered  with,  the  whole 
grasp  is  much  weakened  from  the  failure  of  the  wrist  support  rather 
than  from  any  palsy  of  the  flexors.  Supination  is  possible  with  the 
forearm  drawn  up  by  the  biceps  but  not  when  it  is  extended. 

Wounds  in  the  lower  forearm  above  the  wrist  cause  perhaps  only 
extensor  weakness  of  the  wrist. 


Fig.  246. — Attitude  in  paralysis  of  the  musculospiral.      (Tinel.) 

There  are  few  sensory  changes,  so.me  numbness  and  the  sense  of  being 
cold,  alone,  or  at  times  with  slight  anesthesia  over  the  radial  branch. 
Slight  prominence  on  the  dorsum  of  the  wrist  is  frequent  from  efYusion 
or  from  relaxation  in  the  extensor  sheaths. 

The  electrical  reactions  are  those  of  the  syndrome  of  interruption  in 
the  severe  lesions.  They  may  be  less  pronounced  in  the  simple  com- 
pression cases. 

Treatment. — After  suture  restoration  of  function  in  the  musculo- 
spiral seems  to  take  place  more  rapidly  than  in  any  other  of  the  chief 
forearm  lesions.  With  freedom  from  severe  sepsis,  much  scar  tissue  or 
specially  complicating  factors,  the  recovery  is  apt  to  he  rapid  but  very 
irregular.  Few  patients  seem  to  recover  in  about  constant  ratios. 
The  extensors  of  the  thumb  are  apt  to  recover  the  latest.  Pain  and 
sensory  symptoms  are  few  and  rarely  persist.    Some  patients  make 


MUSCULOSPIRAL  PALSY 


433 


complete  recoveries  in  from  nine  to  fifteen  months.  Motor  inco- 
ordination is  a  fairly  constant  sjnuptom  in  the  first  stages  of  restora- 
tion. 

]Sredian  Ulnar  Muse,  spiral 


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Fig.  247. — Transverse  section  of  median,  ulnar  and  musculospiral  nerves  at  different 
levels  in  the  upper  arm,  to  show  fasciculation.     (Dustin.') 

In  the  surgical  treatment  of  the  injuries  of  the  chief  nerve  of  the 
arm  tlie  principle  of  end-to-end  anastomosis  of   nerve  fascicles  has 

'  Dustin:    La  fasciculation  des  nerfs.  Ambulance  de  "I'Ocean,"  July,   1918,  vol.  ii, 
Fasc.  1. 

28 


434 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


become  highly  important.  Reference  must  be  made  to  the  studies 
already  quoted  for  a  more  detailed  consideration. 

Extension,  even  h^'perextension,  of  the  wrist  in  a  proper  brace  is 
a  sine  qua  non  of  treatment  of  musculospiral  and  radial  palsies.  The 
rest  of  the  treatment  follows  the  usual  lines  already  outlined,  depending 
upon  the  syndrome  present. 

Sciatic  Nerve.— The  sciatic  by  reason  of  its  great  volume,  its  long 
course  and  its  many  branches  is  particularly  prone  to  injury,  particu- 
larly in  war,  in  Tinel's  series  constituting  25  per  cent.,  in  Burrow's 
22  per  cent,  of  the  total  of  all  peripheral  nerve  injuries.  It  arises  from 
the  fourth  and  fifth  lumbar  roots  and  the  first,  second  and  third  sacral, 
uniting  in  one  large  trunk  in  the  lower  third  of  the  thigh  where  it  divides 
with  the  external  and  internal  popliteal  nerves.  Its  neuropathology 
may  best  be  studied  as  (1)  Lesions  of  the  external  popliteal,  (2)  lesions 
of  the  internal  popliteal;  (3)  lesions  of  the  trunk. 


Fig.  248. — -Involved  sensory  area  in 
internal  popliteal  disturbance. 


Fig.  249. — Sensory  distribution  of  the 
external  popliteal  branch  of  the  sciatic. 
Anesthesia  on  back  and  front  of  the  leg. 


External  Popliteal. — ^The  chief  collateral  branches  are  the  long 
saphenous  and  the  peroneus;  the  chief  terminal  branches,  the  anterior 
tibial  and  the  musculocutaneous.  The  external  popliteal  innervates 
the  antero-external  muscles  of  the  leg,  the  extensor  longus  digitorum, 
the  extensor  proprius  pollicis,  the  tibialis  anticus,  peroneus  longus, 
peroneus  brevis. 

Paralysis  therefore  causes  loss  of  abilit^'  to  extend  the  foot  and  of  the 
toes,  rotation  of  the  foot  on  the  ankle  and  raising  of  the  internal  border 
of  the  foot  on  the  ankle.  The  foot  therefore  drops,  the  toes  pointed 
downward.  The  gait  is  of  the  steppage  t^-pe.  The  seated  patient  with 
the  foot  flat  on  the  ground  cannot  ele^•ate  the  toes  nor  raise  the  sole  from 


SCIATIC  PALSIES 


435 


the  ground.    Syndromes  of  interruption  and  of  compression  may  be 
distinguished. 

The  sensory  disturbances  are  as  illustrated.  Trophic  disturbances 
are  dorsum  edemas,  paleness  or  cyaiiosis  of  the  skin,  desquamation, 
ulcers,  hypertrichosis. 


/ 

/^ 

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c 

^^^^^ 

L 

¥ 

R 

( 

^ 

Fig.  250 


Fig.  2.51 
FiG^.  2.50  and  251. — Lesion  of  cauda  at  fourth  lumbar  with  disturbance. 

The  upper  antero-external  area  of  anesthesia  results  from  lesion  of 
the  cutaneous  branch  of  the  peroneus :  the  posterior  from  the  peroneus ; 
the  musculocutaneous  on  the  inferior  aspect  of  the  leg  and  dorsum 
of  the  foot.     The  anesthesia  is  rarely  complete  nor  constant.     It  is 


436 


AFFECTIONS  OF  THE  PERIPHERAL  NERVES 


chiefly  found  on  the  antero-external  surface  of  the  leg  and  the  dorsum 
of  the  foot. 

The  external  popliteal  is  the  most  frequently  injured  branch.  In 
many  respects  it  behaves  similarly  to  the*  musculospiral  of  the  upper 
extremity  in  war  injuries  and  after  suture. 


/S\ 

r^^ 

y^ 

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'-I 

2)- 

W^ 

43vt^ 

I 

u 

"Z^jlfey 

i 

V'^ 

Fig.  252 


Fig.  253 
Figs.  252  and  253. — Lesion  of  cauda  at  fifth  lumbar  with  area  of  sensory  disturbance. 

Internal  Popliteal. — Lesions  here  cause  loss  of  function  of  all  of  the 
posterior  muscles  of  the  leg  and  of  the  plantars.  There  results  a  loss  of 
flexion  of  the  foot,  of  flexion  of  the  toes,  enfeebled  movements  of  rota- 
tion and  adduction,  loss  of  adduction  and  abduction  of  the  toes. 


SCIATIC  PALSIES 


43: 


The  gait  is  not  markedly  altered  to  casual  observation.  The  foot  is 
put  down  flat,  the  patient  does  not  rise  on  his  toes.  On  rising  from  a 
sitting  position,  no  support  is  given  by  the  toes.  The  plantar  arch  is 
highly  curved,  pes  valgus  develops.  The  toes  in  repose  are  hyper- 
extended.    There  is  a  loss  of  the  plantar  and  Achilles  reflexes. 


/jT'tCA 

y^^ 

r^^K 

'jQij 

^^^•TIiZj 

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c^ 

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fy  1 

Fig.  254 


Fig.  255 
Figs.  254  and  255. — Lesion  at  level  of  fourth  lumbar  right. 

Sensory  disturbances  are  marked  as  illustrated.  The  deep  sensi- 
bility fibers  seem  to  travel  mostly  with  this  branch.  It  behaves  as  the 
median  nerve  in  many  respects  and  causalgia  is  here  frequent  and 
regeneration  is  slow  and  painful. 


438  AFFECTIONS  OF  THE  PERIPHERAL  NERVES 

Hyperidrosis  is  frequent,  ulceration  at  times  occurs.  In  neuritic 
tyi)es  the  trophic  disturbances-  are  very  marked  and  widespread.  In 
lesions  of  the  popliteal  nerves  the  trophic  disturbances  are  apt  to  be 
less  than  when  the  sciatic  trunk  is  involved. 


Figs.  256  and  257. — Distribution  of  sciatic  anesthesia. 

In  trunk  lesions  there  is  a  combination  of  both  syndromes  just 
described.  There  is  marked  general  atrophy  of  the  entire  leg  which 
moves  more  as  a  stump  held  rigid  by  the  quadriceps.  The  glutei  may 
be  involved  also.  The  chief  types  seen  are  the  paralytic,  neuritic, 
causalgic,  and  neuralgic.  Partial  and  dissociated  pictures  are  the  rule 
especially  in  the  wounds  of  wa^-fare.  Severe  pains,  tender  nerve  trunks 
and  trophic  changes  are  the  rule.     (See  Lumbosacral  syndromes.) 


CHAPTER  VI. 
LESIONS  OF  THE  SPINAL  CORD. 

The  anatomy,  histology  and  physiology  of  the  spinal  cord  will  be 
discussed  only  in  so  far  as  problems  of  localization  and  pathology  are 
concerned. 

A  large  group  of  disorders,  chiefly  limited  to  the  cord,  come  under 
review.     One  may  present  them  as  a  series  of  s^^ldromes  which  affect: 

1.  Chiefly  the  peripheral  motor  neuron  (anterior  horn  cells). 

2.  Combinations  of  anterior  horn  cells  and  pyramidal  tracts. 

3.  Chiefly  p^Tamidal  tracts. 

4.  Chiefly  posterior  columns. 

5.  Vegetative  cell  groups. 

6.  Central  gray. 

7.  Combined  and  diffuse  lesions. 


Bodices  doraalis  I-t-IX 
Radices  cervicalet 
Posterior  Root  Zon 


Radices  dor  sales  V+XII 
Radices  lumbares 
Radices  sacrales 


cerebello  spinalis^ 
posterior  a 

Limiting  Layer- 


Tract  us  cortico 
spinalis  cruciatas^. 


Tractus  cerebello' 
spinalis  anterior 


al  Field 

descending  dorsal  root  fibers 
■Marginal  Zone 

6 

JTractus  corticospinalis 


ractus  thalamo- 
spinalis 


Ventral  Field 


Fase-  olivares 


Fibrae  associativae  breves 

Fibrae  associativae  longae 


ractus  tectospinalis 
cruciatua 


Fasciculus  marginalia 
Tractus  corticospinalis 
Fasciculus  aulcomaruinalis  f 


Fasciculus  longitudinalis  dorsalis 


Fig.  258. — Cross-section  of  spinal  cord  showing  localization  of  chief  structures  with 

lesions.     (Veraguth.) 

These  subdivisions  are  largely  arbitrary;  at  times  they  correspond 
to  clinical  entities,  so-called,  again  they  are  fortuitous  combinations. 
Thus  a  poliomyelitis  may  clearly  delimit,  i.  e.,  in  its  end-results,  a 
group  of  motor  neurons,  anterior  horn  cells,  while,  on  the  other  hand, 
spinal  syphilis  may  show  any  of  the  localizations  just  tabulated.  In 
the  beginning  of  a  syringomyelia  the  earliest  signs  may  be  those  of 
irritation  of  the  pyramidal  tracts,  i.  e.,  lateral  sclerosis  type  of  lesion. 

(439) 


440 


LESIONS  OF  THE  SPINAL  CORD 


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ACUTE  POLIOENCEPHALOMYELITIS  441 

Soon  added  thereto  atrophy  of  muscles  begins,  /.  e.,  anterior  horn 
syndromes.  Then  a  loss  of  pain  sensibility  with  intact  touch  is  seen, 
i.  e.,  central  gray  lesion.  The  arm  begins  to  be  edematous  and  trophic 
changes  develop;  vegetative  cell  group  involvement.  If  the  student 
keeps  in  mind  the  cross-section  of  the  cord  it  may  be  seen  how  these 
various  pictures  may  be  developed.     (See  Fig.  258.) 

The  chief  clinical  pictures  are  summarized  in  the  table  on  page  440. 

A  study  of  the  cross-section  of  the  cord  will  bring  these  localizing 
factors  more  closely  into  view.  The  localizing  features  are  brought 
out  in  Figs.  27-32,  34-38  (see  pp.  78-79,  86-87). 

Location  of  Lesion.  Chikf  Symptoms. 

1.  In  the  posterior  root  zone.  Irritation  causes  hyperesthesise.     Destruc- 

tion causes  loss  of  superficial  sensibility 
in  the  root  distribution  spreading  over 
at  least  three  roots.  Ataxia  and  event- 
ually astereognosis  in  the  extremity 
involved. 

2.  In  posterior  column  of  one  side.  Anesthesia    to    deep    sensibility    and    to 

touch.  Ataxia  of  metameres  below  the 
lesion. 

3.  In  Goll's  columns  of  both  sides.  Anesthesia  to  deep  sensibility  and  hypes- 

thesia  of  the  lower  extremities  only, 
even  in  high  lesions. 

4.  In  central  gray,  especially  of  anterior         Dissociated    sensibility    (thermanesthesia 

commissure.  and  analgesia  in  the  affected  metameres 

as  indicated  in  the  skin  distribution). 

5.  Posterior  portion  of  the  later  columns         Crossed  hemihyperesthesia  plus  the  symp- 

^•ith  integrity  of  limiting  layer.  toms  of  6. 

6.  PjTamidal  tracts.  Spastic  paralysis  of  the  caudal  metameres 

below  the  lesion  without  reaction  of 
degeneration,  often  crossed  movements, 
no  atrophy  and  with  inci  eased  reflexes. 

7.  Anterior  horns.  Flaccid  paralysis  of  the  muscles  of  a  num- 

ber of  root  zones,  atonia  and  atrophy 
of  muscles  of  involved  metameres; 
R.  D.,  loss  of  reflexes. 

8.  Spinocerebellar  paths.  Bilateral    involvement     causes    cerebellar 

ataxia. 

9.  Lateral  recess.  Vegetative  disturbances  metamerically  dis- 

tributed. 

The  majority  of  these  cord  sjTrdromes  are  considered  here,  some  are 
discussed  under  their  etiological  groupings:  s^'philis  of  the  nervous 
system,  for  instance,  taking  over  tabes,  spinal-cord  gummata,  s;yi)hilitic 
meningomyelitis. 

ACUTE   POLIOENCEPHALOMYELITIS. 

Historical  Note. — This  disease  has  affected  mankind  for  centuries. 
Mitchell  reports  shortening  of  the  femur,  presumably  due  to  this 
disease,  in  an  Egj'ptian  mummy.  Jonathan's  son  (II  Samuel,  iv,  4), 
possibly  had  this  disease.  Numerous  drawings  and  paintings  of  the 
fourteenth   to   sixteenth   centuries    (Hieronymus   Bosch)  ^    attest   to 

IE.  Hollander:  Die  Medizin  in  der  klassischen  Malerei,  1903,  p.  116  (Fig.  66  for 
example  of  marked  quadripedal  progression  in  severe  poliomyelitis).  Also  see  Richer: 
I'Art  et  la  Medecine. 


442  LESIONS  OF  THE  SPINAL  CORD 

its  prevalence  in  those  times.  Thronghoiit  the  period  of  the  later 
Latin  writers  it  was  usnally  included  luider  paralyses,  hemiplegias, 
etc.  Underwood,  in  1784,  described  a  disease,  to  him  new,  "Debility 
of  the  Lower  Extremity,"  that  gave  a  direct  impetus  to  its  defijiite 
separation  as  a  type  by  Jacob  v.  Heine  in  1840.  In  1819  Jorg  gave  an 
excellent  case  history,  and  in  1848,  independent  of  Heine,  Rilliet  and 
Barthiez  contributed  an  important  article  to  French  literature.  They 
called  the  disease  an  essential  paralysis,  and  thought  it  had  little  spinal 
pathology.  Badham,  in  1835,  described  a  group  of  cases  undoubtedly 
of  poliomyelitis.     One  of  his  children  had  an  ocular  palsy. 

Although  much  was  written  previous  to  Duchenne's  time,  his  work 
in  1855  marked  the  beginning  of  a  new  era  in  the  study  of  this  disease 
and  in  Heine's  second  edition,  1860,  the  status  of  the  disease  at  that 
time  is  well  reflected.  In  1865  attention  was  first  called  to  the  possible 
relation  of  infantile  to  adult  poliomyelitis  by  M.  Meyer,  which  study 
was  followed  by  a  large  number  of  further  contributions  from  Charcot's 
students. 

The  anatomical  era  may  be  said  to  have  opened  with  Cornil  in  1863, 
and  there  then  grew  up  the  Charcot  thesis  of  a  primary  affection  of  the 
ganglion  cells  of  the  anterior  horns,  which  has  had  to  give  way  to  a 
more  extensive  pathology  through  the  recent  studies  of  Wickmann, 
Harbitz  and  Scheel,  Flexner,  Strauss  and  others  (1907-1919). 

Seeligmiiller's  masterly  monograph  in  1880  practically  contained 
the  standard  teachings  up  to  the  appearance  of  Medin's^  work  in 
1896,  when  the  epidemiological  features  of  the  disease  were  brought 
out.  In  later  years  Lovegren  (1904),  Wickmann  (1905-1907),  Harbitz 
and  Scheel  (1905)  have  still  further  refined  the  clinical  and  pathologi- 
cal aspects,  while  Flexner,  Landsteiner,  Popper  and  others  have  been 
able  to  convey  the  disease  from  the  human  to  monkeys  and  thence 
to  other  monkeys  (1909-1911). 

The  most  recent  monographic  treatments  of  the  subject  are  found 
in  Wickmann's^  very  able  contribution  and  Miiller's  equally  valuable 
monograph  (1911),  one  by  Romer^  (1911),  a  shorter  study  by  Peabody 
and  Draper  (1913),*  and  a  valuable  study  by  Batten^  and  another  by 
Ruhrah  and  Mayer  (1917). « 

Etiology. — The  disease  is  an  acute  infectious  disease.  The  organism 
is  probably  a  living  one;  it  can  be  conveyed  to  human  beings,  to 
monkeys  and  to  rabbits.  A  small  amount  of  the  emulsion  of  the 
spinal  cord  of  humans  injected  into  the  brain  of  a  monkey  has  caused 
the  disease,  which  Flexner  has  transmitted  from  monkey  to  monkey 
for  twenty-five  generations.  The  organism  is  thought  to  be  a  small 
anaerobic  organism  possibly  isolated  by  Noguchi  (1913)  whose  virus  is 
capable  of  passing  through  a  Berkefeld  filter,  and  which  virus  is 

*  See  translation  of  this  study  by  Jelliffe  and  Dederer:  Alienist  and  Neurologist,  1916. 

2  Nervous  and  Mental  Disease  Monograph  Series,  New  York. 

3  Wm.  Wood  &  Co.,  New  York.  ■•  Rockefeller  Institute. 
6  Batten,  F.  E.:    Brain,  1915. 

^  Lea  &  Febiger:    New  York  and  Philadelphia. 


ACUTE  POLIOENCEPHALOMYELITIS 


443 


destroyed  by  heat  and  weak  disinfectants,  but  not  by  cold  nor  dry- 
ness. In  many  respects  the  virus  resembles  that  of  rabies.  It  has 
been  conveyed  by  direct  injection  of  the  virus  into  the  brain,  through 
the  uninjured  and  scarified  nasal  mucous  membrane,  and  through 
injection  of  the  intestinal  lymph  glands  of  affected  animals  (Plexner). 
An  immunity  of  yet  unknown  length  seems  to  be  established  by 
one  attack.  Second  attacks,  though  rare,  do  occur. ^  The  disease 
seems  to  be  conveyed  by  direct  contact,  through  indirect  contact,  and 
through  nasal  and  gastro-intestinal  secretions.  It  does  not  seem  to 
be  highly  contagious. 


SISTEC!_OF_E.S. 
SEPT.  2ND 


LIVED  OTHER  SIDE 

OF  HOUSE  WITH    C.R. 

ONSET  AUG.  28TH 


Fig.  259. — Development  of  a  contact  group  of  poliomyelitis  cases  in  Jefferson  County 
during  August,  1916.  (Courtesy  of  Dr.  Mathias  Nicoll,  Jr.,  New  York  State  Board 
of  Health.) 

•  Epidemic  extensions  have  now  been  studied  for  nearly  sixty  years, 
and  Cordier  first  expressed  a  belief  in  its  contagiousness.  Badham's 
early  case  reports  seem  to  indicate  a  small  epidemic.  Medin  defi- 
nitely proved  it  (1896).  Some  eighty  or  more  epidemics  have  been 
reported  to  1919.  The  most  recent  pan-epidemic  apparently  started 
in  Norway  and  Sweden  in  1903-1904,  spread  to  the  United  States  in 
1907-1912,  to  Germany  and  France  in  1908-1911,  with  isolated  far- 
lying  outbreaks  in  Cuba,  Australia,  etc.  The  epidemic  of  1916  in  the 
vicinity  of  New  York  is  the  most  extensive  on  record. 

Rural  districts  have  suffered  greatly,  and  density  of  poi)ulation  has 
not  seemed  to  play  a  large  role;  northern  countries  seem  fa\'()rable  to 
the  outbreak  of  the  disease,  and  the  season  of  greatest  intensity  is 
usually  in  the  warm  months  of  July,  August  and  September.  Some 
epidemics  have  occurred  in  winter.     (Wickmann.) 

The  majority  of  the  cases  occur  in  children  from  one  to  five  years  of 
age.  Intra-uterine  cases  are  known^  and  individuals  as  old  as  sixty 
have  contracted  the  disease.     Certain  epidemics  have  shown  marked 


'Taylor,  E.  W.:     Jour.  Norv.  and  Ment.  Dis.,  September,   1916,  for  complete  dis- 
cussion.    Moncrief:  ibid.,  1919. 
2  Batten:  -Brain,  1910. 


444 


LESIONS  OF   THE  SPINAL  CORD 


variability  in  the  matter  of  age  incidence,  the  Swedish  epidemic  of 
1905  ha^'ing  as  high  as  10  per  cent,  adults.  In  large  epidemics  adults 
seem  more  often  affected.  The  male  sex  has  seemed  to  be  more  often 
involved,  but  the  differences  are  not  very  marked.  Nationality  seems 
to  play  little  role,  although  it  has  been  assumed  that  Scandinavians 
are  peculiarly  susceptible.  This  may  only  be  an  indication  of  the 
more  careful  study  given  by  these  authors.  Predisposing  hereditary 
inferiority  factors  are  as  yet  unknown. 


1  1 

1  1  i  M  M  1  !  1  1  1  M  1  11  1  1  1  1  1 

RATE  = 

=  NUMBER  OF  CASES  OCCURR'rNG  AT  [EACrt  AGE  PERIOD 

FER,10,000  IN 

THE  POPULATION. 

R 

URAL 

NEW  YORK 

1 

-bvE 

\n 

120  ^H 

100 1^1 

NEW 

YORK  CITiY 

1 

-^W 

ARR 

1 

80 

-sr-rip 

60 

UPSTAJE 

c 

TIES 

1     I.J._ 

u 

»tAJ 

■10 

5,  -i  i!o 

5" 

- 

- 

lu-j 

20 

j 

, 

RCBEIH 

KranPSB 

Pt 

-'^"■■l 

^^■■B 

0 

RATE 

1 

B^iH                               mu^m 

1!3        77        23         3    1  1   119   1    29          5        0.4         44         15    1     5     1   0.6    1 

120 


100 


0 

RATE 


Fig.  260. — Comparative  incidence  rates  of  poliomyelitis,  by  age  periods,  in  different 
sections  of  New  York  State  during  the  epidemic  of  1916.  (Courtesy  of  Dr.  Mathias 
Nicoll,  Jr.,  New  York  State  Board  of  Health.) 


The  incubation  period  varies  from  one  to  ten  days,  the  majority 
showing  a  period  of  from  one  to  five  days.  In  experimental  polio- 
myelitis of  monkeys  the  incubation  time  is  about  six  to  nine  days. 

Pathology.^ — The  older  concepts  of  a  primary  intoxication  or  of  an 
inflammation  of  the  motor  horn  cells  exclusively  must  be  abandoned. 
Acute  poliomyelitis  is  due  to  an  acute  interstitial  inflammation  (a 
meningomyelitis),  involving  all  parts  of  the  nervous  system.    There 


ACUTE  POLIOENCEPHALOMYELITIS 


445 


is  a  congestion,  infiltration  and  edema  of  the  leptomeninges,  cord, 
medulla,  pons,  cerebellum  and  cerebrum.  The  dura  is  usually  less 
markedly  involved;  the  pia  is  congested  and  infiltrated  with  round 
mononuclear  cells  (h-mphocytes),  particularly  in  the  sacral  and  lumbar 
regions.  The  vessels  are  congested  and  their  sheaths  infiltrated,  the 
progression  of  the  inflammatory  reaction  apparently  following  the 
A  essels  from  the  periphery  to  the  interior  of  the  cord.  The  cerebro- 
spinal fluid  is  increased  in  quantity,  almost  opalescent  early  in  the 
disease,  with  markedly  increased  lymphocytes,  in  some  of  which 
Lafora  and  Hough  have  found  pictures  resembling  the  Leishman- 
Donovan  bodies.  The  fluid  later  becomes  clearer,  but  still  shows  a 
pathological  increase  in  leukocytes. 


I 


-'if<^-«;^ 


Zr:^^»**^  d1^*  tk  *^  V« »  -A  %   » .     V 


\« 


r 

4 

rfgl^'il 

.'  '''■'-■'.:f^''- 

J 

Fig.  2til. — Acute  poliomyelitis,  showing  as- 
sociated meningitis  at  the  edge  of  cord. 


Fig.  262. — Acute  poliomyelitis,  show- 
ing the  vascular  congestion  and  the 
surrounding  infiltration  area  of  the 
anterior  spinal   artery. 


Within  the  cord  the  inflammatory  process  follows  the  pial  processes 
into  the  depths  of  the  anterior  fissure  and  along  the  sheaths  of  the 
central  vessels.  The  posterior  root  fibers  and  the  spinal  ganglia  are 
also  infiltrated. 

The  vascular  lesions  are  particularly  noticeable  and  the  interstitial 
and  ganglionic  changes  depend  largely  upon  them.  The  vessels 
throughout  are  dilated  and  engorged,  the  capillaries  often  being 
enormously  distended.  This  marked  hyperemia  is  found  throughout, 
and  vascular  hemorrhagic  lytic  changes  are  frequent  but  small.  As 
in  rabies,  and  to  some  extent  in  s^']3hilis,  there  is  a  marked  perivascular 
or  intra-adventitial  infiltration,  apparently  of  lym])hocytes,  not 
plasma  cells.     Interstitial  changes  in  the  gray  and  white  matter,  chiefly 


446 


LESIONS  OF   THE  SPINAL  CORD 


of  an  infiltration  of  cells,  and  proliferation  of  glia  cells,  occur.  By 
reason  of  the  rich  blood  supply  the  gray  matter  of  the  anterior  horns 
bears  the  brunt  of  the  inflammatory  edema  and  h^'pe^emia,  with 
destruction  of  many  of  its  motor  cells.  This  is  a  secondary  process. 
Harbitz  and  Scheel  believe  that  small  abscesses  are  formed,  but  this 
is  an  exception  if  it  does  occur. 

The  ganglion  cells  undergo  varying  degrees  of  degeneration,  some- 
what proportional  to  the  infiltration,  the  axis-cylinder  finally  break- 
ing down.  Amid  the  degenerated  cells  many  normal  ones  may  be 
found,  and  the  distribution  of  degenerated  areas  is  very  variable,  the 
sacrolumbar  cord  being  more  seriously  affected,  although  any  part  of 
the  cerebrospinal  axis  may  be  involved.  All  classes  of  ganglion  cells 
go  under,  but  the  lateral  dorsal  cord  regions  are  much  less  involved; 
however,  many  vegetative  system  cells  are  injured. 


Fig.  263.- 


-Acute  poliomyelitis.     The  various  stages  of  destruction  of  the 
anterior  horn  cells.  ,_, 


As  a  rule  the  functional  involvement  by  reason  of  the  edema, 
hyperemia,  and  infiltration,  is  far  in  excess  of  the  permanent  anatomi- 
cal'loss,  hence  the  widespread  character  of  the  paralysis  in  the  early 
stages  and  the  marked  degree  of  recovery  possible.  Only  a  small 
proportion  of  the  primarily  involved  ganglion  cells  degenerate  entirely. 
The  fiber  pathways  may  show  permanent  infiltration  changes  with  the 
production  of  anomalous  spastic  phenomena. 

The  spinal  nerves  are  involved  usually  at  their  junction  with  the 
cord,  and  some  polyneuritis  may  be  present  early  in  the  disease. 

The  medulla,  pons,  cerebellum  and  cerebrum  all  are  involved  to 
a  greater  or  lesser  degree.     Certain  cases  show  that  the  main  lesion 


ACUTE  POLIOENCEPHALOMYELITIS  447 

is  in  one  or  more  of  these  regions,  rather  than  in  the  spinal  cord. 
True  encephaUtis,  with  varying  degrees  of  feeble-mindedness,  is  a 
frequent  end-result  of  these  involvements  of  the  upper-lying  nervous 
structures. 

The  other  organs  of  the  body  do  not  escape.  There  are  evidences 
of  an  acute  general  infection  everx-where.^  Lymphoid  tissues  are 
affected,  including  the  thymus. 

Sjrmptoms. — The  study  of  the  recent  epidemics  has  sho^Ti  a  great 
variability  in  the  affection,  but  practically  all  of  the  cases  show  the 
effects  of  an  acute  infection  as  prodromal  and  early  signs.  The  later 
course  of  the  disease,  dependent  upon  the  locations  chiefly  involved, 
permits  a  separation  into  several  t^'pes  of  which  ^Yickmann  recognizes 
eight  classical  pictures. 

Prodromata  are  usual.  They  vary  in  the  different  epidemics. 
They  consist  of  fatigability,  loss  of  appetite,  slight  digestive  disturb- 
ances, with  nausea,  looseness  of  the  bowels,  coryza  or  bronchial 
irritation  (bronchitis,  bronchopneumonia)  with  skght  elevation  of 
temperature.  Conjunctivitis  may  occur;  lymphatic  swellings  are 
usual.  The  patient  may  thus  suffer  for  twenty-four  to  seventy-two 
hours  before  the  acute  prostrating  effects  of  the  disease  become  mani- 
fest. Some  few  cases  show  almost  complete  recovery  after  such 
prodromata,  and  then  again  taken  ill  suddenly  or  may  go  on  to  recovery 
(abortive  cases) .  Careful  observation  will  probably  reduce  the  number 
of  cases  reported  as  coming  on  without  prodromata. 

The  cerebrospinal  fluid  in  the  prodromal  stages  may  show  opal- 
escence with  very  marked  lymphocytosis.  The  blood  changes  are 
apparently  not  constant.  La  Fetra  has  reported  a  leukocytosis  of 
from  L3,000  to,  20,000;  whereas  Miiller  has  found  a  leukopenia  of 
from  3000  to  5000,  and  also  finds  leukopenia  in  experimental  monkey 
poliomyelitis.  The  hTuphocvtes  are  increased.  Xo  parasites  have 
been  found  in  the  blood  up  to  the  present  time.  ^Slany  bacteria  have 
been  isolated  but  it  is  not  yet  proved  that  they  are  the  true  causative 
agents  (1919). 

After  twenty-four  to  seventy-two  or  more  hours  the  temperature 
suddenly  rises.'  It  varies  from' 99°  F.  to  102.5°  F.  or  even  105°  F.  to 
106°  F.,  and  bears  little  relation  to  the  severity  of  the  disease.  Abor- 
tive cases  have  shown  high  temperatures  and  severe  cases  little. 
Chills  and  convulsions  occasionally  attend  the  rise  in  temperature. 
The  temperature  curve  is  not  characteristic,  being  either  remittent 
or  continuous.  Subnormal  temperatures  occur  and  indicate  marked 
cervical  involvement. 

Headache  is  a  frequent  symptom.  It  is  often  severe  and  usually 
frontal;  it  may  be  occipital,  resembling  a  meningeal  headache.  Pros- 
tration is  marked,  diarrhea  is  extremely  common,  vomiting  is  frequent 
and  constipation  is  not  unusual.  Respiratory  symptoms  are  not 
marked,  save  when  respiratory  palsies  occur.  The  kidneys  sliow  little, 
and  the  heart's  action  is  that  in  an  acute  infection,  in  general  with 

'  Walter:     Deutsch.  Ztschr.,  No.  2,  vol.  xlv. 


448  LESIONS  OF  THE  SPINAL  CORD 

a  tendency  to  show  nervous  tachycardia,  especially  in  the  cases  with 
medullary  involvement.    Weakness  is  the  rule. 

Early  profuse  sweating  is  a  frequent  symptom  and  skin  eruptions 
are  occasionally  seen,  although  herpes  is  rare  in  contrast  with  cere- 
brospinal meningitis.    Other  exanthemata  occasionally  appear. 

Nervous  Symptoms. — As  a  rule  there  is  considerable  restlessness 
especially  in  younger  patients.  The  children  are  peevish,  petulant  or 
very  fretty.  Sleep  is  often  disturbed  with  frequent  crying  out.  Some 
children  lie  drowsy  or  apathetic  and  may,  though  rarely,  show  coma, 
delirium  or  convulsions.  Pain  is  a  frequent  early  symptom  and 
may  be  very  severe.  Marked  hyperesthesia  is  usual  (90  per  cent.), 
and  is  brought  on  by  the  least  attempt  at  motion  of  the  limbs,  pres- 
sure on  the  nerve  trunks,  touching  of  the  skin.  Heine  called  attention 
to  this  in  1840.  Movements  of  the  head  and  spine  are  particularly 
painful  and  some  patients  are  extremely  anxious  and  fearful,  whimper- 
ing continuously,  closely  resembling,  in  the  early  stages,  meningeal 
cases.     There  is  frequent  photophobia  and  hjT)eracusis. 

The  infiltration  in  the  cord  causes  other  sensory  symptoms,  such  as 
paresthesise  and  anesthesise.  Stiffness  is  not  infrequent,  with  slight 
though  not  marked  retraction  of  the  head  (Kernig's  sign)  in  some  and 
varying  degrees  of  rigidity  of  the  limbs,  with  contracted  position  of 
the  lower  limbs  particularly,  are  frequent. 

Twitching  and  jerking  of  the  limbs  are  also  frequent. 

The  stage  of  paralysis  soon  sets  in  and,  according  to  the  predominant 
localization,  following  Wickmann,  eight  types  may  be  distinguished. 
These  types  represent  general  tendencies  rather  than  hard-and-fast 
differences.  The  further  symptomatology  will  be  given  in  accord- 
ance with  these  divisions.  These  types  are:  (1)  spinal  poliomyelitis 
forms  (the  commonest  tA-pe);  (2)  acute  ascending  t\T)es  (Landry's 
paralysis);  (3)  bulbar  or  pontine  forms;  (4)  encephalitic  forms;  (5) 
cerebellar  and  ataxic  forms;  (6)  meningitic  forms;  (7)  neuritic  forms; 
(8)  abortive  forms.  To  these  we  would  add  the  (9)  vegetative  types  in 
which  the  Jacobsohn's  nuclei  are  invaded  with  consequent  trophic  and 
visceral  vegetative  symptoms.  These  are  frequently  mingled  in  the 
general  paralytic  medley. 

1.  Spinal  Forms. — There  is  usually  an  early  prodromal  weakness, 
or  even  a  paresis  which  is  very  widespread.  This  develops  to  a  rela- 
tively marked  paralysis,  sometimes  within  twelve  to  twenty-four  hours, 
but  more  often  in  from  two  to  five  days,  occasionally  after  a  week. 
Careful  observation  has  shown  that  the  paralysis  is  a  gradual,  rather 
than  an  abrupt  one.  It  begins  with  weakness,  advances  to  paresis, 
and  finally  becomes  a  definite  paralysis,  whereas  the  weakness,  how- 
ever, is  very  widespread,  the  palsies  are  less  so,  and  the  paralyses 
even  more  restricted.  When  the  ultimate  stages  are  reached,  the 
weaknesses  clear  up,  the  palsies  gradually  disappear,  and  the  residual 
paralyses  often  represent  but  a  small  part  of  what  appeared  to  be  a 
wholesale  devastation.  This  is  in  strict  accordance  with  the  patho- 
logical features. 


ACUTE  POLIOENCEPHALOMYELITIS 


449 


The  distribution  of  paralyses  is  due  to  factors  concerning  which 
there  is  Httle  definite  information.  Any  muscle  of  the  body  may  be 
involved.  Involmitary  muscles  are  frequently  implicated  (vegetative 
nuclei)  with  chronic  ptoses,  vascular  disorders,  intestinal  symptoms,  etc. 
From  a  purely  statistical  study  of  the  cases  it  has  been  found  that  the 
lower  limbs  are  involved  twice  as  often  as  the  upper;  in  some  epidemics 
four  times  as  often.  The  entire  limb  is  rarely  involved  permanently, 
but  special  muscle  groups  are  picked  out.  Thus  in  the  lower  extremities 
the  quadriceps,  the  peronei,  and  the  tibialis  anticus  are  the  oftenest 


^  Fig.  264. — Abdonunal  muscle  palsy — polio- 
myelitis.    (Frauenthal.) 


Fici.  265. — Paralysis  of  left  arm  and 
left  leg.     (Ruhriih.) 


affected;  in  the  upper  extremities  the  scapular  and  deltoid  muscles. 
Asymmetry  in  the  final  i)icture  is  the  rule.  The  muscles  of  the  trunk 
are  involved  next  most  frequently,  while  the  arms  are  lea.st  frequently 
involved.    Tiie  trunk  muscle  palsies  are  often  overlooked,  however. 

In  young  children  it  is  almost  impossible  to  localize  the  paralyses 
in  the  early  stages,  especially  as  many  such  jjatients  go  through  the 
initial  stages  of  the  disease  without  there  being  a  suspicion  of  the  real 
difficulty.  Here  the  loss  of  the  refiexes,  the  hy])otonus,  the  careful 
scrutiny  of  the  position  of  the  limbs,  the  beha\'ior  to  passive  motion 
29 


450  LESIONS  OF  THE  SPINAL  CORD 

and  resistance  movements  and  the  tickling  responses  lead  to  a  correct 
appreciation  of  the  difficulties.  They  also  permit  a  diagnosis  of  rudi- 
mentary and  of  mild  cases.  Babies  in  arms  behave  as  though  they 
were  lumps  of  dough,  and  the  mother  notices  the  hypotonus. 

Weakness  of  the  muscles  of  the  abdomen  is  an  important  early 
diagnostic  feature,  especially  in  the  differentiation  from  meningitis. 
The  involvement  is  usually  bilateral  and  diffuse.  The  muscles  are 
hypotonic,  and  swell  out  as  though  the  intestines  were  inflated  with 
gas.  There  is  often  a  loss  of  the  epigastric  and  abdominal  reflexes- 
symmetrical  or  asymmetrical.  The  patients,  further,  are  unable  to 
come  from  a  horizontal  to  a  sitting  position.  In  some  patients  the 
abdominal  muscles  are  alone  involved.  Obstinate  constipation  usually 
accompanies  the  abdominal  palsies. 


Fig.   266. — Anterior  poliomyelitis.     Extreme   flexion  deformity  at  the  hips,  inducing 
quadrlpedal  locomotion.     (Gibney.) 

Of  the  back  muscles  the  latissimus  dorsi  are  the  oftenest  involved. 
The  glutei  are  also  somewhat  implicated.  Children  with  these  palsies 
waddle  when  they  walk.  They  behave  like  children  with  muscular 
dystrophy  on  rising  from  the  floor. 

Only  rarely  is  the  diaphragm  implicated.  They  are  usually  among 
the  fatal  cases.  Unilateral  diaphragmatic  palsies  have  been  observed. 
The  muscles  of  the  hips  are  involved  in  at  least  two-thirds  of  the  cases. 

The  following  table  from  Wickmann  will  serve  to  indicate  the 
general  run  of  the  cases.  The  figures  represent  the  study  of  868 
cases  in  the  Swedish  epidemic  of  1905: 


ACUTE  POLIOENCEPHALOMYELITIS  451 

1.  Paralysis  of  one  or  both  legs 353 

2.  Paralysis  of  one  or  both  arms 75 

3.  Combined  paralysis  of  arms  and  legs 152 

4.  Combined  leg  and  thigh  paralyses 85 

5.  Combined  arm  and  thigh  paralyses 10 

6/  Isolated  thigh  paralysis 9 

7.  Paralysis  of  the  entire  musculature 23 

8.  Ascending  paralysis 32 

9.  Descending  paralysis 13 

10.  Combination  of  spinal  and  cranial  nerve  paralyses       ....        34 

11.  Isolated  cranial  nerve  palsies 22 

12.  Localization  uncertain 60 

868 

The  figures  of  the  Committee  of  the  Xew  York  Neurological  and 
New  York  Pediatric  Societies  give  similar  results.^ 

Sensory  Disturhances. — The  older  teachings  that  sensory  disturb- 
ances are  uniformly  absent  is  not  true.  Almost  invariably  there  is 
in  the  beginning  of  the  disease  a  marked  hj^jeresthesia.  The  slightest 
touch  causes  marked  reaction.  Loss  or  diminution  of  sensation  is  also 
not  uncommon.  Complete  anesthesia  is  rare,  but  has  been  observed. 
Hjy-pesthesise  are  common,  and  may  include  both  thermal  and  pain 
sensibilities.  In  .young  children  the  difficulties  of  observation  cause 
one  to  overlook  these  sensory  anomalies. 

Vegetative  Involvements. — The  bladder  is  frequently  involved  in  the 
early  states.  Urinary  retention  is  frequent,  incontinence  is  rare.  As 
a  rule  the  disturbance  is  transitory,  hence  overlooked.  It  is  frequently 
thought  to  be  a  symptom  of  the  general  infection,  but  Wickmarm 
regards  it  as  due  to  central  nervous  lesions. 

Constipation  is  not  unusual,  and  marked  disturbances  of  the  intes- 
tinal functions  do  occur.  These  are  often  of  the  nature  of  colitis, 
mucous  colitis,  etc.  A  chronic  intestinal  weakness  may  be  a  residual 
of  a  poliomyelitis. 

Vascular  disorders  are  very  frequent.  Skin  lesions  from  reduced 
skin  tone  are  often  met  within  in  later  life.  Visceral  ptoses,  relaxed 
ligamentous  attachments,  and  other  vegatative  nerve-level  disorders 
have  been  obser\'ed.    Bony  lesions  are  very  frequent. 

Atrojihic  Stages. — For  a  variable  length  of  time,  a  jear  at  least,  the 
palsied  or  paralyzed  muscles  gradually  regain  their  form  and  then* 
function,  but  sooner  or  later,  depending  on  the  grade  of  central  involve- 
ment, a  more  or  less  permanent  state  of  inactivity  is  reached — a 
residual  period  in  which  an  account  of  stock  may  be  taken.  In  this 
stage  one  meets  with  the  definite  atrophies,  the  beginnings  of  the 
various  deformities,  changes  in  the  bones  and  joints,  and  the  residual 
secretory  and  trophic  anomalies. 

The  various  deformities  belong  more  in  the  domain  of  orthopedics 
and  caiuiot  be  taken  uj)  liere.  Pes  equinus,  pes  calcaneus,  pes  valgus, 
pes  varus,  hyperextension  of  the  knee-joint,  genu  \'algum,  genu 
varum,  scolioses,  kjqjhoses,  lordoses,  torticollis,  flail  arm,  etc.,  are 

'Nervous  and  Mental  Disease  Monograph  Series  No.  7,  New  York,  1910. 


452 


LESIONS  OF  THE  SPINAL  CORD 


among  the  more  common  residual  deformities.  Seeligmiiller's  mono- 
graph, already  cited,  treats  of  them  at  great  length,  also  consult 
monographs  of  Ruhrtih  and  Mayer,  Lovett  and  ^'ulpius. 

The  permanent  vasomotor  disturbances  are  chiefly  cold  extremities 
and  cyanosis.    Dryness  of  the  skin  is  frequent. 

2.  Acute  Ascending  Form.^ — Landry's  Paralysis.^ — Landry,  in  1859, 
described  an  acute  ascending  paralysis,  which  later  study  has  shown  to 
be,  for  the  most  part  at  least,  a  true  polioencephalomyelitis.  In  certain 
cases  it  is  a  neuritis.  Rabies  also  has  a  Landry  paralysis  type.  Here 
the  palsy  shows  itself  usually  first  in  the  lower  extremities,  then  the 
muscles  of  the  hip,  the  abdomen,  the  thorax,  and  the  cranial  nerves 
are  involved,  and  death  generally  occurs  through  implication  of  the 
cardiac  and  respiratory  synapses.  Death  takes  place  in  from  four 
to  five  days,  usually  with  clear  consciousness,  or  slight  coma.  The 
sensibility  is  usually  intact,  or  only  slightly  dulled. 

Occasionally  the  patients  recover,  and  then  show  the  residuals  of 
a  severe  spinal  poliomyelitic  type,  with  mixtiu-e  of  bulbar  or  pontine 
features. 

A  descending  form  is  also  to  be  distinguished.  This  is  much  rarer. 
Here  the  bulbar  symptoms  develop  early,  and  the  spinal  extension 
shows  later. 

The  majority  of  the  fatal  cases  of  epidemic  polioencephalomyelitis 
show  the  type  of  a  Landry  paralysis. 


Fig.  267. — Eye  palsies,  poliomyelitis. 
(P^rauenthal.) 


Fig.  268. — Facial  nerve  palsy,  polio- 
myelitis.    (Frauenthal.) 


3.  Bulbar  and  Pontine  Forms. — Here  the  features  that  stand  in 
the  foregrounfl,  either  as  initial  or  as  residual  conditions,  are  the 
cranial  nerve  palsies.  A  large  number  of  the  patients  with  poliomyelitis 
show  some  cranial  nerve  complications,  but  when  these  are  the  chief 


ACUTE  POLIOENCEPHALOMYELITIS         .  453 

features,  and  the  spinal  palsies  are  the  minor  complicating  factors, 
then  one  speaks  of  the  bulbar  and  pontine  types. 

]\Iany  of  these  patients  show  isolated  palsies — others  have  two  or 
more  cranial  nerve  involvements.  The  facial  is  oftenest  implicated 
(10  per  cent.),  next  the  hypoglossal.  These  are  usually  unilateral 
although  ]\Iedin  has  described  a  bilateral  facial  palsy.  Eye-muscle 
palsies  are  infrequent;  the  third  and  fourth  less  often  than  the  sixth 
(Wickmann,  ^Midler) . 

Ptosis,  ophthalmoplegia  interna,  externa,  nystagmus  are  among 
the  rarities.  Pupillary  disturbances  are  not  infrequent,  according 
to  Lundborg.  Here  both  light  and  accommodation  reflexes  are  inter- 
fered with,  ^'ery  rarely  one  observes  amam'osis  with  optic-nerve 
involvement.  The  presence  of  choked  disk  in  meningitis  is  an  impor- 
tant differential.  The  IXth,  Xth,  Xlth  are  involved,  usually  in  the 
fatal  cases,  occasionally  unilateral  phar\Tigeal  and  laryngeal  palsies  are 
residual  conditions.  Speech  disturbances  may  be  present,  but  are 
infrequent.  Complete  central  deafness  has  been  observed.  Midbrain 
involvements  with  peculiar  tremors,  vertigoes,  forced  movements, 
athetoid  movements,  paralysis-agitans-like  movements,  rotary  move- 
ments, ataxias,  cerebellar  ataxias,  cerebellar  gait,  etc.,  are  among  the 
curiosities.  Howe^  has  described  an  interesting  thalamic  s\Tidrome 
case. 

4.  Encephalitic  or  Cerebral  Form. — Striimpell  called  particular  atten- 
tion to  the  possibility  of  a  pure  encephalitic  form  of  the  disease,  thus 
widening  the  conception  from  a  poliomyelitis  to  a  polioencephalo- 
myelitis.  Medin's  valuable  study  confirmed  his  teaching,  and  the 
investigations  of  recent  years  have  still  further  amplified  the  find- 
ings. Here  the  inflammatory  reaction  spreads  throughout  the  entire 
cortex,  as  in  the  cord.  The  central  and  frontal  g^Ti,  the  basal  ganglia, 
the  internal  capsule,  and  centrum  ovale  are  most  frequently  involved. 

Here,  in  addition  to  headache,  stupor,  and  convulsions  one  encounters 
spastic  palsies,  hemiplegic  or  diplegic  in  tN'pe,  usually  associated  with 
bulbar  palsies. 

These  cerebral  forms  are  probably  rare,  and  often  fatal.  They 
are  also  rare  in  experimental  monkey  poliomyelitis.  ^Nliiller  is  inclined 
to  regard  the  spastic  palsies  that  occasionally  occur  as  due  to  pontine 
rather  than  to  motor  cortex  involvement,  whereas  Wickmann  lays 
particular  stress  upon  the  probabilities  of  their  cortical  origin.  Some 
patients  with  lethargic  encephalitis  are  poliomyelitic  in  origin,  others 
result  from  other  infections,  notably  influenza,  whereas  Strauss  has 
described  a  special  type  of  lethargic  encephalitis  which  he  regards  as 
a  distinct  type. 

5.  Cerebellar  Forms.— Medin  described  forms  in  which  the  patients 
showed  ataxia  in  walking,  with  staggering  or  ataxic  gaits,  and  others 
behaving  like  Friedreich's  disease  of  acute  onset.  These  forms  are 
closely  allied  to  the  bulbar  and  pontine  types,  and  also  may  be  oc- 

1  Neurol.  Bull.,  1919.  vol.  ii. 


454  L'lJSIOKS  OF  THE  SPINAL  CORD  ^^ 

casioned  by  extension  of  the  disease  to  the  cerebelhim.     The  latter 
structure  is  ahuost  ahvays  involved  to  some  extent  in  this  disease. 

G.  Meningitic  Forms. — Here  meningeal  symptoms  occupy  the  fore- 
ground. Headache,  Aomiting,  pain  in  the  neck  with  stiffness,  Kernig's 
sign,  stiffness  of  the  back,  opisthotonos,  convidsions,  strabismus, 
somnolence,  and  imconsciousness  are  present.  These  cases  either  then 
de^'elop  marked  spinal  and  bidbar  symptoms  of  the  ordinary  type,  or 
the  symptoms  recede  with  either  minor  residuals,  eye  palsies,  etc.,  or 
go  on  to  complete  recovery. 

7.  Polyneuritic  Forms. — ^The  study  of  recent  epidemics  has  shown 
the  great  frequency  with  which  pain  is  found  in  the  initial  history. 
In  many  cases  there  are  painful  nerve  trunks,  with  Lasegue's  phenom- 
enon, and  great  hyperalgesise  over  the  entire  body,  resembling  polyneu- 
ritis. Anatomically,  however,  marked  neuritic  changes  are  wanting. 
These  cases  Wickmann  prefers  to  call  nem-itis-like. 

8.  Vegetative  Types.- — In  many  of  the  patients  with  severe  mesen- 
cephalic or  spinal  invoh'ements  the  vegetative  nuclei  are  implicated 
with  the  \\'ell-known  trophic  and  secretory  disturbances.  There  are, 
however,  a  number  of  cases  in  which  some  of  these  nuclei,  from  the 
ciliary  ganglion  down  to  the  sacrum,  are  alone  picked  out  by  the  patho- 
logical process.  There  result  a  great  group  of  minor  cases  with  residual 
difficulties  left  in  the  vegetative  arc  reflex  fmictionings.  Some  of  these 
are  confused  with  so-called  neurotic  or  fmictional  cases. 

9.  Incomplete  or  Minor  Forms. — The  study  of  the  recent  epidemics 
has  shown  that  in  a  number  of  patients  the  illness  began  with  the 
characteristic  symptoms  of  poliomyelitis,  and  then  went  on  to  recovery 
without  any  palsies.  In  others  again,  widespread,  though  mild  palsies 
with  hypotonia  developed  and  complete  recoveries  occurred  within  a 
short  time.  Wickmami  brought  these  facts  into  prominence,  and 
showed  that  these  were  to  be  regarded  as  abortive  cases.  The  most 
frequent  forms  under  which  these  cases  develop  are  (1)  that  of  a  mild 
meningitic  type  with  the  usual  prodromal  signs,  associated  with  the 
neck  symptoms,  stiffness,  pains,  sometimes  opisthotonos  and  the  like; 
(2)  cases  with  the  symptoms  of  a  general  infection  only;  (3)  cases 
ruiming  a  course  like  an  influenza;  (4)  cases  with  marked  gastro- 
intestinal signs. 

Wickmann  has  estimated  that  at  least  15  per  cent,  of  all  the  cases 
can  be  grouped  mider  this  rubric,  while  Miiller  believes  them  to  occur 
much  oftener,  indeed,  more  often  than  the  usual  type.  Their  signifi- 
cance in  the  epidemiology  of  the  disease  is  great,  because  it  is  highly 
probable  that  its  spread  may  be  conditioned  by  these  ambulatory 
abortive  cases.  They  are  more  frequent  in  children,  but  may  also 
be  present  in  adults.  If  Miiller's  stand  be  correct,  viz.,  that  they  occur 
oftener  than  the  well-developed  forms,  their  importance  from  this 
standpoint  is  enormous.  The  question  of  rarity  of  the  disease  after 
puberty  may  be  solely  conditioned  by  the  fact  of  previous  minor 
attacks  in  many  individuals.  IMiiller  states  that  in  the  study  of  small 
epidemics  he  has  found  that  in  the  abortive  cases  there  is  a  marked 


ACUTE  POLIOENCEPHALOMYELITIS  455 

tendency  to  show  the  same  symptoms  in  their  epidemic  extension. 
Thus  gastro-intestinal  cases  give  rise  to  gastro-intestinal  cases,  respira- 
tory to  respiratory,  meningeal  to  meningeal,  etc. 

Diagnosis.- — Sporadic  and  epidemic  polioencephalomyelitis  are  prob- 
ably conditioned  by  the  same  etiological  factors.  If  an  epidemic  is  in 
progress,  particular  attention  should  be  given  to  all  acute  infections, 
whether  they  show  marked  palsies  or  not,  and  careful  examination 
be  made  of  nerve  tenderness,  hypotonias,  tendon  reflexes,  and  limb 
motility.  INIost  cases  develop  either  in  the  guise  of  general  infections 
with  temperatiu-e,  or  with  distinct  local  symptoms,  either  in  the  respi- 
ratory or  gastro-intestinal  tracts,  or  in  the  meninges.  Influenza,  poly- 
neuritis, angina,  bronchitis,  gastro-enteritis,  typhoid  and  epidemic 
cerebrospinal  meningitis  come  into  review.  A  marked  general  h3T)er- 
esthesia,  and  a  distinct  pathological  tendency  to  perspiration  is  sig- 
nificant. The  careful  studies  of  Peabody,  Draper  and  Dochez  suggest 
the  general  blood  picture  of  an  acute  infection.  There  is  a  general 
polymorphonucleosis,  with  a  fairly  constant  lymphocyte  increase. 
30,000  white  cells  per  cu.  mm.  is  not  unusual,  the  pohnuclears  often 
using  10  to  15  per  cent.;  a  diminution  in  lymphoc}i;es,  15  to  20  per  cent, 
may  also  be  present.  Sleepiness,  to  drowsiness  of  the  children,  during 
the  day,  wakefulness,  and  fretfulness  at  night,  easy  fatigability,  weak- 
ness of  the  extremities,  loss  of  muscular  tonus,  especially  in  the  abdom- 
inal muscles,  with  meteorism  and  loss  of  the  abdominal  reflexes,  point 
to  poliomyelitis.  An  early  lumbar  puncture  will  resolve  many  of  the 
difficulties.  The  cerebrospinal  fluid  is  rarely  normal.  There  is  an 
increase  in  the  cells,  sometimes  to  high  numbers,  chiefly  70  to  80 
per  cent,  polynuclears.  Later  the  lymphocytes  are  increased  markedly, 
chiefly  the  mononuclears.  Influenza  is  separated  with  considerable 
difficulty  in  the  early  stages,  so  much  so  that  certain  observers  (Bors- 
trom)  have  held  that  poliomyelitis  is  nothing  but  a  several  neural  type 
of  influenza.  IMonkey  experimentation  by  Flexner  and  many  others 
has  disposed  of  parts  of  this  hypothesis.  Recent  epidemics  of  lethargic 
encephalitis  also  raise  this  point  of  poliomyelitis. 

Polyneuritis  also  offers  particular  difficulties.  This  is  rare  in  children 
apart  from  diphtheritic  neuritis,  and  is  usually  quite  symmetrical  in 
its  development.  The  time  needed  for  development  of  the  palsies  is 
longer,  the  pains  are  more  persistent,  there  are  usually  more  objective 
sensory  disturbances,  particularly  deep  sensibility,  and  early  edemas 
are  more  frequent. 

In  diphtheritic  neuritis,  cardiac  irregularities  are  the  rule,  in  polio- 
myelitis the  exception;  the  palsies  of  the  palate  are  further  charac- 
teristic in  the  former. 

Pure  neuritic-like  forms  of  poliomyelitis  are  sometimes  present, 
but  there  is  here  more  tendency  for  a  mild  dissociation  syndrome, 
diminution  of  pain  and  temperature  sensibility.  Neuritis  is  more  apt 
to  include  all  the  forms  of  sensibility,  or  gives  a  diminution  in  touch 
(epicritic)  with  an  increase  in  pain  sensibility.  In  very  small  childroU 
these  dift'erences  are  difficult  to  bring  out.     Williamson  lays  stress 


456 


LESIONS  OF   THE  SPINAL  CORD 


upon  the  loss  of  bony  sensibility  in  neuritis,  whereas  in  poliomyelitis  it 
is  rare. 

Further  differentials  from  Wernicke's  polioencephalitis  superior 
myelitis,  hematomyelia,  myotonia  congenita,  hysteria,  Parrot's  palsy 
in  hereditary  sv-philitics,  etc.,  must  be  sought  for  in  monographs. 


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Fig.  269. — Relative  severity  of  poliomyelitis  epidemic  of  1916  in  certain  areas,  judged 
by  the  comparative  incidence  and  death-rates  recorded.  (Courtesy  of  Dr.  Mathias 
Nicoll,  Jr.,  New  York  State  Board  of  Health.) 

In  the  meningitic  forms,  and  in  many  of  the  ordinary  spinal  cases 
the  separation  from  spinal  or  tuberculous  meningitis  is  very  difficult. 
The  lumbar  puncture  here  usually  clears  up  the  diagnosis.  Clinically, 
the  more  marked  mental  symptoms,  the  marked  degree  of  stiffness  of 
the  spinal  column,  Kernig's  sign,  and  at  times  papillary  edema,  ear 
complications,  and  herpes,  all  speak  for  meningitis.  In  tuberculous 
meningitis  the  spinal  fluid  findings  and  the  longer  course  usually 
establish  the  diagnosis. 

Prognosis. — The  older  teachings  that  the  disease  is  rarely  fatal  and 
always  shows  persistent  palsies,  must  be  modified  in  both  directions. 


ACUTE  POLIOENCEPHALOMYELITIS  457 

Many  fatal  cases  do  occur,  and  complete  recoveries  are  frequent. 
In  certain  epidemics  the  mortality  is  very  high  (42  per  cent.),  in  others 
only  10  per  cent.,  comiting  only  those  patients  with  evident  palsies. 
In  the  New  York  epidemic  of  1907  the  mortality  was  approximated 
as  5  per  cent.  In  that  of  1916  the  mortality  was  much  higher  (20 
per  cent.). 

If  the  very  mild  cases  are  included  the  percentage  falls  markedly. 
The  mortality  is  higher  in  the  older  patients,  as  high  as  50  per  cent, 
in  one  epidemic  (Lindner  and  Malley).  The  period  of  danger  lies 
usually  in  the  fourth  and  fifth  day  of' the  disease.  In  the  second  and 
third  week  bronchopnemnonia  is  a  dangerous  complication. 

As  for  complete  recoveries,  they  have  varied  from  10  to  50  per  cent, 
in  the  various  epidemics,  and  are  more  common  in  younger  children 
than  in  those  over  fourteen  years  of  age.     (See  Wickmann.) 

From  the  standpoint  of  electrical  prognosis,  the  older  views  are 
certainly  false.  The  whole  subject  is  in  need  of  entire  revision.  A  total 
loss  of  faradic  excitability  after  a  week  is  no  certain  criterion  of  perma- 
nent palsy,  as  has  been  taught  by  Oppenheim  and  others. 

Reparation  takes  place  most  rapidly  in  the  first  six  months,  but 
continues  throughout  a  year  or  more,  and  with  continuous,  rational 
treatment  weak  and  paralyzed  muscles  will  continue  to  improve  for 
many  years. 

Treatment. — Prophylaxis,  treatment  of  the  acute  stage,  and  of  the 
chronic  stages  are  to  be  distinguished. 

Prophylaxis. — Isolation  and  disinfection  are  as  yet  unprecise  in 
their  application.  The  mode  of  transmission  of  the  virus  is  as  yet 
unknown,  and  whereas  there  is  little  difficulty  in  isolating  the  severely 
attacked,  the  abortive  cases  are  not,  and  rarely  can  be,  properly  regu- 
lated. That  abortive  cases  do  carry  the  disease  seems  definitely  proved. 
The  proper  length  of  time  for  isolation  has  not  been  determined. 
Wickmann  regards  three  weeks  as  sufficient,  Miiller  eight  weeks.  As 
yet  the  data  do  not  permit  definite  counsels. 

The  virus  in  monkey  poliomyelitis  seems  to  have  been  found  in  the 
nasal  mucous  membranes  and  in  the  feces.  Hydrogen  peroxide,  1 
per  cent,  solution,  and  menthol  solutions  can  be  used  for  the  former, 
formaldehyde  or  carbolic  acid  for  the  latter.  Special  attention  should 
be  directed  toward  the  pocket  handkerchief. 

Rooms  may  be  disinfected  with  formaldehyde.  Preventive  inocula- 
tion, analogous  to  Pasteur  rabies  treatment,  may  become  practicable 
in  time. 

Acute  Stage. — Absolute  rest  in  bed  is  to  be  enforced,  and  if  an 
epidemic  be  in  progress,  children  with  mysterious  diarrheas,  pseudo- 
influenzas,  mild  neuritic  pains,  etc.,  with  fever,  should  be  kept  in  bed, 
even  after  they  appear  to  have  recovered.  The  patients  who  have 
been  sick  for  a  couple  of  days,  and  who  get  up  and  around,  and  are 
then  suddenly  stricken  down,  are  in  reality  very  lumierous.  Foresight 
here  cannot  be  overvalued. 

Counter-irritation  to  the  spine,  by  mustartl  plaster  or  other  means; 


458  LESIONS  OF  THE  SPINAL  CORD 

prompt  catharsis,  first  by  enema  and  then  by  salme  cathartics,  should 
be  employed.  The  motor  restlessness  and  pain  are  best  controlled  by 
analgesics,  and  the  various  salicylate  preparations.  Codeine  may  be 
employed,  but  its  inhibitory  action  upon  the  bladder  should  not  be 
overlooked.    Lumbar  puncture  is  often  of  signal  service. 

Diaphoresis  by  hot  packs  is  helpful  for  the  pains,  muscular  soreness, 
and,  possibly,  in  aidmg  elimination.  Frequent  treatment  by  free  use  of 
deep,  warm  baths,  102°  to  104°  F.,  is  highly  desirable.  The  warm  water 
relieves  the  sense  of  pain  and  the  irritability  markedly,  and  is  greatly 
appreciated  by  the  patient.  The  baths  can  be  repeated  every  three 
or  four  hours;  the  time  in  the  bath  is  from  ten  to  twenty  minutes. 
On  taking  the  child  from  the  bath,  he  should  be  rolled  in  a  blanket  and 
dried  in  bed.    Continuous  warm  baths,  94°  to  96°  F.,  are  worth  trying. 

In  the  later  stages  the  bath  treatment  is  invaluable  for  straightening 
out  the  contracted  limbs  and  aiding  in  active  motion. 

Urotropin  may  be  administered.  It  is  thought  to  reduce  to  formal- 
dehyde in  the  cerebrospinal  fluid  in  sufficient  amounts  to  act  as  an 
antiseptic.    The  hypothesis  lacks  definite  experimental  confirmation. 

The  use  of  the  serum  of  those  who  have  had  an  attack  of  poliomye- 
litis, preferably  recently,  has  seemed  to  be  of  service  in  some  patients. 
The  best  results  have  seemed  to  follow  the  combined  interspinal  and 
intravenous  injections  of  the  serum.  It  is  of  service  only  in  the  pre- 
paralytic stages.  Horse  sera  are  being  evolved.  What  their  value 
may  be  is  not  yet  decided.^ 

Certain  very  severe  and  apparently  fatal  cases  may  be  saved  by 
continuous  artificial  respiration  with  oxygen  administered  by  pressure 
apparatus,  and  in  low  blood-pressure  and  in  threatened  respiratory 
paralysis  cases,  adrenalin  by  mouth,  injection,  or  by  rectum  has 
definite  value.  3  c.c.  to  1  to  1000  solution,  every  four  hours,  diluted 
with  2  c.c.  salt  solution  by  intraspinous  injection  is  Meltzer's  recom- 
mendation. 

Chronic  Stages. — After  the  acute  stage  has  passed  there  is  the  long, 
hard  stage,  lasting  for  weeks  or  months  or  years,  of  partial  or  total 
paralysis,  great  weakness,  extreme  tenderness,  nervous  irritability,  and 
muscular  pains,  with  gradually  developing  deformities. 

The  diet  should  be  full  and  regularly  administered.  A  careful 
inventory  should  be  made  of  every  affected  muscle,  both  in  terms  of 
its  functional  capacity,  and  its  electrical  excitability,  the  milliamperes 
necessary  for  bringing  about  contraction  being  carefully  noted.  Full 
measurements  of  the  limbs  should  be  taken.  Careful  note  must  be 
taken  of  the  position  of  the  limb  in  bed,  and  appropriate  support  given 
to  relieve  pain,  avoid  stretching,  and  diminish  contraction.  Bed- 
sores, sore  heels,  elbows,  etc.,  need  very  careful  treatment. 

Chief  reliance  is  now  laid  upon  massage,  passive  motions  and 
resistance  motions.  Here  the  deep,  warm  bath  is  invaluable,  serving 
to  help  the  motions  of  the  limbs,  and  to  straighten  out  the  contractions. 

1  Rurah,  loc.  cit. 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES  459 

Movements  in  water  are  more  easily  performed,  and  small  children  can 
play  in  the  deep  tub  for  hours.  Special  exercises  should  be  planned, 
both  for  the  water  and  in  bed.  Special  calisthenics  must  now  be  devel- 
oped, dependmg  upon  the  muscle  group  involved.  Especial  emphasis 
must  be  laid  upon  the  patient's  conscious  and  voluntarj^  participation 
in  these  exercises.  The  best  method  to  carry  these  out  is  by  the 
Mensendieck  system  which  is  a  scientific  type  of  muscle  kinetics. 

Electricity  is  of  almost  no  service  whatever.  The  physiological 
stimulus  of  muscles  comes  from  the  cerebral  cortex.  Hence  muscle 
exercises,  controlled  by  the  patient's  active  desires,  not  formal  calis- 
thenics, is  the  best  type  of  training. 

Particular  attention  must  be  directed  to  the  mental  life  of  the  child. 
He  is  apt  to  be  morose,  reticent,  shy,  and  resentful,  becomes  very 
sensitive  if  his  deformity  be  marked,  and  selfishness  and  exactingness 
develop  easily  if  pampered  because  of  his  weakness. 

In  the  later  stages,  and  for  those  old  enough,  swimming  is  the  best 
exercise. 

The  correction  of  the  deformities  is  a  matter  for  the  orthopedic 
surgeon.  IVIechanical  aid  should  be  given  as  soon  as  possible,  if  it 
carries  out  a  progressive  therapeutic  principle.  Operative  procedures 
should  be  conservatively  considered,  and  not  used  too  early.  These 
patients  make  wonderful  recoveries  unaided,  or  by  the  persistent  use 
of  the  Mensendieck  exercises.  Many  operative  procedures  are,  how- 
ever, imperative.  Ner^^e  splicing,  and  tendon  splicing  have  their 
special  indications. 

Electrical  therapy  is  of  use  only  as  a  bridge  to  gap  the  period  after 
the  first  onset  until  such  time  as  definite  willed  movements  can  be 
started. 

Drug  therapy  is  of  purely  sjTnptomatic  value.  Iron,  strychnine, 
calcium  are  the  most  useful  remedies,  and  meet  special  indications. 

PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES. 

The  spinal  motor  neuron  is  differentiated  into  three  parts,  termed 
the  anterior  horn  cell,  the  motor  nerve  fiber,  and  the  muscle  plate. 
It  would  be  a  great  advance  toward  the  simplification  of  the  vexed 
questions  regarding  this  group  if  a  clinical  di^•ision  could  be  made  in 
strict  accordance  with  the  pathological  alterations  of  these  parts 
To  a  certain  extent  only  is  this  justified.  For  categorical  purposes 
then  one  can  distmguish:^ 

1.  The  progressive  nuclear  atrophies,  or  myelogenous  or  myelo- 
pathic types:  Etiology:  Heredity,  trauma,  cold,  toxins,  infections. 
Anatomy:  Primary  degeneration  of  the  anterior  honi  cells,  with 
secondary  fiber  or  muscle  changes.  Forms:  Infantile,  Iloft'mann- 
Werdnig;  adult,  Aran-Duchenne ;  mixed  forms. 

2.  The   neural   neuritic,    or   spinal   neuritic   atrophies:     Etiology: 

'Kiigelgen:  Beitrage  zur  neuralcn  progressiven  Muskeltrophien,  Arch.  f.  Psych., 
1909,  xlv,  944. 


460  LESIONS  OF   THE  SPINAL  CORD 

Heredity.  Anatomy:  Primary  degeneration  of  the  motor  nerve  fibers 
with  secondary  ganghon,  cord,  and  muscle  changes.  Forms:  Pero- 
neal-arm  type  (Charcot-Marie-Tooth) ;  tabetic  type  (Dejerine-Sottas) ; 
peroneal  type  and  arm  type  (Sainton  and  Haenel). 

3.  The  muscular  dystrophies  or  myogenic  types.  Etiology:  Heredity 
Possible  vegetative  nerve  disorder  (endocrinopathic).  Anatomy: 
Primary  changes  in  muscles.  Forms:  Hereditary  (Leyden-Mobius) ; 
juvenile  pseudohypertrophy  (Landouzy-Dejerine) ;  and  mixed  forms 
(Erb-Zimmerlin). 

The  separation  of  these  forms  has  occupied  the  attention  of  neurol- 
ogists since  about  1849  when  Duchenne  first  attacked  the  problem  of 
dismemberment  of  this  large  group  with  muscular  wasting. 

Clinicians  for  many  centuries  had  described  progressive  muscular 
wasting.  Pictiu-es  and  images  in  stone  and  wood  of  the  muscular 
atrophies  and  muscular  dystrophies  dating  from  the  fifteenth,  sixteenth 
and  seventeenth  centuries  are  in  existence.  Van  Sweiten,  Aberccombie, 
and  others  gave  general  descriptions.  This  group  was  first  broken 
into  by  Duchenne,  in  1849,  by  the  loose  description  of  a  special  type, 
which  a  year  later  Aran  (1850)  supplemented.  Cruveilheir,  in  1853, 
and  Luys,  in  I860,  sharpened  the  picture  somewhat  by  their  demonstra- 
tion of  the  exclusive  implication  of  the  anterior  horns.  In  1865 
Charcot  isolated  the  amyotrophic  lateral  scleroses  within  the  group. 
Duchenne  himself  had,  in  1853,  also  set  aside  the  pseudohypertrophies, 
the  muscular  featiu"es  and  varieties  of  which  were  later  demonstrated 
by  Eulenberg  (1866)  Charcot,  Leyden,  and  Dejerine.  Wachsmuth, 
in  1864,  recognized  the  bulbar  forms.  Finally,  Duchenne  also  called 
attention  to  the  presence  of  sensory  anomalies  in  certain  of  his  1853 
studies  on  the  atrophies;  these  were  for  the  most  part  syringomyelias, 
which  Kahler  and  Schultze  separated  definitely  in  1882.  Another 
small  group  were  the  neuritic  atrophies,  first  clearly  recognized  by 
Dumesnil  (1864).  Thus  it  took  thirty  years  for  the  sorting  out  of 
this  medley  of  muscular  atrophies;  the  groups  as  they  are  at  present 
recognized,  at  least  two  of  which,  syringomj'elia  and  amyotrophic 
lateral  sclerosis,  being  entirely  set  outside  of  the  muscular  atrophies 
yer  se.  One  result  was  that  the  original  disorder  of  Aran-Duchenne 
was  so  much  reduced  that  finally  Marie,  in  1894,  tried  to  sweep  it 
away  entirely,  but  his  iconoclasm  had  not  yet  been  justified. 

In  this  volume  the  muscular  dystrophies  are  removed  entirely  and 
treated  as  vegetative  nerve  disorders  of  the  muscle  (see  Part  I). 

Group  1.    The  Progressive  Nuclear  Atrophies:  (1)  Spinal,  (2)  Bulbar, 
and  (3)  Mesencephalic  Forms. 

1.  Spinal. — Three  main  forms  are  here  to  be  recognized,  (a)  Chronic 
poliomyelitis  per  se,  with  lesions  limited  primarily  to  the  anterior  horn 
cells;  (6)  Aran-Duchenne's  disease,  progressive  spinal  muscular  atrophy 
and  (c)  infantile  hereditary — Werdnig-Hoft'mann. 

The  two  former  types  are  much  alike,  and  many  authors  maintain 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES 


461 


their  identity.  They  are  here  considered  separately,  since  such  a 
course  seems  justified  chnically  if  not  pathologically.^ 

(a)  Chronic  Poliomyelitis. — Here  are  grouped  those  rare  subacute 
or  chronic  muscular  atrophies  usually  occurring  in  the  late  years 
of  life,  in  which  certain  toxic  agents  seem  to  account  for  the  disorder. 
Such  toxic  factors  are  lead,  diabetes,  syphilis,  and  other  unknown 
toxemias.  Heredity  is  sometimes  present.^  Severe  shell  explosion 
injuries  may  cause  it.'^ 

The  anatomical  lesions  are  a  progressive  destruction  of  the  anterior 
horn  cells,  with  chronic  vascular  alterations. 


Fig.  270. — Chronic  poliomyelitis  showing  atrophy  of  hands. 

Syviptoms. — There  is  a  progressively  developing  flaccid  paralysis, 
with  antecedent  weakness  and  diminution  or  loss  of  tendon  reflexes, 
but  without  sensory  or  trophic  signs.  The  nerve  trunks  are  not  ten- 
der or  swollen.  Fibrillary  twitching  appears,  the  myotatic  irrital)ility 
is  increased,  reaction  of  degeneration  is  present.  There  are  no  disturb- 
ances of  the  rectal,  vesical,  or  genital  functions,  and  the  bulbar  nuclei 
are  rarely  involved. 

The  localization  may  vary;  the  legs  or  arms  first  showing  weakness 
or  atrophy,  sometimes  one  arm  or  both,  one  arm  and  one  leg,  or  both 
legs.    The  atrophy  and  palsies  usually  take  place  slowly,  with  at  times 


'  MarburK:  Handbuch.  d.  Neurologic  1911,  ii,  280,  for  complelc  literature;  Love- 
green,  Zur  Kenntnis  der  Pol.  acuta  und  chronica,  Kargcr,   1904. 

'^Dana:  Familial  Form  of  Progressive  Spinal  Myopathy,  Jour.  Nerv.  and  Ment. 
Dis.,  1914,  xli,  681. 

'  See  Aran-Duchenne  section. 


462 


LESIONS  OF  THE  SPINAL  CORD 


periods  of  more  rapid  development,  and  also  stationary  periods  during 
which  no  advance  is  made  for  years,  or  the  patient  slowly  recovers. 

The  distribution  of  the  atrophic  muscles  is  characteristic.  Certain 
muscle  groups  are  spared.  The  arrangement  is  segmental,  not  radicu- 
lar. The  triceps  may  escape  with  all  the  other  muscles  of  the  arm 
attacked,  or  the  flexors  of  the  fingers  may  be  intact.  In  the  legs  the 
tibialis  anticus  may  stand  out  unimpaired.  The  course  is  usually 
very  chronic,  lasting  years.  Subacute  onsets  are  more  favorable. 
Some  patients  recover,  especially  those  with  suspicion  of  a  radiculitis 
or  a  neiu-itis.  In  the  progressive  cases  the  patients  at  times  develop 
bulbar  symptoms,  and  die  of  aspiration  pneumonia,  or  weakness. 


Fig.  271. — Primary  nuclear  atrophy. 
(G.  M.  Hammond.) 


Fig.  272. — Primary  nuclear  atrophy. 
(G.  M.  Hammond.) 


Treatment. — Rest,  hydrotherapy,  gentle  massage,  electricity,  over- 
feeding are  the  chief  indications.    Syphilitic  cases  need  specific  therapy. 

{}))  Aran-Duchenne  Type. — Progressive  Muscular  Atrophy. — Etiology. 
— Here  heredity  may  play  a  part  in  the  development  of  a  familial  form, 
seen  in  infancy,  and  also  in  adults  (Bernhardt).  It  may  follow  acute 
poliomyelitis.  Other  factors,  as  toxemias,  exposure  to  cold  and  wet, 
overexertion,  are  not  definitely  established.  In  the  recent  world  war 
s.hell  concussions,  probably  causing  minute  hematomyelias,  possibly 
vacuum  caisson  changes,  have  resulted  in  diffuse  disseminated  types  of 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES  463 

muscular  atrophy.^  Syphilis  may  also  be  an  etiological  factor.-  Occu- 
pation atrophies  are  at  times  incorrectly  included  here.  Pathologically 
there  is  degeneration  of  the  anterior  horn  cells  and  fibers,  with  some 
secondary  degenerations  in  the  anterolateral  columns. 

It  is  a  rare  disorder.  The  syndrome  begins  very  slowly,  the  upper 
extremities  are  attacked,  and  rarely  the  lower.  The  smaller  muscles 
of  the  hand  are  initially  affected.  Weakness,  atrophy  and  flaccid 
paralysis  are  the  sequence.  Fibrillary  twitchings  accompany  the 
atrophy  and  there  is  reaction  of  degeneration  in  the  muscles  involved. 
Sensory  and  trophic  disturbances  are  lacking  and  the  tendoa  reflexes 
are  diminished  or  lost.  Early  overexcitability  of  the  tendon  reflexes 
may  be  noted  in  both  of  these  forms. 

Oppenheim  has  called  attention  to  the  tendency  for  these  atrophies 
to  jump  from  one  muscle  group  to  another.  The  atrophy  is  very 
gradual;  the  disease  advancing  slowly  for  years.  Remissions  occur, 
but  recoveries  are  very  unusual. 

The  distribution  of  the  affected  muscles  varies  considerably.  Death 
usually  results  from  paralysis  of  the  muscles  of  respiration. 

Diagnosis. — The  separation  of  these  two  forms  is  at  times  impossible. 
There  is  a  tendency  for  the  former  to  advance  more  rapidly,  to  involve 
the  lower  limbs,  to  advance  to  the  medulla,  to  show  longer  remissions, 
and  to  recover.  Further,  there  is  a  greater  tendency  to  segmental 
distribution  in  the  muscle  groups  affected  in  the  former  type,  with 
marked  loss  of  power  followed  by  atrophy.  In  the  latter  t\T)e  the 
atrophy  seems  to  precede  the  palsies,  and  the  patients  are  often  very 
adept  with  their  residual  muscles. 

The  sensory  disturbances  of  occupation  atrophies,  of  neuritis,  of 
syringomyelia  should  exclude  these,  while  the  increased  tendon  reflexes 
of  amyotrophic  lateral  sclerosis  eliminates  that  disorder.  Radiculitis 
needs  separation.  Here  the  atrophies  are  apt  to  be  unilateral,  and  are 
radicular  in  distribution.  There  is  a  low-grade  neuritis,  and  the 
Dejerine-Klumpke  syndrome  appears  in  the  cervical  type. 

Treatment. — This  may  be  treated  as  the  preceding  affection,  but 
such  therapy  seems  of  little  avail.  Strychnine,  arsenic,  and  fats  are 
indicated.  Newer  ideas  must  be  gained  if  any  effective  control  of 
this  disorder  is  to  be  hoped  for;  the  older  methods  are  useless. 

(c)  Infantile  Hereditary  Forms.  (Werdnig^-Hoff mann^ . — ^These  are 
rare  hereditary  types,  beginning  in  infancy,  first  described  in  1891. 
The  disease  begins  gradually,  usually  in  the  pelvic  girdle  and  thighs 
(ileopsoas,  quadriceps  femoris).  Later  the  back,  neck,  and  shoulder 
girdle  are  involved.  Finally  the  distal  extremities  are  involved.  The 
intercostals  and  diaphragm  are  often  affected.  Occasionally  the  bulbar 
muscles  atrophy.  The  atrophies  are  usually  symmetrical,  often  asso- 
ciated with  apparent  hypertrophy  (adiposis). 

•  Levi:  Rev.  Neur.,  1918,  p.  476.  Francois:  Rev.  Neur.,  xxiii,  2,  494.  Claude: 
Presse  m6d.,  October  11,  1915;  Soc.  Mc'd.  d.  Hop..  February  11,  1916. 

^Souques:  Nouv.  icon,  de  la  Salpfetrifere,  1915,  xxvii,  297.  Lerouge:  Thfese  de 
Paris,  1913. 

3  Archiv  f.  Psychiatric,  22,  26.  *  Deutsch.  Ztschr.  f.  Ncrvenh.,  3,  10,  18. 


464  LESIONS  OF  THE  SPINAL  CORD 

There  is  gradually  increasing  paresis  and  loss  of  skin  and  tendon 
reflexes.  Fibrillary  twitching  seems  to  be  absent  and  a  peculiar 
trembling  of  the  fingers  is  described.  Scolioses,  talipes,  and  other  con- 
tracture states  develop.  These  little  patients  often  acquire  unique 
modes  of  locomotion.  Faradic  currents  are  borne  better  than  galvanic 
currents,  but  reaction  to  both  is  diminished  or  lost.  The  course  is 
usually  progressive,  rarely  stationary. ^    Mental  reduction  is  not  usual. 

2.  Bulbopontine  Types.  Chronic  Progressive  Bulbar  Palsies.— In 
these  forms  the  progressive  atrophy  is  limited  to  the  muscles  of  the 
face,  tongue,  palate,  and  larynx. 

Etiology. — Nothing  is  known  of  the  causative  factors.  Certain 
toxic  factors — lead,  syphilis — have  seemed  to  play  a  role  at  times; 
constantly  recurring  electrical  shocks  were  a  factor  in  one  patient 
personally  observed;  the  majority  give  no  clue  as  to  etiology.  A  few 
cases  are  observed  in  childhood,  still  fewer  in  adult  manhood;  the 
majority  occur  after  thirty-five  years. 

Symptoms. — There  is  a  slowly  progressive  weakness  of  the  tongue  and 
muscles  of  the  cheeks  and  lips,  followed  by  fibrillary  twitchings  and 
slowly  progressive  atrophy.  Speaking  becomes  fatiguing,  and  slight 
changes  in  the  voice  become  apparent.  In  eating  the  patients  find 
they  must  use  their  fingers  in  dislodging  food  from  behind  the  teeth  in 
the  cheek.  Gradually  increasing  difficulty  in  swallowing  is  observed, 
and  difficulties  in  breathing  appear.  Finally  the  speech  becomes 
lalling,  dysarthric,  the  lingual  letters  d,  t,  I,  r,  n,  s,  are  first  slurred 
over,  then  the  labial  letters  b,  p,f,  m,  w,  o,  v.  The  laryngeal  weakness 
causes  hoarseness,  monotony,  and  finally  aphonia.  The  pharyngeal 
muscles  and  those  of  the  tongue  do  not  act  in  concert,  and  liquid  food 
regurgitates  through  the  nose. 

The  face  becomes  atrophied  below,  the  lips  thin  and  folded.  The 
tongue  is  atrophied,  wrinkled  and  tremulous  and  finally  lies  inert  on 
the  floor  of  the  mouth.  Ihe  patients  cannot  protrude  the  lips,  nor 
whistle.  The  temporals  and  masseters  also  beccme  aft'ected,  and  the 
movements  of  the  jaw  become  impossible.  The  masseter,  pharyngeal, 
and  vomiting  reflexes  are  absent.  The  sensibility  is  intact.  The  upper 
face  region,  including  the  levator  palpebrce,  is  rarely  affected. 

Vasomotor  disturbances  are  occasionally  observed.  Vagus  involve- 
ment causes  cardiac  irregularity. 

The  usual  progression  is  from  the  tongue  to  the  lips,  then  to  the  other 
facial  muscles,  and  finally  to  the  fifth,  and  hypoglossal  and  vagus 
muscles.  An  apparent  increase  in  saliva,  an  annoying  symptom,  is 
largely  dependent  upon  the  inability  to  swallow. 

The  I  sual  course  extends  over  four  or  five  years,  death  taking  place 
most  frequently  from  pneumonia  or  from  inanition. 

Pathology. — Here  one  finds  changes  in  the  bulbar  nuclei,  precisely 
analogous  to  those  found  in  progressive  spinal  nuclear  atrophy.  The 
corticobulbar  tracts  may  be  involved  somewhat,but  rarely  to  the  degree 

1  Late  literature.  Batten:  Lancet,  June  3,  1911.  Senator:  Charite  Annalen,  1902. 
Lange:     Deutsch.  Ztschr.  f.  Nervenh.,  1910,  p.  40. 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES  465 

found  in  amyotrophic  lateral  sclerosis,  yet  intermediary^  forms  are  to 
be  expected. 

Diagnosis. — Unusual  types  of  gliosis,  of  multiple  sclerosis,  tumors, 
gummata,  arterioslcerosis,  general  paresis  may  cause  somewhat 
similar  pictures  at  first,  but  these  also  soon  show  other  symptoms, 
indicating  that  the  lesion  is  not  confined  to  the  nuclear  structures. 
Pseudobulbar  palsy  shows  palsied  muscles,  still  electrically  reflexly 
excitable,  without  atrophies.  Myasthenia  gravis  shows  the  t>T)ical 
electrical  myasthenic  reaction. 

Prognosis. — ^Absolutely  bad,  save  m  syphilitic  cases. 

Treatment. — ^Thus  far  none  is  known.  One  should  always  be  on  the 
lookout  for  specific  causes  of  the  s^Tidrome,  especially  in  the  initial 
stages.    Here  a  nihilistic  therapy  will  be  recognized  too  late  for  recovery 

Galvanization  has  seemed  to  help  the  swallowing  of  some  patients. 
Atropine,  hyoscyamus,  etc.,  or  other  related  drugs  can  be  used  to 
control  the  salivation,  and  the  pains  and  discomforts  of  coughing, 
dyspnea,  etc.,  relieved  by  narcotics.  Specific  therapy  is  valuable  in 
the  Wassermann  positive  cases. 

3.  Pontomesencephalic  Forms. — Chronic  Progressive  Ophthalmo- 
plegia.— Chronic  progressive  ophthalmoplegias  as  a  part  of  an  amyo- 
trophic lateral  sclerosis,  of  tabes,  of  general  paresis,  multiple  sclerosis, 
tumor  of  corpora  quadrigemina,  or  other  organic  disease  are  not  rare. 
As  pure,  chronic  nuclear  aiTections  they  are  infrequent.  L  thoff  credits 
them  as  high  as  14  per  cent,  of  the  chronic  ophthalmoplegias. 

There  is  beginning  weakness  of  the  eye  muscles,  usually  the  external 
rectus,  with  slight  internal  strabismus,  frequently  worse  at  night. 
Diplopia  is  not  usually  present,  because  of  the  gradual  development  of 
the  disorder,  and  its  symmetry-.  In  hereditary,'  tj'pes,  in  infants, 
binocular  vision  may  not  have  developed. 

The  internal  eye  muscles  are  usually  intact,  although  occasionally 
irregular  pupils  occur;  light-immobile  pupils  are  not  present  although 
loss  of  accommodation  movements  has  occurred.  Ptosis  is  not  infre- 
quent; usually  worse  on  one  side. 

In  a  completely  developed  case  the  Hutchinson  face  develops; 
bilateral  ptosis  with  inability  to  move  the  eyeballs. 

The  disorder  is  usually  progressive.  Starr  has  described  a  stationary 
condition,  and  some  patients  recover  entirely. 

Treatment. — When  a  Wassermann  test  has  revealed  a  positive 
reaction,  mercury  and  iodides  are  indicated;  otherv\'ise  general  tonics, 
strychnine,  iron,  arsenic,  and  general  dietetic  treatment  directed  to 
the  reduction  of  arteriosclerotic  changes.  Endocrinopathic  stigmata 
should  receive  careful  attention  and  glandular  therapy  attempted  if 
such  are  present. 

Group  2.    The  Neural,  Neuritic,  or  Spinal  Neuritic  Atrophies. 

Hard-and-fast  lines  cannot  as  yet  be  dra\m  between  the  neuritic 
atrophies  and  certain  nuclear  atrophies  on  the  one  hand,  nor  certain 
30 


466 


LESIONS  OF  THE  SPINAL  CORD 


dystrophies  on  the  other.  Jendrassik,  m  a  recent  monograph  (1911), 
speaks  of  them  as  "dystrophy  forms  with  degeneration  of  the 
peripheral  nerves." 

Many  forms  of  neural  atrophy  have  been  described.  The  most 
characteristic  are:  (1)  The  peroneal-forearm  type — Charcot-Marie- 
Tooth;  (2)  the  tabetic,  or  h^^pertrophic  interstitial  neuritic  type — 
Dejerme-Sottas,  Marie's  familial  form ;  (3)  the  peroneal  type — Sainton; 
(4)  the  arm  type — Haenel. 


Fig.  273. — Charcot-Marie-Tooth  disease.     Atrophy  of  the  legs  below  the  knees  and  of 
the  arms  below  the  elbows.     (Starr.) 


1.  Peroneal-forearm  Type. — Charcot,  Marie  (1886)  and  Tooth 
described  this  form  which  is  characterized  by  muscular  wasting  in  the 
distal  parts  of  the  extremities,  from  knee  and  elbow  outward.^ 

Etiology. — Heredity  is  the  chief  factor  recognized .^  Eichorst  found 
thirteen  cases  in  six  generations  and  Haenel  thirty-two  in  four,  while 

1  Rev.  de  med.,  1881,  1886;  Brain,  x,  243.  Spiller:  Jour.  Nerv.  and  Ment.  Dis.,  1907. 
Souques:     Nouv.  iconog.  de  la  Salpetrifere,  1915,  xxv,  175. 

2  See  Geneological  Tree  of  Twenty  Members  in  Maslianka:  Thfese  de^Paris,  1913,  and 
in  Souques:  loc.  cit.,  1915. 


PRIMARY  PROGRESSIVE  MUSCULAR  ATROPHIES  467 

others  have  found  only  one.    Syphilis  may  bring  about  a  very  similar 
picture.^ 

Symptoms. — The  feet  are  usually  first  involved.  There  is  early 
equmus  position  from  loss  of  power  of  extension  of  the  great  toe. 
Then  the  leg,  from  the  knee  do^^Tl,  thins  and  atrophies.  The  gait 
thereby  becomes  either  widespread  or  steppage.  A  similar  process 
begins  in  the  forearms,  usually  advancing  from  the  muscles  of  the  hand. 
Monkey  hand,  or  main  en  grifPe  develops.  Rarely  the  upper  extremity 
atrophy  precedes.  The  shoulder,  arm,  neck,  back,  hips,  and  thigh 
muscles  remain  intact. 


Fiu.  274. — The  ueurilie  form  of  muscular  atrophy.     (Spillcr.) 


Sensory  changes  are  usually  present,  and  slight  pains,  intermittent 
and  lancinating  in  character,  coming  and  going,  hyperesthesia,  par- 
esthesia, hj-pesthesia,  especially  to  cold,  are  present.  Painful  nerve 
trunks  are  occasionally  found.  Vasomotor  disturbances  are  frequent. 
Cokl,  marble-like  skin  with  increased  secretion  is  observ'ed. 

The  knee-jerks  are  often  initially  increased ;  later  they  are  dimhiished 
or  absent.  The  Achilles  reflex  is  usually  absent,  as  is  the  radial- 
periosteal  reflex. 

«  Levi:     Nouv.  iconog.  dc  la  Salpctru'rc,  1917,  xxviii,  260. 


4C^S 


LESIONS  OF  THE  SPINAL  CORD 


Reaction  of  degeneration  in  the  regions  affected  is  the  rule.  Fibril- 
lary contractures  and  muscle  unrest  are  constant,  often  persisting 
during  sleep.   Tremors  and  choreic-like  movements  are  not  uncommon . 

Course.^ — The  disease  usually  begins  in  childhood,  advances  slowly, 
usually  progressively,  sometimes  halting  for  long  periods. 

Pathology. — Since  Hoffmann's  description  (1889)  of  a  neuritis  in 
the  distal  peripheral  nerves,  this  disorder  has  passed  as  a  mixed  neu- 
ritic  atrophy,  but  later  Siemerling,  Gierlich,^  Kugelgen,^  Spiller,'  and 
others  have  shown  changes,  not  only  in  the  peripheral  nerves  and 


Fig.  275. — The  neuritic  form  of  muscular  atrophy.     (Spiller.) 

muscles,  but  that  there  were  extensive  changes  in  the  other  parts  of 
the  nervous  system,  in  the  eighteen  to  twenty  cases  thus  far  autopsied 
(1910).  These  were  parench;vTnatous  and  fatty  degeneration  of  the 
muscles,  ascending  degeneration  of  the  peripheral  motor  nerves, 
especially  intermuscular  branches,  with  chronic  interstitial  neuritis, 
degeneration  of  spinal  ganglia,  atrophy  and  loss  of  anterior  horn  cells, 
sclerosis  of  columns  of  Goll  and  Burdach,  with  changes  in  lateral 
columns  and  posterior  roots. 


1  Arch.  f.  Psychiatrie,  1909. 


2  Ibid. 


'  Loc.  cit. 


AMYOTROPHIC  LATERAL  SCLEROSLS  469 

2.  Tabetic  Type  (Dejerine-Sottas^). — This  is  apparently  a  heredi- 
tary form,  the  sjTiiptoms  beginning  in  childhood.  The  muscular 
changes  are  similar  to  those  seen  in  the  Charcot-]Marie-Tooth  forms, 
but  the  sensory  nervous  system  is  much  more  markedly  involved. 
There  are  present  pupillary  changes — myosis,  or  even  Argyll-Robertson 
pupils,  beginning  or  complete  Romberg,  nystagmus,  and  ataxia.  Pain 
is  present.  The  peripheral  nerves  are  enlarged  and  palpable.  Reaction 
of  degeneration  was  stated  to  be  absent  by  Dejerine  and  Sottas. 

It  may  easily  be  confounded  with  a  juvenile  tabes.  Dejerine 
maintains  that  it  has  no  relation  to  the  Charcot-Marie-Tooth  atrophies. 

Other  forms  are  described  in  which  the  atrophies  are  limited  to  the 
lower  extremity  (Sainton),  to  the  upper  extremity  (Haenel),  and  to  the 
femorotibial  region  (Eichorst).    The  transition  t^-pes  are  many. 

Diagnosis. — A  complete  separation  of  all  the  forms  is  not  possible 
in  the  present  state  of  our  knowledge.  The  ^Yassermann  reaction  and 
cerebrospinal  fluid  examination  will  probably  throw  considerable 
light  on  the  hypertrophic  neuritic  types  of  Dejerine,  and  may  remove 
them  from  this  group  entirely;  also  cases  described  by  Marie  under  a 
similar  name. 

For  the  classical  cases  of  neuritic  muscular  atrophy  there  is  little 
ground  for  confusion,  but  the  numerous  aberrant  forms  introduce 
difficulties. 

The  separation  from  the  dystrophies  is  usually  made  on  the  ground 
of  pseudohypertrophy  and  the  absence  of  reaction  of  degeneration  in 
these  cases.    Certain  transition  forms  are  undifferentiable. 

Chronic  poljTieuritis  is  rarely  hereditary  (unless  one  follows  Oppen- 
heim  in  classing  the  neuritis  atrophies  here  under  consideration  as 
hereditary  chronic  multiple  neuritis).  In  polyneuritis  there  is  rarely 
a  club-foot,  the  progress  is  more  rapid,  and  recoverv'  is  apt  to  take 
place.  With  tabes,  only  Dejerine's  t}j>e  can  cause  confusion.  The 
age  and  heredity  are  the  chief  features.  Newer  studies  on  comple- 
ment-fixation and  spinal  fluid  are  wanting. 

In  amyotrophic  lateral  sclerosis  the  increased  reflexes,  the  extension 
to  the  bulbar  nuclei,  the  spasticity,  and  rapid  course  are  characteristic. 
Multiple  sclerosis,  syringomyelia,  chronic  poliomyelitis,  myotonia, 
and  hereditary^  ataxia  occasionally  call  for  difi'erentiation. 

Prognosis. — Usually  poor,  but  the  disease  is  very  chronic,  and 
patients  die  after  twenty  to  thirty  years  of  intercurrent  disorders. 
Sometimes  stationary  periods  are  met  with. 

Therapy. — Electricity,  baths,  massage,  internal  secretions,  general 
tonics,  and  orthopedics  are  useful.    A  few  cures  are  known. 

AMYOTROPHIC  LATERAL  SCLEROSIS. 

History. — The  position  of  the  progressive  muscular  atrophies  with 
their  many  clinical  variations,  and  the  anomalous  pathological  find- 

'  Arch,  de  neurol.,  No.  91,  sen  ii,  vol.  xvii;  Rev.  de  m6d.,  1897;  Rev.  neurol.,  1902- 
1906. 


470  LESIONS  OF  THE  SPINAL  CORD 

ings  is  still  in  need  of  more  detailed  analysis.  Charcot,  in  1865,  sepa- 
rated from  this  mass  a  special  group  which  combined  the  features 
of  a  poliomyelitis  and  a  lateral  sclerosis.  Atrophy  with  spasticity 
was  the  determining  feature,  and  in  1872-1874  after  previous  studies 
with  Joffroy  and  (lombault  he  gave  it  definite  form.  Dejerine  later 
(1883)  showed  that  the  glosso-labio-laryngeal  palsies  of  Duchenne 
were  a  constituent  part  of  the  disease,  and  the  work  of  Kojewnikoff, 
Marie,  Roth,  Florand,  Holmes,  Spiller,  Raymond,  Cestan  and  others 
has  served  to  establish  this  disorder  upon  a  sound  pathological  and 
clinical  basis. 

It  is  a  disease  of  the  entire  motor  neuron,  although  Holmes  has 
shown  that  this  is  not  universal. 

Etiology. — This  is  a  comparatively  rare  disease.  Little  is  known 
concerning  its  causes.  It  has  been  thought  of  as  a  primary  atrophy 
of  the  motor  system — an  abiatrophy,  but  this  is  only  pushing  the 
explanation  back  a  step  fiu-ther.  It  is  most  prevalent  in  early  adult 
life,  thirty  to  forty,  but  it  is  also  known  to  occur  in  children  (Erb-Seelig- 
miiller),  and  Soques,  Roussy,  Raymond,  Probst  and  others  have 
described  cases  occurring  in  the  fifth  decade.  Women  seem  to  have 
the  disease  somewhat  oftener  than  men,  but  the  differences  are  not 
striking.  The  statistics  of  the  Vanderbilt  Clinic  for  ten  years  show  the 
reverse  conditions — 11  men  and  6  women. 

iVrteriosclerosis,  infection,  intoxication,  syphilis  have  each  been 
shown  to  play  some  role  in  the  causation  in  some  patients.  The 
relationship  of  trauma  is  still  unsolved.  Bing  has  reported  a  case 
following  an  ascending  neuritis  cf  the  median  from  trauma.^ 

Pathology. — ^While  the  disease  is  one  in  which  the  entire  motor 
system  is  predominantly  affected,  this  is  not  exclusively  so,  as  Holmes 
has  definitely  shown.  The  spinal  motor  neurons  are  chiefly  affected, 
both  centrally  and  peripherally.  The  anterior  horn  cells  are  degen- 
erated, and  the  motor  nerve  fibers  as  well. 

The  corticospinal  tracts  are  also  degenerated.  This  degeneration 
can  be  followed  to  the  Betz  cells  of  the  Rolandic  cortex  in  practically 
all  of  the  cases  of  any  duration,  but  in  some  patients  the  degeneration 
in  the  pyramidal  tracts  has  not  been  traced  beyond  the  pons. 

Degeneration  in  the  bulbar  motor  nuclei  is  the  rule,  and  in  the  cortico- 
bulbar  tracts,  as  was  demonstrated  by  J.  Dejerine  in  1883.  The  third, 
fourth,  and  sixth  nerve  nuclei  usually  escape,  though  a  few  recorded 
degenerations  here  are  known  (Hoche,  Pal,  etc.). 

Degenerations  can  also  be  traced  in  the  corpus  callosum.  Following 
the  degeneration  there  is  some  replacement  sclerosis.  This  widespread 
degeneration  in  the  motor  system  may  be  accompanied  by  other 
degenerations,  however,  although  such  degenerations  seem  to  stand  out 
less  prominently  in  the  clinical  picture.  Thus,  degenerations  in  the 
lateral  columns,  Clarke's  columns,  posterior  longitudinal  bundles, 
spinocerebellar  tracts,  and  ventrolateral  ground  bundles  are  recorded. 

lErb:  Deutsch.  Ztschr.  f.  Nervenh.,  1911,  vol.  xliv.  Bing:  Rev.  Suisse  de  med., 
July  24,  1915. 


AMYOTROPHIC  LATERAL  SCLEROSIS 


471 


Symptoms. — The  disorder  may  begin  in  almost  any  motor  nerve 
region:  bulbar,  cervical,  dorsal  or  lumbar;  more  than  one  area  may 
be  initially  affected  simultaneously,  but  the  usual  beginning  lesions  are 
predominantly  in  the  muscle  groups  of  the  cervical  cord.  The  order 
of  involvement  is  predominantly  radicular. 

The  usual  symptoms  are  muscular  weakness,  with  wasting  of  the 
muscles,  usually  distally,  with  increased  reflex  excitability.  The 
patients  first  note  that  the  hands  get  tired,  they  are  clumsy  in  dress- 
ing and  undressing.  Buttoning  and  unbottoning,  sewing,  writing, 
and  the  carrying  out  of  the  more  delicate  finger  manipulations  become 
increasingly  difficult,  first  from  fatigue,  then  from  stiffness  and  motor 
palsy.  Atrophy  of  the  thenar  and  hypothenar  eminences  occurs,  then 
progresses  to  the  muscles  of  the  forearm,  arm,  and  shoulder-girdle. 


Fig.  276. — Amyotrophic  lateral  sclerosis  showing  atrophy  of  the  arms. 

Weakness  and  stiffness  then  make  themselves  evident  in  the  lower 
extremities,  and  a  similar  progression  takes  place.  Then  after  months 
or  a  year  or  so  the  muscles  of  the  lips,  of  the  tongue,  and  swallowing 
and  phonation  gradually  become  implicated.  The  lips  become  thinned, 
(tapir  mouth)  the  tongue  atrophies,  speech  fatigue  is  followed  by 
inability  to  speak;  swallowing  occurs  with  regurgitation,  cardiac 
irregularities  develop,  and  the  patients  die  of  aspiration  pneumonia, 
weakness  or  other  intercurrent  affections. 

With  the  atrophies  there  develops  a  gradually  increasing  spasticity. 
The  tendon  reflexes  are  increased,  thefe  is  increased  jaw-jerk,  or 
mandibular  clonus;  the  biceps  and  triceps,  and  scapular  reflexes  are 
increased,  patellar  clonus,  increased  knee-jerks,  increased  Achilles, 
ankle-clonus,  Babinski  and  Oppenheim  reflexes  are  manifest. 

The  muscles  themselves  show  increased  myotatic  irritability,  fibril- 


472  LESIONS  OF  THE  SPINAL  CORD 


1 


lary  contractions  are  common,  or  the  atrophies  may  be  so  marked 
that  flaccid  conditions  develop.    The  reaction  of  degeneration  occurs. 

Sensory  anomahes  are  extremely  rare.  Pain  is  probably  present 
at  some  period  in  many  cases;  it  is  that  of  fatigue  or  spasm,  however. 
The  bladder  and  rectal  functions  are  not  usually  involved,  and  the 
majority  of  the  patients  show  only  the  involvement  of  the, motor 
neurons. 

Mental  symptoms  are  prominent  late  in  the  disease;  increased 
emotionalism  is  usually  present,  and  as  the  patients  are  often  much 
depressed,  the  involuntary  crying,  which  is  very  frequent,  is  augmented 
thereby. 

As  has  been  noted,  the  onset  may  be  in  any  part  of  the  motor  sys- 
tem— thus  the  disorder  may  progress  for  some  time,  even  to  death, 
as  a  bulbar  type  of  palsy,  with  increased  reflexes  in  the  motor  cranial 
nerves;  death  occurring  in  some  instances  before  there  are  any  spinal 
symptoms.  Again  the  distribution  may  be  hemiplegic,  and  then 
triplegic,  and  finally  quadriplegic  and  bulbar. 

Again  the  disorder  may  bear  the  stamp  of  a  lower  extremity  para- 
plegia, or  an  upper  limb  diplegia;  again,  whereas  the  distal  muscles 
are  usually  initially  involved,  some  patients  show  proximal  atrophies  in 
either  upper  or  lower  extremities;  and  the  more  cases  reported  the 
greater  are  the  possibilities  encountered  in  the  way  of  anomalous 
localization. 

But  throughout,  after  a  certain  lapse  of  time,  the  combination  of 
weakness,  atrophy,  increased  reflex  activity,  without  sensory  changes, 
stamps  the  process  as  essentially  a  motor  degeneration,  and  whereas 
one  may  even  find  the  spastic  phenomena  antedate  the  atrophic  changes, 
yet  in  the  end  the  two  level  up,  unless  the  course  has  been  unusually 
active,  and  death  results  early  from  the  disease  or  from  intercurrent 
disorder.  Here  the  pathological  picture  may  seem  to  contradict 
the  clinical  findings,  and  the  interpretation  remains  that  the  patient 
did  not  live  long  enough  to  show  the  classical  syndrome. 

On  account  of  this  polymorphism,  certain  authors  have  erected  types 
which  may  be  useful  clinically,  but  which  are  rarely  borne  out,  save  for 
certain  periods,  during  the  development  of  the  disease.  Thus  Raymond 
and  Cestan^  make  (1)  an  ordinary  spinal  type  with  the  classical  main  en 
griff e  of  Charcot,  increased  reflexes,  particularly  in  the  upper  extremities, 
less  marked  in  the  lower,  with  occasional  failure  of  the  Babinski  reflex; 
(2)  lahioglossolaryngeal  type — apparently  more  frequent  in  women — 
which  begins  in  the  muscles  of  the  lips,  tongue,  pharynx,  or  larynx, 
occasionally  with  facial  palsies,  inability  to  close  the  eyes,  etc.,  with 
fibrillary  twitchings,  lively  masseter-jerk.  Some  of  these,  but  not  all, 
run  a  rapidly  fatal  course  from  four  to  nine  months,  and  die  before  the 
lower  neurons  show  their  characteristic  changes;  (3)  an  amyotrophic  type 
with  pronounced  atrophies  which  overshadow  or  mask  the  spasticities 
and  the  increased  reflexes.     These  patients  resemble  chronic  polio- 

iRev.  neurol.,  1905. 


FRACTURE  AND  DISLOCATION  SYNDROMES  473 

myelitis,  often  for  years,  and  constitute  a  most  difficult  group  to  differ- 
entiate. (4)  A  spastic  type,  in  which  the  reverse  condition  exists, 
and  increased  reflexes  and  spasms,  contractures,  etc.,  antedate  the 
atrophies.    These  resemble  multiple  sclerosis,  lateral  sclerosis,  etc. 

Finally,  it  should  be  recognized  that  certain  patients  represent 
transition  forms;  they  are  not  pure  tj^pes,  and  the  apparent  clinical 
picture  of  amyotrophic  lateral  sclerosis  is  due  to  other  than  the  recog- 
nized pathological  foimdations  of  this  disorder.^ 

Course  and  Duration. — ^The  average  time  of  duration  of  some  hundred 
or  more  cases  analyzed  is  about  two  years;  but  this  by  no  means  tells 
anything  about  any  individual  case.  Death  in  four  months  after 
the  onset  has  been  the  result  in  a  score  or  more  of  cases,  some  have 
persisted  ten  j'ears,  and  a  few  questionably  diagnosed  cases  even  much 
longer.  The  rule  is  toward  steady  progression;  there  are  few  leaps 
and  regressions,  as  is  seen  in  multiple  sclerosis,  and  so  far  as  is  known 
the  disease  is  a  fatal  one.  A  few  stationary  cases  are  reported.  Bulbar 
palsies,  when  not  among  the  initial  symptoms,  usually  develop  before 
the  second  year,  and  are  often  the  cause  of  death  by  choking,  or 
dyspnea.  Cardiac  irregularities,  pneumonia,  exhaustion,  secondary 
infections,  these  are  the  usual  modes  of  death.    Suicide  is  unusual. 

Diagnosis. — ^The  chief  disorders  needing  separation  are  multiple 
sclerosis  and  progressive  spinal  muscular  atrophy  or  chronic  polio- 
myelitis. The  former  rarely  shows  atrophies,  and  the  eye-grounds, 
ocular  muscle  signs,  and  characteristic  tremors  should  separate  this; 
the  latter  disorder  when  it  shows  increased  reflexes,  as  is  occasionally 
the  case,  is  separated  with  great  difficulty.  Lumbar  puncture  does 
not  throw  any  light  on  the  diagnosis. 

Treatment. — No  specific  is  known.  Overfeeding,  light  massage, 
much  rest,  avoidance  of  fatigue  of  affected  muscles,  caffeine,  strych- 
nine, are  the  only  methods  at  present  of  service.  Certain  anomalous 
thjToid  insufficiencies  have  been  associated  with  progressive  muscular 
wasting,  and  indicate  a  field  for  experimental  therapeutics.  Parathy- 
roid myatonias,  myasthenias,  family  periodic  paralyses,  etc.,  offer  a 
suggestion  in  this  line  of  a  perverted  parath}Toid  activity  that  may  be 
seized  upon  for  the  study  of  the  mineral  metabolism,  especially  of 
calcium.  Personal  cases  treated  along  lines  suggested  by  the  mineral 
metabolism  of  the  body  offer  a  glimmer  of  hope  in  a  hitherto  hopeless 
disease.     Sj^hilitic  cases  need  specific  therapy. 

FRACTURE,  DISLOCATION  AND  CONCUSSION  SYNDROMES. 

Spinal  cord  injuries  result  from  bullets,  penetrating  instruments, 
or  from  blows  or  falls. 

Bullets  and  cutting  instruments  cause  hemorrhage,  with,  as  a  rule, 

'  For  more  recent  studies  of  this  disorder  see:  Blaeden:  Med.  Jour.  So.  Africa,  1917, 
xiii.  59.  Hassin:  Med.  Record,  1917,  xci,  228.  Kempner:  Med.  Klinik,  1918,  xiv,  729. 
Hoffman:  Duetsch.  med.  Wchnschr.,  1918,  p.  1151;  Miinchen.  mod.  Wchnschr.,  1918, 
p.  1062.  Rhein:  New  York  Med.  Jour.,  1917,  cv,  915.  Rossi:  Cron.  d.  CI.  Med. 
Geneva,  1917,  xxiii,  161.     Throckmorten :    Jour.  Iowa  State  Med.  Soc,  1917,  Ixxi,  177. 


474 


LESIONS  OF  THE  SPINAL  CORD 


partial  or  complete  severance  of  the  cord,  with  septic  infections  of  the 
cord  and  meninges.  Blows  and  hard  falls  occasion  fractures  or  disloca- 
tions, with  crushing  of  the  cord  to  a  greater  or  lesser  degree.  Mild 
injuries  may  result  merely  in  the  bruising  of  the  cord,  or  minute 
hemorrhages  within  the  cord  or  the  pial  or  dual  spaces  only  may 
be  produced,  sometimes  even  from  excessive  exertion,  long  marching, 
severe  athletic  exercises,  sudden  spinal  torsions,  etc.  Dislocation 
of  a  vertebra  practically  always  causes  a  crushing  of  the  cord;  the 
so-called  dislocations  without  spinal  cord  injury  are  more  apt  to  be 
wrenches  of  the  vertebrae  or  verv  limited  dislocations.     Fractures 


Fig.  277. — Total  cross-section  of  spinal  cord  at  the  level  of  Cs  showing  the  results 
upon  the  long  fiber  tracts.  Middle  section  indicates  level  of  injury.  Cross-hatching 
above  and  below  indicate  the  degenerations  or  loss  of  function.     (Veraguth.) 


may  result  with  but  few  spinal  symptoms;  crushing  of  the  cord  causes 
more  or  less  disintegration,  usually  accompanied  by  severe  hemorrhages 
within  or  without  the  cord  substance  (hematomyelia,  dural  hemor- 
rhage). Hematomyelia  usually  extends  up  and  down  the  cord  from 
the  point  of  injury,  sometimes  involving  several  segments  of  the  cord. 
Symptoms. — Fractures  or  dislocations  of  the  spinal  vertebrae  usually 
cause  local  deformity,  much  pain  and  muscular  rigidity,  particularly 
on  motion.  X-ray  examination  reveals  the  nature  and  extent  of  the 
bony  lesion.  The  motor  and  sensory  phenomena  at  the  level  and  below 
the  site  of  the  lesion  indicate  the  location,  extent  and  character  of 


FRACTURE  AND  DISLOCATION  SYNDROMES 


475 


the  injury  to  the  spinal  cord.  The  s\Tiiptoms  usually  develop  imme- 
diately following  the  injury,  become  slightly  progressive,  if  hemorrhage 
only,  and  then  slowly  recede  until,  after  a  variable  length  of  time, 
usually  from  two  to  three  months,  the  residual  symptoms  indicate  the 
permanent  impairment  of  function. 

The  chief  syndromes,  which  develop  from  injury  to  the  spinal  cord 
at  different  levels,  are  here  described  by  means  of  charts.  A  careful 
sensory  examination  is  indispensable  and  should  follow  the  directions 
already  laid  down. 


Fig.  278  and  279. — Showing  superficial  sensibility  disturbances  in  complete  transverse 
lesions  of  the  cord  at  the  levels  of  d,  d,  De  and  Li,  respectively.     (Veraguth.) 

It  is  important  to  recall  that  the  spinal  cord  segments  and  the  verte- 
bral segments,  while  nearly  corresponding  during  infancy,  do  not  in 
the  adult.  It  is  rare  that  absolutely  symmetrical  involvements  result 
from  spinal  injury. 

A  total  destruction  of  the  spinal  cord  will  result  in  initial  and  in 
residual  symptoms.  This  may  arise  from  accident,  bullet  wound, 
diving,  caisson  disease,  myelitis,  tumor — Pott's  disease  chiefly.  The 
course,  progression  and  mode  of  treatment  w  ill  depend  entirely  upon 
the  causative  lesion. 


476 


LESIONS  OF  THE  SPINAL  CORD 


The  Great  War  has  added  an  enormous  amount  of  new  material 
to  the  subject  of  spinal  cord  injury.  Whereas  peripheral  nerve  injuries 
have  been  comparatively  more  frequent,  yet  spinal  injuries  have  been 


General  Func- 
SiTE  OF  Lesion.  tion. 


Initial  Syndrome. 


He.'sidual  Syndrome. 


Motility. 


Symmetrical  flaccid  palsy  of 
the  lower  extremities. 


Symmetrical  spastic  paralysis 
of  lower  extremities  to  level 
of  L.  4. 


Lumbar  ^^ments 
(L.  0) 


Symmetrical  flaccid  pals.v  of 
the  abdominal  muscles  and 
lower  extremities. 


Symmetrical  spastic  palsy  of 
the  abdominal  muscles  and 
lower  extremities. 


Symmetrical  palsy  of  preced- 
ing plus  flaccid  palsy  of 
intercostals. 


Symmetrical   spastic   palsy    of 
preceding   plus   intercostals. 


Sapsory 


I  Segmentg 
(D.  6.) 


J  Preceding  signs  plus  flaccid 
palsy  of  upper  limbs  and 
disturbances  of  breathing. 

Symmetrical  loss  of  superfi- 
cial and  deep  sensibility  of 
the  lower  extremities.  Re- 
tention of  spinal  sensory 
functions  below  lesion. 

Preceding  plus  loss  of  super- 
ficial and  deep  sensibilities 
between  D.  6  and  L.  3. 


Preceding  plus  loss  of  super- 
ficial and  deep  sensibilities 
between  D.  1  and  D.  6. 


When  not  resulting  in  death 
total  bilateral  spastic  par- 
alysis. 


Cervico  Dors 
{C8.  Dl.) 


Preceding  plus  loss  of  super- 
ficial and  deep  sensibility 
between  C.  4  and  D.  1. 

Total  loss  of  patellar, Achilles, 
plantar  reflexes.  Cremas- 
teric preserved. 


Preceding  plus  loss  of  abdom- 
inal reflexes. 


Patellar  reflex  retained.  Achilles 
increased.  Clonus  and  Bab- 
inski  and  contralateral  Bab- 
inski  obtained. 


Preceding     plus     spontaneous 
abdominal  movements. 


Preceding    with    oculopupil- 
lary  disturbances. 


Preceding   with   oculopupillary 
disturbances. 


Cervical 
(C.A.) 


DlsturUances 


Preceding  without  oculo- 
pupillary signs  but  with 
loss  of  reflexes  of  upper 
extremities. 

Total  bladder  and  intestinal 
palsy. 


Symmetrical     dilatation     of 
vessels  of  lower  extremity! 


Preceding  with  vascular  dila- 
tation in  upper  extremities. 


Preceding  without  oculomotor 
disturbance  and  increased 
reflexes  of  upper  extremities. 


Total  bladder  and  intestinal 
palsy  with  later  hyperirri- 
tability;  also  of  genital 
reflexes  without  priapism. 

Objective  symmetrical  chilli- 
ness and  cyanosis  of  the  legs. 
Later  sacral  decubitus.  Skin 
and  nail  disturbances. 

Preceding  with  objective  sym- 
metrical cold  and  cyanosis 
of  the  arms.  Shoulder-girdle 
decubitus  possible 


Fig.  280. — Localization  of  symptoms  at  different  levels  of  the  cord.     (Veraguth.) 


The  First  Sensory  Neuron  and  the  Origin  of  the  Secondary  Sensory 
Pathways  of  the  Anterolateral  CoUimn  of  the  Spmal  Cord- 


i 


\ 


FRACTURE  AND  DISLOCATION  SYNDROMES  477 

by  no  means  rare.  The  student  interested  in  the  complexities  of  the 
nmnerous  problems  arising  from  spinal  cord  injuries  of  war  are  referred 
to  Schaller's  excellent  critical  review.^ 

The  chief  s;yTiiptoms  of  acute  transverse  lesion  are  expressed  in  the 
table  Fig.  280  and  diagrams  according  to  the  site  of  the  lesion. 

Lesions  of  the  upper  cervical  segments  usually  cause  instant  death 
from  respiratory  paralysis.  INIarked  h^-perthermia  often  characterizes 
upper  cervical  lesions.  Injiu-y  lower  down  is  very  frequent,  occurring 
in  workmen  from  objects  falling  upon  the  bent  neck  or  in  reckless 
di\'ing.  The  symptoms  are  charted.  Many  of  these  patients  live  for 
some  time,  even  years,  dying  usually  from  bladder  and  kidney  com- 
plications. An  inferior  cervical  syndrome  is  described  by  Holmes,^ 
which  is  characterized  by  h\'pothermia,  lowered  arterial  tension,  slow 
pulse,  diminished  urinary  secretion,  and  general  stupor,  in  addition 
to  the  usual  motor  and  sensory  losses.  Dorsal  lesions  are  compara- 
tively rare,  and  often  show  the  BroAvn-Sequard  syndrome,  which  latter 
may  however,  develop  from  lesions  at  all  levels  above  the  conus. 
Localized  lesions  at  Di,  D4  often  show  decided  increase  in  the  pulse 
rate  as  an  additional  localizing  sign.  Middorsal  lesions  frequently 
show  persistent  vomiting.    This  is  a  vegetative  pathway  disturbance. 

Lower  dorsal  and  upper  lumbar  lesions  are  the  most  frequent  of  all. 
Their  symptoms  are  indicated  in  the  chart.  Lesions  from  D5  Dg  often 
present  polyuria. 

Injury  to  the  lower  lumbar  vertebrae  causes  cauda  equina  lesions,  as 
the  cord  proper  terminates  at  about  the  first  lumbar.  And  Collier 
has  shown  that  in  intradural  hemorrhage  a  tendency  for  the  blood  to 
collect  in  the  cauda  often  gives  rise  to  cauda  equina  syndromes,  the 
injury  often  being  higher  up. 

Intramedullary  lesions — hematomyelia — ^have  a  symptomatology  all 
their  o^\^^. 

Careful  sensory  testing  shows  that  in  an  intramedullary  lesion  there 
is  a  tendency  toward  a  complete  separation  of  the  impulses  underlying 
the  appreciation  of  posture,  the  discrimination  of  two  points,  and  their 
correlated  faculties  from  those  of  other  sensory  groups. 

All  painful  and  thermal  impulses  coming  from  the  periphery  undergo 
regrouping  after  entering  the  spinal  cord,  and,  whether  they  arise 
in  the  skin  or  in  deeper  structures,  become  arranged  according  to 
functional  similarity.  Then,  after  a  longer  or  shorter  course,  they 
pass  away  to  the  opposite  side  of  the  spinal  cord.    (See  Plate  XL) 

This  process  of  filtration  leaves  all  the  impulses  associated  with 
postural  and  spatial  recognition  to  continue  their  course  unaltered 
in  the  posterior  columns;  they  are  the  survivors  of  peripheral  groups 
broken  up  by  the  passing  away  of  certain  components  into  secondary 
afferent  systems.  At  any  point  in  the  spinal  cord,  these  columns  trans- 
mit not  only  impulses  from  the  periphery  which  are  on  their  way,  after 
a  shorter  or  longer  passage,  to  regrouping  and  transformation,  but  at 

1  Archives  of  Neiirology  and  Psychiatry,  vol.  i,  No.  4,  471. 

2  British  Med.  Jour.,  1915,  ii,  769  et   seq. 


478 


LESIONS  OF  THE  SPINAL  CORD 


1 


the  same  time  they  form  the  path  for  impulses,  arising  both  in  the 
cutaneous  and  deep  afferent  systems,  which  undergo  no  regrouping 
until  they  reach  the  nuclei  of  the  medulla  oblongata. 


/ 

/^ 

'^^t^^A 

C 

^cS^ 

L 

¥ 

R 

( 

r^^ 

^y^  ^ 

Fig.  281 


1 


Figs.  281  and  282. 


Fig.  282 
-Partial  lesion  of  fourth  lumbar  segment  from  bullet  wound. 


Thus,  a  lesion  confined  to  one-half  of  the  spinal  cord,  even  at  its 
highest  segment,  may  interfere  with  the  passage  of  sensory  impulse, 
some  of  which  are  travelling  in  secondary  paths,  while  others  are  still 
within  the  primary  level  of  the  nervous  system.  All  impulses  concerned 
with  painful  and  thermal  sensations  from  distant  parts,  disturbed  by 
such  a  lesion,  will  be  travelling  in  secondary  paths  and  will  have  come 


FRACTURE   AND  DISLOCATION  SYNDROMES 


479 


from  the  opposite  half  of  the  body;  for,  after  regrouping,  they  have 
passed  across  the  spinal  cord.  But  those  impulses  underlying  the 
appreciation  of  posture,  the  compass  test,  size,  shape,  form,  weight, 
consistence,  vibration,  will  be  affected  on  the  same  half  of  the  body 


Fig.  283 


Fig.  284 
Figs.  283  and  284. — Partial  lesion  of  fifth  lumbar  segment  from  bullet  wound. 

as  the  lesion.    They  still  remain  in  paths  of  the  primary  level  and 
have  undergone  no  regrouping.    (See  Plates  X  and  XI.) 

In  such  a  case  the  parts  on  the  side  opposed  to  the  lesion  may  be 
insensitive  to  pain,  heat  and  cold;  but  all  the  postural  and  spacial 
aspects  of  sensation  will  be  perfectly  maintained.    Yet,  all  power  of 


480 


LESIONS  OF  THE  SPINAL  CORD 


recognizing  position,  of  estimating  size,  shape,  form,  and  weight,  or  of 
discriminating  the  two  compass  points,  will  be  lost  in  the  limbs  which 
lie  on  the  side  of  the  lesion,  although  tactile  sensibility  and  localization 
of  the  spot  stimulated  may  be  perfectly  preserved. 


Fig.  285 


Figs.  285  and  286.- 


FiG.  286 
-Partial  lesion  of  fourth  lumbar  vertebra  from  bullet. 


This  remarkable  arrangement  enables  one  to  analyze,  as  Head  has 
pointed  out,  the  nature  of  the  peripheral  impulses  upon  which  depends 
the  power  of  postural  and  spacial  recognition.  Obviously  even  at 
the  periphery,  they  must  be  independent  of  touch  and  pressure.  The 
power  to  distinguish  two  points  applied  simultaneously  and  to  recog- 


FRACTURE  AND  DISLOCATION  SYNDROMES  481 

nize  as  such  size  and  shape,  requires  as  a  preUminary  the  existence  of 
sensations  of  touch;  but  the  patient  may  be  deprived  of  all  such  powers 
of  spacial  recognition  without  any  discoverable  loss  of  tactile  sensibility. 
In  the  same  way  our  power  to  appreciate  the  position  of  a  limb,  or  to 
estimate  the  weight  of  an  object,  is  based  upon  impulses  which,  even 
at  the  periphery,  exist  apart  from  those  of  touch  and  pressure,  called 
into  simultaneous  being  by  the  same  external  stimulus. 

This  long  delay  of  the  postural  and  spatial  elements  in  reaching 
secondary  paths  enables  them  to  give  off  afferent  impulses  into  the 
spinal  and  cerebellar  coordinating  mechanisms,  which  lie  in  the  same 
half  of  the  spinal  cord.  The  impulses  which  pass  away  in  this  direction 
are  never  destined  to  enter  consciousness  directly.  They  influence 
coordination,  unconscious  posture  and  muscular  tone,  and,  although 
arising  from  the  Same  afferent  end-organs,  they  never  become  the  basis 
of  a  sensation. 

Finally,  the  last  survivors  of  these  impulses  from  the  periphery 
become  regrouped  in  the  nuclei  of  the  posterior  columns  and  cross  to 
the  opposite  half  of  the  medulla  oblongata  in  paths  of  the  secondary 
level.  So  they  pass  to  the  optic  thalamus  and  thence  to  the  cortex,  to 
underlie  those  sensations  upon  which  are  based  the  recognition  of 
posture  and  spatial  discrimination. 

Diagnosis. — Accurate  testing  of  the  motor  and  sensory  functions 
leaves  no  doubt  as  to  the  presence  of  a  spinal  cord  injury  of  a  major 
grade.  The  results  of  minute  lesions  may  escape  recognition.  Total 
severance  of  the  cord  is  unusual,  most,  even  severe  injuries,  leave  some 
pathways  undivided,  which,  after  the  immediate  effects  of  the  injury 
have  subsided,  give  some  sensory  response.  Total,  complete  severance 
causes  absolute  anesthesia  to  all  forms  of  stimuli,  flaccid  paraplegia, 
with  loss  of  all  reflexes  and  all  visceral  reactions  below  the  site  of  the 
lesion.  This  has  been  termed  Bastian's  (1882)  law  and  has  been  widely 
accepted.  The  recent  war  experiences  have  tended  to  modify  it  some- 
what. Kiddoch  has  shown  in  complete  section  there  is  a  stage  of 
flaccidity,  then  a  stage  of  reflex  activity,  when  the  bladder  and  rectum 
may  act  automatically  (vegetative  pathways)  and  muscle  tonus  may 
pick  up  and  then  a  stage  of  gradual  failure  of  all  reflex  activities. 

Half-sided  lesions  produce  the  typical  Browoi-Sequard  syndromes, 
which  vary  according  to  the  segments,  as  seen  in  the  accompanying 
scheme  (Fig.  288).  Recent  war  studies  of  the  sensibility  crossing  tend 
to  show  that  in  the  middorsal  regions  pain  and  temperature  fibers  tend 
to  decussate  almost  within  one  segment,  in  the  superior  dorsal  regions 
two  segments  are  used  and  in  the  cervical  cord  pain  fibers  traverse 
five  to  six  segments,  temperature  four  to  five.  There  are  reasons  to 
suppose  that  below  I)i2  the  sensibility  fibers  do  not  cross  at  all, 
hence  tiie  Bnnvn-Sequard  dissociation  syndrome  occurs  in  lesions  only 
al)ove  Li. 

(Jertain  other  signs  are  found  in  incomplete  section.    Among  them 
are  slower  relaxation  phases  in  the  knee-jerks;  no  marked  difl'erence  in 
tonus  between  flexor  and  extensor  groups,  presence  of  postural  reflexes 
31 


482 


LESIONS  OF  THE  SPINAL  CORD 


of  the  nature  of  defence — due  to  integrity  of  some    prop^iocepti^ 
spinal  pathways. 

Prognosis. — This  is  bad  in  practically  all  spinal  injuries  save  sma 
hemorrhages  or  limited  cauda  equina  lesions.  High  lesions  are  almoe 
immediately  fatal.  Lower-lying  ones — fourth  cervical  and  down- 
vary  in  their  immediate  and  remote  results,  according  to  the  site  c 
the  lesion  and  its  extent. 


Fig.--  287. — Lesion  of  the  cord  producing  a 
Brown-Sequard  syndrome.     (Veraguth.) 


Fig.  288. — Brown-Sequard  syn- 
drome. Black  represents  superficial 
anesthesia  and  joint  anesthesia: 
passing  hyperesthesia  and  par- 
alysis.    (Veraguth.) 


Collier^  advises  careful  study  of  the  plantar  reflexes  as  offering  some 
clues  as  to  prognosis.    A  progressive  change  from  no  reflex  to  reduced 
reflex  or  response  to  extensor  response  is  indicative  of  some  recovery 
and  argues  for  incomplete  section — the  reverse  progression  indicates! 
a  bad  prognosis.  i 

The  question  of  regeneration  of  spinal  neurons  has  not  as  yet  been  I 
entu-ely  settled  experimentally,  but  the  preponderance  of    practical 


1  Lancet,  1916,  i,  700. 


FRACTURE  AND  DISLOCATION  SYNDROMES 


483 


evidence  is  against  it.^  Pathways  once  destroj'ed  remain  so.  Just 
at  what  period  it  may  be  said  that  the  residual  symptoms  will  be 
permanent  is  largely  a  matter  of  the  extent  of  the  lesion.    As  a  rule 


Cervical  \  1 
segments/ 


Initial  Stndrome. 

On  the  side  of  the  lesion.  Flac- 
cid palsy  of  muscles  whose 
neurons  lie  caudad  to  lesion. 

Normal  on  opposite  side 

z'  On  the  side  of  the  lesion.  Disturb- 
ance of  deep  sensibility,  espe- 
cially joint  hypesthesia,  caudad 
to  affected  metameres.  Small 
zone  of  superficial  anesthesia 
above  the  palsied  motor  zone. 
Hypesthesia     for    touch    caudal 

.       to  affected  metameres. 

^  On  the  crossed  side,  and  partly 
on  the  side  of  lesion,  because 
of  limitations  in  crossing  space 
of  fibers  below  lesion,  super- 
ficial hypesthesia,  especially 
thermohypesthesia  and  hypal- 
gesia. 

V^  As  above. 
On  the  side  of  the  lesion.     Loss 
of  tendon  and  skin  reflexes  of 
the  lower  extremity.     Babin- 
ski  phenomenon. 

On  opposite  side.  Loss  of  skin 
reflexes  of  the  lower  extremity. 

Preceding  and  loss  of  abdominal 
reflex. 

Preceding  and  loss  of  tendon 
reflexes  of  the  upper  extremity 
of  the  side  of  the  lesion. 

On  the  side  of  the  lesion  the 
skin  of  the  caudal  metameres 
red  and  hot. 


On  the  crossed  side  normal. 
Preceding  plus  oculopupillary 
signs  on   affected  side. 


Residual  Syndrome. 

On  side  of  lesion,  spas- 
tic palsy  of  muscles 
whose  neurons  lie 
caudad  to  lesion. 

Normal  on  side  oppo- 
site to  lesion. 


Hyperesthesia        disap- 
pears shortly. 


On  the  side  of  the 
lesion.  Increase  of  ten- 
don and  skin  reflexes. 

Babinski.  Contralat- 
eral Babinski. 


Preceding  and  increased 
abdominal  reflexes  on 
side   of   lesion. 

Preceding  plus  hand- 
clonus,  rotation  clo- 
nus, etc.,  on  affected 
side. 

On  side  of  lesion  the 
skin  of  the  caudal 
metameres  either 
normal  or  cyanotic 
or    objectively    cool. 

On  crossed  side  nor- 
mal. Preceding  with 
oculopupillary  signs 
on  the  side  of  lesion. 


Fig.  289. — Scheme  showing  localization  and  .symptoms  in  Brown-Sequard  syndromes. 

(Veraguth.) 

the  findings  at  three  months  are  ai)t  to  be  those  of  permanence,  still 
occasionally  marked  improvement,  ])articularly  in  bladder  and  rectal 
functions,  may  take  place  after  a  year  or  more  of  total  loss.     IVIany 

'  See  Jacob,  Girou  et  Ferrand:  Bull,  de  I'Acad.  de  med.  de  Paris,  1915,  Ixxiv,  423,  for 
report  of  so-called  succes-sful  cabling  of  spinal  cord.  Morat:  Lyon  Chir.,  1914-15,  xii, 
761.     CoUica:  liiforma  mod.,  1917,  xxiii,  583. 


484  LESIONS  OF  THE  SPINAL  CORD 

spinal  Ifsions,  especially  those  dependent  chiefly  on  hemorrhage,  will 
show  widespread  symptoms  early,  and  later  these  will  be  reduced  to  a 
minimum.  The  chief  bugbear  as  to  life  is  the  bladder.  Great  care 
should  be  taken  of  it  in  the  early  stages,  and  every  available  measure 
taken  to  keep  it  sterile. 

Treatment. — Sm-gical  treatment  is  indicated  in  most  spinal  injuries 
even  though  the  prognosis  is  not  at  all  reassuring.  The  persistence  of 
some  form  of  sensibility  (all  types  should  be  tested)  is  an  indication 
that  the  cord  is  not  completely  severed.  Operation  during  shock  is  not 
advisable,  yet  too  long  a  delay  is  unwarranted,  even  if  all  sensibility 
seems  abolished  below  the  lesion.  The  functional  loss  practically 
always  exceeds  the  anatomical  defect.  X-ray  examination  should  be 
made  immediately  for  diagnosis,  and  if  there  is  evidence  of  compression, 
operation  is  advisable  as  early  as  is  compatible  with  the  patient's 
condition.  In  the  absence  of  evidence  of  compression,  earlier  surgeons 
advised  against  operation  as  dangerous  and  futile.  Since  in  the  hands 
of  competent  surgeons  most  of  the  dangers  of  spinal  cord  surgery  have 
disappeared,  there  is  a  growing  tendency  to  operate  more  freely  and, 
at  times,  fortunately.    More  often  there  is  little  gain. 

Operations,  after  spinal  injury,  are  often  futile,  yet  with  care  rarely 
do  any  harm,  and  not  infrequently,  if  the  cord  itself  is  only  being 
pressed  upon  by  hemorrhage,  may  be  distinctly  advantageous.  Evi- 
dent surgical  indications  (fracture,  dislocation)  should  be  met.  The 
patient  is  rarely  benefited  but  is  entitled  to  the  doubt. 

The  general  treatment  of  the  patient  is  of  great  importance.  It 
should  be  directed  toward  giving  a  maximum  of  relief  from  distress; 
avoidance  of  bladder  infection,  preventing  bed-sores,  and  careful 
bowel  attention.  Antispasmodics  and  analgesics  may  have  to  be  used 
for  a  long  time,  but  morphin  and  its  derivatives  are  to  be  avoided  if 
possible,  save  in  those  agonizing  cases  where  it  gives  the  only  relief 
from  torture.    Electricity  is  largely  a  placebo. 

Concussion  Syndromes. — A  mass  of  material  from  the  war  has 
contributed  to  a  knowledge  of  severe  spinal  cord  injuries  without 
direct  impact  of  bullets  or  spinal  fragments,  but  from  mass  mechanical 
factors  which  have  produced  disseminated  hemorrhage,  hematomyelias, 
vacuum  pressure  changes  as  in  caisson  accidents  and  a  host  of  irregular 
forms  giving  rise  to  direct  and  indirect  forms  of  severe  spinal  cord 
injury  with  somatic  syndromes.  Edematous  swelling,  focal  necroses, 
localized  disseminated  hemorrhages,  with  secondary  cavity  formations 
and  extensive  fiber  and  cell  degenerations  have  been  observed.  The 
resulting  syndromes  have  been  various.  Severe  quadriplegias,  hemi- 
plegias, monoplegias,  diplegias,  cerebellospastic  tjT^es  and  anomalous 
mixtures  of  these  have  been  described.  In  fact  a  great  multiplicity  of 
types  shading  from  the  most  severe  persisting  quadriplegias  to  the 
cases  not  definitely  to  be  separated  from  the  more  psychogenic  war 
neuroses  of  the  spinal  cord  are  abundant  in  the  literature  to  which  the 
student  must  tm-n  as  they  cannot  all  be  discussed  here.^ 

1  See  Schaller's  Digest,  loc.  cit. 


COMPRESSION  OF   THE  CORD  485 

COMPRESSION  OF  THE  CORD. 

Compression  of  the  cord,  as  a  slow  chronic  process  results  from  (1) 
bony  hx-pertrophies,  (2)  tuberculosis,  (3)  tumors,  (4)  s^-philis,  (5) 
aneurisms,  (6)  meningeal  disease. 

Bony  Hypertrophies  (Osteitis,  Osteitis  Deformans,  Osteo-arthritis, 
Spondylosis  Rhizomelique) . — Under  these  various  names  one  finds 
patients  who  present  signs  of  more  or  less  spinal  cord  compression, 
either  vnth.  or  without  stiff  backs  or  deformities. 

They  show  gradually  increasing  weakness,  going  on  to  paresis, 
or  complete  paraplegia  with  spasticity  and  increased  reflexes.  Fre- 
quently there  is  severe  pain  and,  when  the  bony  disease  impinges  upon 
the  intervertebral  foramina,  neuritic  pains  and  symptoms  of  peripheral 
nerve  palsy  develop.  (See  Plexus  Palsies.)  X-ray  examinations  reveal 
the  nature  of  the  bony  changes  and  the  location  of  the  pressure. 

Tuberculosis  (Caries). — This  is  a  most  widespread  cause  of  spinal 
cord  compression.  The  tuberculous  focus  usually  begins  within  the 
body  of  the  vertebrse;  breaking  do'^ii  and  destruction  of  the  vertebrae 
take  place,  with  displacement  and  projection  of  the  vertebrae  either 
forward,  backward  or  laterally,  causing  the  various  deformities  of 
Pott's  disease.  The  tuberculous  process  usually  spreads  to  the  spinal 
meninges  (tuberculous  pachjTneningitis) .  Thus  if  the  compression 
occurs  it  may  be  from  both  processes.  Even  more  rarely,  tuberculous 
myelitis  occurs  from  direct  extension,  usually  through  the  honphatic 
channels. 

Symptoms. — The  chief  symptoms  are  pain  in  the  back,  often  reflected 
and  complained  of  in  knee  (important  to  bear  in  mind  in  children), 
stiffness  of  muscles,  rigidity  and  tenderness  on  motion  in  the  early 
stages.  With  the  development  of  a  kyphosis,  lordosis  or  scoliosis, 
deformities  appear  and  s\Tnptoms  of  cord  compression  and  plexus 
pressure  begin.  The  plexus  symptoms  varj'  with  the  segments  involved, 
as  already  discussed.  (See  Peripheral  Plexus  Xeuritides.)  The  cord 
compression  gives  rise  to  increasing  bilateral  spasticity  below  the  site 
of  the  lesion,  slowly  developing  sensory  impairment,  which  may  develop 
into  signs  of  a  complete  cutting  off  of  the  entire  cord  pathways  below 
the  site  of  the  compression. 

Lymphocytosis  in  the  cerebrospinal  fluid  is  a  frequent  sign.  X-ray 
examination  will  early  reveal  the  signs  of  tuberculous  bony  disease. 

The  symptoms  usually  show  very  gradually,  as  the  tuberculous 
disease  is  usually  a  slow  one.  Occasionally  they  show  a  fulminating 
course,  especiaUy  in  childhood,  or  even  in  young  adults. 

The  outcome  depends  upon  the  success  obtained  in  combating  the 
tuberculosis  by  orthopedic,  surgical  and  general  health  measures. 
Early  diagnosis  is  essential,  and  the  spinal  fluid  examinations  and 
x-ray  findings  will  aid  in  such  an  early  sizing  up  of  the  situation, 
which  will  lead  to  the  proper  procedures.  Surgical  therapy  early, 
Albee's  bone  splint  or  related  procedure  may  help  to  do  away  with  the 
cumbersome  braces  formerly  so  extensively  used  in  treating  Pott's  disease. 


486 


LESIONS  OF   THE  SPINAL  CORD 


Spinal  Cord  Tumors. — The  consideration  of  spinal  cord  tumors 
makes  a  large  chapter  in  contemporary  sensorimotor  neurology,  which 
can  only  be  sketched  here.  They  are  comparatively  rare,  yet  frequent 
enough  to  put  one  on  one's  guard  in  any  spinal  cord  condition  showing 

compression  phenomena,  i.e.,  weak- 
ness, spasticity,  and  increased  re- 
flexes, i.  e.,  pressure  symptoms  of 
the  spinal  motor  pathways. 

Spinal  cord  tumors  are  as  varia- 
ble as  those  found  within  the  cranial 
cavity.  They  are  found  extradur- 
ally,  durally,  intradurally  and  intra- 
medullary. They  are  small  and 
large,  and  located  at  any,  some- 
times at  all,  levels  (multiple  sarco- 
mata) of  the  cord.  The  tumors  of 
the  spinal  cord  are  identical  in 
histological  features  with  those  of 
the  brain  {q.  v.).  Pseudotumors — 
cysts,  angiomata,  serous  collections, 
enlarged  veins,  and  many  anomalies 
occur  within  the  spinal  canal  and 
produce  tumor  symptoms.  For  the 
most  part  they  lie  laterally  and 
posteriorly,  and  are  thus  more  ac- 
cessible to  surgical  removal.  They 
occur  at  all  ages,  and  in  both  sexes, 
and  show  a  frequency  comparable  to 
those  located  in  the  cranial  cavity. 
Symptoms. — The  chief  symptoms 
of  spinal  cord  tumor  are  pains, 
sensory  signs  in  the  skin,  and  evi- 
dences of  motor  paresis. 

Sharply  localized  pains  at  the  site 
of  the  tumor  are  frequent,  but  may 
be  absent;  all  pains  may  be  ab- 
sent, but  this  is  infrequent.^  The 
more  usual  pains  are  those  of  the 
root  area  or  areas  in^'ol^'ed  by  the 
tumor.  The  pains  are  usually  uni- 
lateral, but  widespreading  tumors 
cause  bilateral  pain  later.  Any 
so-called  neuralgia,  sciatica,  etc., 
may  be  the  initial  pressure  pain  of  a  spinal  cord  tumor  (see  discussion 
on  neiu-algias).  Under  the  popular  misnomer,  "rheumatism,"  many 
spinal  cord  tumors  and  neuralgic  pains  are  hidden  until  it  is  too  late 
to  obtain  relief. 

»  Bailey:     Jour.  Am.  Med.  Assn.,  1914. 


Fig.  290. 


-Osteophytes  of  spinal  cord. 
(Larkin.) 


TUMORS  OF  THE  CORD  487 

!The  .T-ray  picture  is  usually  negative.  The  cell  count  of  the  cerebro- 
spinal fluid  often  gives  definite  information  relative  to  the  subject  of 
'  irritative  meningeal  signs  (high  cell  count  meaning  acute  inflammatory 
[disease),  and  most  tumors  show  a  high  globulin  content  in  the  cerebro- 
\  spinal  fluid.  The  Wassermann  technic  will  rule  out  gummata  as  a  rule. 
'  In  some  tumors  there  is  an  absence  of  fluid,  or  fluid  under  a  very  low 
i  pressure,  below  the  site  of  the  tumor. 

The  motor  compression  signs  are  variable,  paresis  advancing  to  par- 
alysis (paraplegic),  h^-pertonicity,  spasticity,  increased  reflexes,  Bab- 
inski,  clonus,  etc.  At  the  level  of  the  tumor  there  may  be  destructive 
lesions  with  signs  of  peripheral  motor  neuron  disease,  i.  e.,  atrophy, 
loss  of  reflexes  in  the  diseased  area,  reaction  of  degeneration,  trophic 
changes  (bed-sores,  etc.) 

The  sensory  phenomena  will  vary  also,  one  side  often  showing  more 
markedly  than  the  other.  There  is  hyperesthesia  at  about  the  level  of 
the  lesion  which  is  changed  to  various  grades  of  anesthesia  below 
the  lesion.  Slight  loss  of  epicritic  touch  is  apt  to  be  an  early  sign. 
Greater  sensory  loss  advances  with  increasing  compression.  Bladder 
and  rectal  disturbances  are  frequent.  Small  tumors  (cysts,  etc.),  intra- 
medullary, sometimes  extramedullary,  cause  typical  dissociation  symp- 
toms with  retention  of  epicritic  tactile  sensibility  and  loss  of  proto- 
pathic  pain  and  thermal  sensibility. 

The  symptoms  for  localization  of  the  tumor  have  already  been 
discussed. 

Diagnosis. — X-ray  examination,  spinal-fluid  examination,  and  a 
complete  neurological  status  should  enable  one  to  arrive  at  a  satis- 
factory diagnosis.  The  chief  diagnostic  problem  is  syphilitic  meningo- 
myelitis.  Bony  disease  is  usually  excluded  by  the  .r-rays.  Multiple 
sclerosis  is  not  infrequently  ushered  in  with  sensory  signs,  also  sjTingo- 
myelia.  They  present  real  difficulties  in  diagnosis.  The  application 
of  proper  neurological  and  psychoanalytic  procedures  will  determine 
the  conversion  mechanisms  of  hysteria. 

Intramedullary  or  extramedullary  diagnosis  is  practicafly  unim- 
portant since  spinal  cord  surgery  has  become  so  satisfactory.  Theoret- 
icallv  emploved,  the  problem  is  of  interest;  practically,  less  so.  For 
localization  symptoms  consult  Figs.  278,  279,  280,  287,  288,  and  Plates 
X  and  XI. 

Treatment. — Apart  from  syphilis  the  treatment  is  surgical  and  it 
should  be  employed  early  and  for  practically  any  or  all  tumors;  not 
necessarily  with  the  promise  of  cure  but  as  a  routine  exploratory  pro- 
cedure. In  skilled  hands  the  danger  is  slight,  and  apart  from  special 
contra-indications,  the  risk  is  very  frequently  worth  while,  as  very 
unexpected  things  turn  up  within  the  spinal  cavity.^ 

The  localization  is  the  most  difficult  problem.  Most  tumors  are 
found  a  couple  of  inches  above  the  site  usually  sought  for  as  determined 
by  the  level  of  the  anesthesia.  Surgical  progress  relative  to  spinal  cord 

'  Elsberg;    Surgery  of  the  Spinal  Cord.     Frazier:    Surgery  of  the  Nervous  System. 


488  LESIONS  OF  THE  SPINAL  CORD 

tumors  is  rapidly  advancing  and  cannot  be  discussed  here.  The 
general  prognosis  has  improved  both  with  reference  to  the  finding,  as 
well  as  the  successful  removal  of  the  tumors.  There  may  be  recurrences, 
and  many  patients  are  not  benefited.  Little  is  to  be  expected  in  those 
long-standing  tumor  cases  with  marked  signs  of  spinal  cord  compression 
degeneration.  Yet  every  patient  is  entitled  to  the  benefit  of  the  doubt. 
Surgical  failure  is  better  than  a  let-alone  policy  covered  up  by  morphin- 
ism. This  latter  even  is  not  necessary,  since  careful  division  of  the 
affected  sensory  roots  in  inoperable  cases  may  give  relief  from  pain, 
even  if  life  cannot  be  saved. 

Most  patients  die  of  the  myelitis  bed-sore  and  bladder  complications 
after  one  or  two  years  in  inoperable  cases. 

LATERAL  SCLEROSIS  GROUP. 

Historical. — These  disorders,  which  clinically  may  resemble  a  number 
of  spinal  cord  conditions,  have  been  isolated  from  the  mass  of  rachial- 
gias  of  the  eighteenth  century,  and  from  the  paraplegias  and  myelitides 
of  the  nineteenth,  more  particularly  by  Seguin,  Tiirck  and  Erb.  The 
latter,  in  1875,^  posited  a  hypothetical  degeneration  of  the  pyramidal 
tracts  in  their  spinal  course,  terming  the  disease  primary  spastic 
paralysis.  Charcot  accepted  Erb's  teachings  and  spoke  of  the  dis- 
turbance as  a  spasmodic  tabes  dorsalis.  The  disorder,  as  understood 
by  Erb,  has  b(een  found  to  be  much  less  frequent  than  was  at  first 
supposed,  as  the  lateral  sclerosis  picture  was  found  to  be  but  one  stage 
of  a  number  of  other  cerebral  and  spinal  affections,  notably,  as  in 
multiple  sclerosis,  syringomyelia,  hydrocephalus,  tumor  of  the  cord, 
anemia  of  the  cord,  diffuse  myelitis,  senile  changes  and  amyotrophic 
lateral  sclerosis,  etc.  Of  recent  summaries,  those  of  Erb^  and  Spiller^ 
are  available. 

Pathology. — Ideally  this  consists  of  a  simple  degeneration  of  the 
pjTamidal  tract  which  rarely  ascends  to  the  cortex.  A  replacement 
glia  infiltration  is  present.  But  few  autopsies  are  recorded  with  the 
ideal  lesion. 

Symptoms.— These  are  exclusively  motor,  and  usually  of  the  lower 
extremities  alone,  although  the  disorder  may  show  itself  in  the  arms. 
The  ordinary  picture  is  that  of  a  spastic  paraplegia,  i.  e.,  motor  weak- 
ness, increased  reflexes,  clonus,  Babinski,  spasticity,  with  no  sensory 
or  visceral  signs. 

Gradually  increasing  weakness  of  one  or  both  legs  is  first  observed 
after  a  long  walk,  or  dancing,  or  any  prolonged  muscular  exertion. 
A  certain  stiffness  develops,  but  very  slowly,  often  only  after  several 
years.    The  tendon  reflexes  are  exaggerated  at  a  very  early  date. 

Slight  motor  weakness  supervenes,  and  some  stiffness  to  passive 
motion,  and  then  the  patients  note  that  their  gait  is  more  constrained; 

1  Virchows  Arch.,  vol.  Ixx. 

*Deutsch.  Ztschr.  f.  Nervenh.,  1903,  No.  23. 

'  Oslei's  Modern  Medicine,  2  ed.,  vol.  v.     Bono:     Rev.  d.  med.,  March,  1908. 


LATERAL  SCLEROSIS  489 

they  do  not  bend  their  knees,  but  shuffle  somewhat  and  stumble 
easily.  The  limbs  become  stiffer  and  stiffer,  the  extensor  muscles 
being  more  involved,  this  brings  about  extreme  rigidity  at  the  joints. 

In  this  stage,  which  may  be  arrived  at  in  a  few  years,  or  more  often 
after  many,  the  patient  walks  with  a  stiflf,  stubbing  tread,  perhaps 
the  knees  overlap  (scissors  walk)  or  knock  each  other;  the  toes  are  not 
lifted,  but  rather  shoved  along,  thus  wearing  the  shoes  markedly  at 
the  toe. 

The  cutaneous  and  tendon  reflexes  are  exaggerated.  There  are 
markedly  increased  knee-jerk,  patellar  clonus,  ankle-clonus,  Babinski, 
and  paradoxical  and  Bechterew-Mendel  signs;  Striimpell's  tibialis 
phenomenon  is  apt  to  be  marked.  Spasms  frequently  occur  in  the 
affected  limbs.    In  the  latest  stages  flexor  contractm-es  usuall}'  result. 

Sensory  symptoms,  ssLve  the  muscular  pains  of  spasm,  are  absent. 
The  bladder  and  rectum  are  not  implicated.  In  rare  instances  the 
upper  extremities  are  involved,  and  even  the  bulbar  motor  tracts. 

Forms  and  Varieties. — 1.  Hereditary  Familial  Types  (Strijmpell,^ 
Newinark^). — Here  the  disorder  shows  itself  either  in  childhood,  or 
late  in  life,  and  many  generations  may  be  affected. 

The  symptoms  are  those  of  muscular  hypertonia,  spasticity,  exag- 
gerated tendon  reflexes,  and  later  palsies  and  contractures.  The 
skin  and  anal  reflexes  are  less  apt  to  be  involved  in  the  familial  tj'pes. 
In  some  cases  (Xewmark,  Lorrain)  there  are  more  widespread  disorders, 
optic  atrophy,  feeble-mindedness,  muscular  atrophies,  etc.  Deep  sensi- 
bility is  occasionafly  involved  with  slight  Romberg. 

The  changes  in  these  patients  tend  to  become  more  or  less  stationary 
after  a  certain  length  of  time,*  but  the  disease  may  extend  to  the  upper 
part  of  the  cerebrospinal  axis. 

2.  Infantile  Types. — Infantile  types  beginning  from  three  to  six 
years,  are  due  to  developmental  defects  in  the  pyramidal  system.  Here 
the  lower  extremities  are  most  involved.  The  advent  of  the  Wasser- 
mann  technic  is  relegating  the  greater  number  of  these  to  some 
intra-uterine  or  early  sj'philitic  process. 

3.  Unilateral  Ascending  and  Descending  Tyyes^ — ^The  validity  of 
this  t\^e  is  not  yet  established.  It  shows  itself  as  a  gradually  pro- 
gressive hemiplegia,  supposedly  due  to  primary  degeneration  of  the 
pyramidal  tracts. 

4.  Mixed  Types. — These,  previously  described  as  due  to  sj'phUis,  to 
lead,  to  lathyrus  poisoning,  anemia,  etc.,  are  more  properly  more  or 
less  irregular  forms  of  myelitis,  and  are  treated  under  that  title. 

5.  Congenital  Type  (Little's  Disease). — This  will  be  considered  under 
Diseases  of  the  Brain.  The  p>Tamidal  tract  disease  is  secondary  to 
other  lesions. 

Diagnosis. — Pure  tj^es  of  lateral  sclerosis  are  rare.  The  underlying 
condition  often  develops  after  careful  observation,  sometimes  extended 

*  Arch.  f.  Psych.,  x,  xvii.  *  Deutsch.  Ztschr.  f.  Nervenh.,  1904,  p.  27. 

*  Bono:     Rev.  d.  m6d.,  March,  1908,  for  summary  of  all  cases  tc  1908. 

*  Mills  and  Spiller:     Bull.  Univ.  Penn.,  1906. 


490  LESIONS  OF  THE  SPINAL  CORD 

over  several  years.  The  chief  disorders  to  bear  in  mind  are  multiple 
sclerosis,  compression  from  tumor,  amyotrophic  lateral  sclerosis, 
myelitis,  old  encephalitis,  brain  tumor,  with  or  without  hydrocephalus. 

Hysteria  can  be  readily  excluded  by  the  careful  scrutiny  of  the 
reflexes  (Babinski,  Grasset,  Hoover  signs,  etc.) 

Treatment. — Foerster's  operation,  or  posterior  root  section,  may 
help  the  spasticities  in  some  patients.  At  the  present  time,  nothing 
is  known  that  will  stop  the  advance  of  the  disorder.  A  Wassermann 
examination  should  be  made  in  all  cases,  as  in  some  an  unknown 
syphilitic  element  has  been  revealed.  Motor  training  (Mensendieck, 
Franz  methods)  will  help  the  patient  to  control  the  spasticities. 

COMBINED  SCLEROSES.     COMBINED  DEGENERATIONS. 

One  speaks  didactically  of  a  combined  sclerosis  as  a  system  disease, 
in  which  the  lateral  and  posterior  columns  are  involved;  clinically 
a  combination  of  tabes  and  lateral  sclerosis.  It  is  a  doubtful  question 
whether  any  one  disease  exists  which  may  be  designated  as  a  combined 
sclerosis,  but  a  great  number  of  conditions  are  found  in  which  both 
lateral  and  posterior  columns  are  degenerated  to  a  greater  or  less 
extent,  and  as  a  result  varying  symptom-pictures  are  present  as  the 
one  or  the  other  is  more  or  less  implicated. 

Thus  in  true  tabes,  degeneration  of  the  lateral  columns  not  infre- 
quently occurs;  in  general  paresis  there  are  typical  combined  scleroses; 
in  many  cases  of  syphilitic  meningomyelitis,  degenerations  of  posterior 
and  lateral  columns  occur,  likewise  in  severe  anemias,  in  poisoning 
from  lead,  ergot,  pellagra,  lathyrus,  various  bacterial  and  protozoan 
infections;  in  the  senile  cord  similar  changes  are  found.  In  fact,  a  great 
variety  of  degenerations  are  found  in  the  cord  involving  both  sets  of 
columns  to  a  greater  or  less  extent.  Sano  proposes  to  divide  this  group 
into  the  pseudosystem  diseases  and  the  polysystem  diseases.^ 

Whether,  as  Westphal  first  maintained  (1867),  there  also  exists  in 
this  motley  medley  a  true  system  disease  of  these  columns  is  not  yet 
a  settled  question.  Among  this  large  group,  however,  certain  clinical 
types  stand  out,  which  permit  of  more  or  less  clear-cut  description.  In 
practice  they  are  separated  one  from  another  only  with  great  difficulty. 
By  Leyden  and  his  school,  most  of  these  disorders  were  classed  with  the 
chronic  myelitides.  Henneberg^  uses  the  term  funicular  myelitis  for 
one  group  of  non-system  combined  degenerations.  These  latter  are 
usually  due  to  blood  changes,  and  should  be  classed  with  the  myelitides 
rather  than  with  the  combined  scleroses. 

The  more  fixed  of  the  combined  scleroses  types  which  are  here 
considered  are:  (1)  combined  sclerosis  (ataxic  paraplegia  (Westphal)), 
and  spastic  paraplegia  (Striimpell)  forms;  (2)  general  paresis  forms 
(see  under  General  Paresis);  (3)  toxic,  anemic  forms;  (4)  senile  forms. 
There  are  many  intermediary  forms. 

1  Bull,  de  I'Acad.  Roy.  de  M6d.  de  Belg.,  1903. 

2  Arch.  f.  Psychiat.,  1905,  40. 


COMBINED  SCLEROSES  491 

1.  Combined  Sclerosis  (Ataxic  Paraplegia  (Westphal)  Types). — 
Here  the  characteristic  features  are  those  of  a  spinal  tabes,  with  some 
signs  of  spasticity,  i.  e.,  Babinski's  reflex,  and  a  crawling  rather  than  a 
t^'pical  tabetic  gait.  Ataxia,  pains,  bladder  disturbances,  sometimes 
pupillary  stiffness,  etc.,  indicate  that  the  meningoneuritic  element  is 
predominant,  the  pjTamidal  tract  involvement  of  less  marked  extent. 
Certain  patients  start  with  typical  ataxic  signs,  then  gradually  develop 
spasticities  and  the  spastic  element  finally  becomes  predominant. 

Spastic  Ataxia  Type. — Here  the  spastic  element  enters  predominantly 
into  the  picture.  Weakness  precedes,  the  gait  then  becomes  stiff,  and 
the  toes  drag;  there  are  increased  skin  and  tendon  reflexes,  just  as 
in  atj^pical  lateral  sclerosis;  then  pains  develop;  radicular  sensory 
distm-bances  commence;  the  knee-jerks  become  diminished;  hypotonia 
gradually  takes  the  place  of  hypertonia;  one  leg  may  be  h^-potonic, 
the  other  hypertonic;  visceral  disorders  are  added,  and  ataxia  and 
Romberg  are  present.  Possibly  there  is  added  optic  atrophy,  or 
pupillary  stiffness.  Nystagmus  is  not  infrequent.  The  Babinski  reflex 
is  apt  to  persist. 

The  course  in  both  forms,  of  which  there  are  all  possible  gradations, 
is  chronic.  Complete  disuse  of  the  lower  limbs  follows.  The  patients 
are  bed-ridden  with  contracted,  drawn-up  limbs.  Twitchings,  spasms, 
and  bed-sores  supervene. 

2.  Combined  Sclerosis  in  Paresis. — (See  Paresis.) 

3.  Toxic  Anemic  Forms. — Here  one  may  group  a  motley  array  of 
combined  scleroses.  These  are  due  to  poisoning  from  the  lepra  bacillus, 
to  diabetes,  to  lath\Tus,  to  pellagra,  to  ergot,  to  alcohol,  carcinosis, 
malaria,  chronic  septic  states,  pathogenic  protozoa,  tuberculosis, 
Addison's  disease,  anemia,  leukemia,  etc.^ 

The  anemic  t\'pes  offer  special  features,  especially  those  of  pernicious 
anemia.^  The  anemias  may  be  of  manifold  pathogeny.  They  are 
infrequent.  The  patients  complain  of  paresthesia  of  the  extremities, 
there  is  slight  paresis  and  frequently  some  ataxia  which  latter  is  prone 
to  increase  with  loss  or  increase  of  tendon  reflexes,  deep  sensibility  loss. 
In  the  spastic  types  the  knee-jerks  are  increased  with  clonus,  Babinski, 
and  increased  knee-jerks.  In  the  ataxic  t^-pes  there  is  a  trend  toward 
loss  of  reflexes,  etc.  The  spinal  disorder  in  the  pernicious  anemic  t^'pe 
is  progressive,  and  the  patient  dies  in  a  year  or  two  with  great  emacia- 
tion, cachexia,  and  prostration.  The  precise  relationship  between  the 
spinal  changes  and  the  pernicious  anemia  is  not  definitely  understood. 
Other  types  have  similar  symptoms  but  vary  in  their  course. 

4.  Senile  Forms. — Slowly  progressing  weakness  of  the  limbs,  with 
numbness,  palsies,  and  stift'ness  is  frequent  in  many  old  people.  There 
develops  a  shuffling  gait,  and  gradually  a  more  or  less  complete  para- 
plegia with   increased   reflexes,   Babinski   and   clonus.     The   upper 

'  For  literature  to  1903,  see  Sano:   loc.  cit. 

'Lube:  Deutsch.  Ztschr.  f.  Nervenh.,  1914,  xlvi,  299.  Lichtheim,  1887.  Putnam 
and  Dana:  Jour.  Nerv.  and  Ment.  Dis.,  1891.  Minnich,  1892.  See  Nonne  for  different 
types,  Deutsch.  Ztschr.  f.  Nervenh.,  1895. 


492  LESIONS  OF  THE  SPINAL  CORD 

extremities  share  somewhat  in  the  feebleness,  tremor  and  spasticity. 
These  senile  myelopathies  are  very  diverse  in  their  nature.  Lher- 
mitte'  finds  (1)  perivascular  sclerosis,  (2)  marginal  scleroses,  and  (3) 
combined  scleroses  of  the  pyramidal  and  posterior  tracts.  Crouzon 
has  found  in  this  last  group,  paretospasmodic,  ataxospasmodic,  and 
ataxo-cerebello-spasmodic  types.  Thus  it  may  be  seen  that  the  senile 
cord  offers  a  great  variety  of  pathological  changes  with  a  large  series 
of  closely  related  clinical  pictures. 

SYRINGOENCEPHALOMYELIA. 

Historical. — As  long  ago  as  1564,  Etienne  described  cavity  formation 
in  the  spinal  cord;  it  was  further  recorded  a  hundred  years  later  by 
Bonet  (1688)  in  his  celebrated  Sepulchretum.  Morgagni  (1740)  and 
Portal  (1800)  saw  and  described  cases,  and  OUivier,  of  Anglers  (1834), 
first  gave  the  name  syringomyelia  to  what  had  been  taught  by  Etienne 
to  be  a  persisting  central  canal,  but  which  Ollivier  claimed  was  a  patho- 
logical formation. 

The  studies  of  Gull,  of  Landau,  and  Nonat,  with  those  of  Stilling 
and  Waldeyer,  first  gave  the  impetus  to  the  correlation  of  the  ana- 
tomical lesion,  and  the  clinical  symptoms.  Duchenne  (in  1853)  then 
called  attention  to  certain  muscular  atrophies  with  sensory  anomalies, 
which  differed  markedly  from  the  chronic  muscular  atrophies  he  was 
describing;  but  it  was  not  until  as  late  as  1882  that  both  Kahler  and 
Schultze  brought  out  the  factors  that  permitted  a  diagnosis  during  life, 

This  marks  the  period  of  active  neurological  interest  in  the  disease 
with  a  sudden  growth  in  its  symptomatology.  Wichmann  (1887)  and 
Anna  Baumler  (1888)  published  monographs,  the  latter  collecting 
112  cases. 

In  the  six  years  following,  up  to  the  appearance  of  the  first  edition 
of  Schlesinger's  masterly  monograph,  contributions  appeared  from 
everywhere,  and  the  related  subjects  of  leprosy  and  Morvan's  disease 
took  on  an  active  interest.  Schlesinger's  (1894)  monograph  fixed  the 
lines  of  research,  which  are  shown  in  his  thorough  and  monumental 
second  edition  of  1902,  a  monograph  of  some  600  pages,  since  which 
time  few  striking  additions  have  been  made.  Haenel's  contribution  to 
Lewandowsky's  Handhuch  gives  the  later  literature  to  1911. 

Etiology. — Men  are  more  frequently  affected  than  women,  in  about 
the  proportion  of  2  to  1.  About  70  per  cent,  of  the  cases  occur  before 
the  age  of  forty,  the  greatest  age  of  incidence  being  between  the  ages 
of  twenty  and  thirty.  It  is  not  known  that  occupation  has  any  bear- 
ing on  the  etiology.  Toxic  factors  are  not  proved;  infections  may 
play  a  role  in  causing  cord  hemorrhages,  emboli  or  thrombi,  with 
secondary  cavity  formation — Schlesinger  believes  that  in  such  develop- 
ments the  cord  was  not  previously  normal.  Syphilis  may  be  such  an 
infecting  agent,  also  the  typhoid  bacillus.    Syringomyelia  in  mother 

1  Th^se  de  Paris.  1907. 


SYRINGOENCEPHALOM  YE  LI  A 


493 


and  son,  and  in  different  members  of  the  same  family  has  been  observed, 
but  Schlesinger  did  not  note  any  hereditary  history  in  any  of  his 
numerous  cases. 


Fig.  291. — Syringomyelic  ca\'ity  in  the  cord  (cervical  region.) 


Fig.  292. — Another  level  of  the  cord,  showing  gliomatous  tumor  cavity, 


Traumatism  undoubtedly  ])lays  a  role.  Hematomyelia  develops 
after  spinal  traumata,  and  then  may  give  rise  to  secondary  cavity 
formation;  large  numbers  of  which  have  been  reported  resulting  from 
the  Great  War.    Local  traumata  with  ascending  neuritis  have  possibly 


494  LESIONS  OF  THE  SPINAL  CORD 

given  rise  to  later  developing  syringomyelia.  Leprosy  may  also  con- 
dition a  syringomyelia. 

The  essential  feature  is  an  organ  inferiority.  The  various  factors 
just  enumerated  are  purely  contributory,  either  singly  or  one  or  more 
together. 

Symptoms. — Like  multiple  sclerosis,  syringomyelia  is  characterized 
by  its  extremely  rich  and  variable  symptomatology;  like  this  disorder 
also,  almost  anything  is  to  be  expected,  since  with  the  gradual  increase 
in  extension  of  the  cavity  formation  in  the  cord,  new  areas  become 
involved,  and  older  boundaries  enlarged.  It  is  therefore  a  disorder 
which  is  apt  to  show  a  gradually  developing  symptomatology,  and 
persisting  as  it  does  many  years,  a  rich  array  of  pictures  may  be 
presented.  Again  it  is  very  irregular  in  its  manifestations,  although 
certain  features  form  the  background  of  the  disorder.  These  chief 
features  are:  (1)  a  peculiar  dissociation  of  the  sensory  impulses:  (2) 
muscular  atrophy  and  other  trophic  disturbances,  affecting  chiefly  the 
skin  and  joints;  (3)  motor  disturbances  either  irritative  or  paretic  in 
character.  Not  all  of  the  cases  show  these  symptoms,  but  inasmuch 
as  the  cavity  formation  is  apt  to  occupy  certain  portions  of  the  cord 
more  often  than  others,  this  grouping  of  symptoms  occurs  more  often 
than  other  groupings. 

1.  Sensory  Dissociation  (Kahler,  Schultze). — ^This  consists  in  a  loss 
of  ability  to  recognize  sensations  of  heat  and  cold  and  sensations  of 
pain  but  without  any  loss  of  touch,  especially  of  epicritic  touch. 

This  dissociation  varies  considerably.  It  may  be  absent.  In  one 
patient  or  in  one  area  the  thermo-anesthesia  is  very  profound,  the  loss 
of  pain  sense  less  so — again,  in  others,  the  reverse  is  true.  In  the 
majority  of  patients  the  loss  is  only  a  partial  one,  not  an  absolute  one. 
Most  cases  if  seen  early  enough  will  show  only  slight  reductions — 
epicritic  heat  and  cold  are  lost  before  protopathic  heat  and  cold — 
sometimes  the  reverse  is  true,  or  thermo-anesthesia  and  analgesia 
may  be  present  on  one  side  only.  Again  heat  or  cold  alone  may  be 
affected.    Epicritic  touch  may  be  involved,  but  it  is  not  usual. 

These  sensory  anomalies  may  be  distributed  over  very  small  areas, 
rarely  bulbar  (mucous  surfaces  included),  most  often  cervical;  or 
they  may  extend  almost  throughout  the  entire  spinal  axis  from  the 
trigeminus  to  the  cauda  (loss  of  testicle  pains,  and  analgesia  of  the 
bladder,  etc.). 

The  areas  of  diminished  or  lost  sensibility  to  heat  and  pain  are 
usually  bilaterally  asymmetrical,  not  infrequently  they  are  unilateral 
for  a  time,  then  spread  to  the  opposite  side.  They  show  the  most 
unique  distributions.  The  distribution  may  be  exquisitely  radicular; 
again,  it  is  predominantly  segmental  or  metameric.  Schlesinger's 
most  recent  researches  speak  for  the  segmental  type  of  distribution 
for  the  majority  of  the  cases  (Fig.  293). 

In  the  beginning,  one  frequently  encounters  the  glove  and  stocking 
type  of  sensory  changes.  Later  a  whole  limb  will  be  involved;  there 
may  be  girdle  sensation.  A  hemianalgesia,  or  hemithermo-anesthesia 
may  be  present.    Allochiria  is  usually  absent. 


SYRINGOENCEPHALOMYELIA  495 

The  sensory  changes  usually  take  years  to  develop.  This  sensory 
dissociation  is  frequently  preceded  by  paresthesise — burning  pains, 
cold  spots,  neuralgia-like  pains,  etc.  Position  sense  is  rarely  involved, 
even  in  the  advanced  cases.  Tactile  agnosia  (astereognosis)  is  frequent. 
The  bony  sensibility  is  very  frequently  involved  (Egger) .  It  may  also 
be,  although  rarely,  an  initial  sign.  Deep  pressure  sense  is  often 
involved.    Romberg  and  ataxia  are  naturally  not  infrequent. 


Fig.  293. — Syringomyelia,   showing  the   dissociated  loss  of   sensibility.     Areas  of  loss 

of  sensibility  to  pain. 

2.  Muscular  Atrophies  and  Other  Trophic  Changes. — ^These  changes 
occur  most  often  in  the  small  muscles  of  the  hand  (ulnar  distribution), 
and  of  the  arms.  Thus  are  produced  typical  daw-hands,  which  develop 
slowly  and  insidiously.  Here,  again,  no  absolute  rule  is  followed.  The 
interossei  may  atrophy  first,  or  the  muscles  of  the  thenar  or  hypo- 
thenar  eminences.  A  (median)  typical  monkey  atrophic  hand  may  be 
present,  or  the  "preacher-hand."  Or  the  atrophy  in  the  hands  may  be 
skipped  or  combined  with  that  of  the  shoulder-girdle  (Aran-I)uchenne 
types),  the  wings  of  the  scapula  stand  out,  etc.     Atrophy  of  the 


496 


LESIONS  OF  THE  SPINAL  CORD 


trunk  muscles  results  in  various  distortions  of  the  spinal  column. 
Pes  equinus,  pes  valgus,  etc.,  occur  in  the  lower  extremity,  though  less 
frequently.  The  bulbar  nuclei  may  be,  involved,  causing  speech  dis- 
turbances; and  these  may  be  initial  symptoms,  though  infrequent. 
Cerebral  extension  is  even  recorded  (Spiller). 

These  atrophies  are  usually  progressive;  like  the  sensory  changes, 
they  are  usually  bilaterally  asymmetrical,  and  show  much  variability. 
Thus  one  may  find  a  claw-hand  on  one  side,  and  a  monkey-hand  on 
the  other,  etc. 

Reaction  of  degeneration  varies,  being  present  in  some  and  not  in 
others.    Increase  of  electrical  excitability  is  present  in  others. 


Fig.  294. — Sjiingomyelia  ("prediger  hande"). 


Fibrillary  twitchings  are  common  in  the  degenerating  muscles,  and 
even  more  active  movements  are  observed,  consisting  in  static  tremors, 
choreic-like  movements,  intention  tremors,  or  paralysis-agitans-like 
tremors.  Cramps  are  not  infrequent,  and  peculiar  myotonic  contrac- 
tions. These  irritative  motor  phenomena  belong  more  to  the  early 
stages. 

The  motor  power  is  uniformly  diminished  with  the  muscular  atrophy, 
and  spasm  or  contractions  and  rigidity  may  be  present.  Spasticity 
is  not  infrequent  in  the  lower  extremities.  This  only  argues  for 
pressure  upon  or  involvement  of  the  p.>Tamidal  tracts  by  the  lesion. 

The  gait  is  not  infrequently  involved.  The  patients  tire  easily; 
they  commence  to  walk  more  slowly;  hemiplegic  types,  paraplegic 
types,  patients  bent  to  one  side,  or  bent  strongly  forward  are  seen 
in  the  later  stages.  Ataxic  and  tumbling  gaits  belong  to  the  curiosities 
with  rare  bulbar  localizations.  .Schlesinger  reports  a  large  variety  of 
rare  anomalies. 


S  YRINGOENCEPHALOM  YE  LI  A 


497 


Trophic  Disturbances. — ^These  may  be  many.  They  may  vary  in 
number  and  extent  in  strict  accordance  with  the  involvement  of  the 
trophic  cells  in  the  cord. 

In  the  skin  one  finds  h^^peremias,  either  active — idiopathic  conges- 
tive erythemas — or  passive,  with  the  formation  of  dark  red  patches  or 
various  patches  of  a  dark  blue  color — cyanotic.  These  changes  are 
usually  associated  with  others  of  the  muscles  or  of  the  bones. 
Cutaneous  anemias  and  cutaneous  edemas  (succulent  hand),  with 
or  without  distinct  Raynaud  s\Tnptoms,  may  occur.  The  glandular 
activities  of  the  skin  also  suffer.  The  perspiration  may  be  absent,  or 
excessive,   or  one  encounters  curious  anomalies,   such  as  increased 


Fig.  295. 


-Syringomyelia,  showing  the  curvature  of  the  back  and  the  atrophy  of  the 
muscles  of  the  hands. 


perspiration  to  cold  stimuli,  or  islets  of  "increased  perspiration  in  normal 
skin,  or  perspiration-absent  areas.  These  areas  of  altered  perspiration 
show  anomalous  distributions  quite  comparable  to  those  seen  in  the 
sensory  sphere.    Changes  in  the  fatty  secretions  may  be  looked  for. 

Chronic  dermatoses  are  also  encountered  with  exudative  phenomena; 
urticarias,  angioneurotic  manifestations,  phlyctenulae,  dermatitis  bul- 
losa. Hemorrhagic  infiltrations,  or  even  ulcer  and  gangrene  of  the 
skin  are  known.  True  hypertrophies,  sclerodermas  and  changes  in 
the  nails  are  recorded. 

The  bony  structures  also  suffer.  Arthropathies  are  common  (10 
per  cent. — Sokoloff ;  25  per  cent.^Schlesinger),  but  more  often  in  the 
32 


498 


LESIONS  OF  THE  SPINAL  CORD 


Fig.  296. — Pontine  syndrome,  with  eye  palsies  of  central  origin  and  syringomyelic  dis- 
sociation. There  is  here  a  crossed  hemianesthesia  with  alternating  paralysis  of  the 
VI  and  VII  cranial  nerves,  anesthesia  of  the  V  nerve  due  to  hemorrhage  in  the  lateral 
and  lower  portion  of  the  pontine  tegmentum  of  the  left  side.  The  right-hand  figure 
shows  the  hemianesthesia,  dissociated  as  in  syringomyelia  (hemianalgesia  and  hemi- 
thermanesthesia  due  to  lesion  of  the  crossed  sensory  pathways  of  the  lateral  portion  of 
the  reticular  formation.  There  is  preservation  of  the  tactile  and  postural  sensibilities 
and  of  the  stereognostic  sense,  because  of  the  incomplete  extension  of  the  lesion  to  the 
median  lemniscus  (Rm).  The  left-hand  figure  shows  (1)  atrophic  paralysis  of  the  VII 
nerve  with  reaction  of  degeneration,  lagophthalmia,  drooping  of  the  lips,  loss  of  facial 
mimicry,  paralysis  of  the  entire  left  facial  (VII)  indicated  (Fig.  a) :  (2)  anesthesia  of 
the  face,  following  involvement  of  the  descending  root  of  the  trigeminus  (see  V  on  a') ; 
(3)  paralysis  of  the  external  rectus  with  convergent  strabismus  by  reason  of  the  over- 
action  of  the  antagonists.  Furthermore,  there  is  a  paralysis  of  the  lateral  movements 
of  the  eyeballs  toward  the  left  notwithstanding  the  integrity  of  the  posterior  longitu- 
dinal fasciculus  (Ftp.),  of  the  nucleus  of  the  VI  and  of  the  adjacent  reticular  formation. 
The  lesion  of  Deiters'  nucleus,  and  of  the  labyrinthine  oculorotary  fibers  which  unite 
Deiters'  nucleus  (ND)  to  the  nuclei  of  the  III  and  VI  causes  this.  By  reason  of  the 
overaction  of  the  antagonists  the  patient  looks  to  the  right.  (After  Dejerine.)  For 
abbre\dations  on  the  anatomical  sketch  see  section  on  Midbrain  and  Plate  VIII. 


SYRINGOENCEPHALOMYELIA  499 

upper  extremities,  in  sharp  contrast  to  the  lower  hmb  distribution  of 
tabes.  They  usualh'  occur  late  in  the  disease  and  persist  for  many 
years.  The  onset  is  usualh'  acute,  with  pain,  swelling,  and  destruction. 
Mild  cases  cause  little  or  no  deformity.  Both  atrophic  and  h^-per- 
trophic  changes  occur,  with  fixation  of  the  joints,  dislocations  or  frac- 
tures. The  sensory,  secretory  and  trophic  disturbances  are  usually 
in  closely  related  areas,  and  hony  sensory  anomalies  are  to  be  expected. 
Occasionally  suppurations  occiu. 

Bony  changes  in  the  vertebrae,  with  thorax  deformities,  occur  either 
as  a  consequence  of  arthropathies,  or  as  muscular  atrophies.  These 
scolioses  occur  usually  in  the  upper  dorsal,  and  give  rise  to  compen- 
satory curvatures. 

The  bony  structiu-es  of  an  entire  limb  may  be  involved — a  hand  or 
a  foot — with  atrophies  of  the  muscles.  Here  acromegaly  is  to  be  ruled 
out  usually  by  the  deformed,  contracted  nature  of  the  sjTingomyelic 
hand  or  foot,  the  marked  muscular  atrophy,  and  the  isolated  character 
of  the  limb  involved.  S\Tingobulbia  may  cause  a  facial  hemiatrophy 
or  hemihypertrophy. 

Reflexes. — Great  variability  and  changeability  is  present.  The  skin 
reflexes  vary  from  complete  loss  to  exaggeration.  The  Babinski  reflex 
may  or  may  not  be  present,  as  well  as  the  abdominal,  epigastric  and 
cremasteric  reflexes. 

The  tendon  reflexes  are  often  striking  in  that  one  reflex  in  the  arm 
for  instance  will  be  lost,  the  others  present,  or  even  exaggerated.  The 
same  holds  true  in  the  lower  extremity,  but  is  less  striking.  Increased 
reflexes  are  here  more  apt  to  be  found.  Increased  knee-jerk,  Achilles- 
jerk,  and  even  ankle-clonus  are  not  infrequent,  particularly  in  high- 
lying  s\Tingomyelias — bulbar,  cervical  and  upper  dorsal.  Lost  knee- 
jerks,  unilateral  or  bilateral,  may  also  occur  in  sjTingomyelia,  either 
as  an  early  or  a  late  symptom,  and  may  be  associated  with  increased 
skin  reflexes  (Babinski). 

Rare  cases  are  encountered  with  increased  jaw  reflexes. 

Visceral  Symj^tovis. — Bladder  disturbances  are  not  the  rule, -although 
at  times  appearing,  usually  late  in  the  disease.  When  appearing  early 
they  are  apt  to  be  transitory.  They  may  be  sensory  or  motor,  irrita- 
tive or  paralytic.  Cystitis  is  not  infrequent  in  the  later  stages  of  the 
disease.  In  sjTingobulbias  one  finds  anomalies  of  secretion,  poljau'ia, 
glycosuria,  diabetes  insipidus,  pollakiiu-ia. 

Obstipation  is  not  uncommon;  incontinentia  alvi  less  so.  Loss  of 
sexual  power  and  of  desire  also  occur.  Persistent  priapism  is  one  of 
the  curiosities  of  this  disorder;  also  analgesia  of  the  testicles. 

Bulbar  Sym2:>toms.—1he^e  are  frequent,  when  isolated  being  then 
termed  syringobulbia.  They  are  usually  more  benign  than  wjien 
found  in  other  affections  (chronic  bulbar  palsy),  have  a  very  chronic 
course,  are  usually  unilateral,  and  often  implicate  the  vagus.  They 
occur  in  about  one-third  of  all  cases.  Certain  affections  of  cranial 
nerves  have  been  recorded  for  many  years.  Smell  and  hearing  are 
involved  rarely.    Vestibular  vertigo  is  also  rare.    A  dulling  of  the  sense 


500  LESIONS  OF  THE  SPINAL  CORD 

of  taste  is  not  infrequent,  and  shows  irregularities  of  disturbance — 
half -tongue  (Dejerine);  dissociation  (Hitzig,  Simon) — anteroposterior 
changes. 

Optic  nerve  atrophy  or  swelling  of  the  disk  occurs  in  advanced 
bulbar  cases;  a  few  cases  show  restriction  of  the  color  fields.  The  eye 
muscles  may  show  nystagmus  or  nystagmoid  movements,  paralyses 
and  sympathetic  complications.  H,  Simon  reports  an  interesting  case 
of  nystagmoid  movements  with  both  eyes,  absent  when  only  one  was 
in  use.  Schlesinger  reports  transitory  diplopias  in  11  per  cent,  of 
the  cases ;  they  are  not  infrequently  early  signs,  and  do  not  necessarily 
recur.    The  abducens  is  frequently  affected. 

Ptosis  is  not  infrequent,  and  is  often  an  important  early  sign.  Its 
relations  to  the  s\Tnpathetic  are  intimate.  Myosis  is  found  in  a  small 
percentage  of  cases,  and  differences  in  the  pupil  are  frequent,  at  least 
25  per  cent.  (Schlesinger).  They  are  mostly  due  to  sympathetic 
palsies.  Argyll-Robertson  pupil  has  been  observed,  but  chiefly  in  pre- 
Wassermann  days,  hence  the  absence  of  syphilis  has  not  been  proved; 
its  presence  in  syringomyelia  is  anatomically  conceivable. 

The  Klumpke  syndrome  is  frequently  met  with  and  points  to  the 
upper  dorsal  localization  of  the  lesion.  Myosis,  diminished  lid  aper- 
ture, retraction  of  the  bulbi,  and  anomalous  pupillary  reactions  (loss 
of  cocain  dilatation,  etc.)  are  signs  of  sympathetic  involvement. 

The  trigeminus  distribution  shows  sensory  anomalies,  either  as 
paresthesiae  and  pains  (tic  douloureux)  or  as  analgesise,  with  loss  of 
the  trigeminal  reflexes  and  corneal  trophic  changes.  Dissociation  in  the 
trigeminal  distribution  is  also  observed.  Pain  and  temperature  are 
mostly  affected.    Deep  sensibility  is  less  often  involved. 

The  facial  is  involved  in  but  few  cases  (3  per  cent.)  but  impairment 
of  ability  to  swallow  is  not  infrequent.  The  tongue  is  involved,  pro- 
jects to  the  paretic  side,  shows  fibrillary  twitching,  and  hemiatrophy. 
The  soft  palate  and  laryngeal  musculature  is  often  involved  at  the 
same  time,  which  with  the  loss  of  sensation  often  causes  deglutition 
pneumonia. 

Laryngeal  palsies  are  infrequent.  They  are  characterized  by  the 
palsy  of  one  recurrent  nerve,  and  the  usually  accompanying  homo- 
lateral palsies  of  the  pharynx  and  soft  palate.  Posticus  palsy  and 
homolateral  bulbar  palsies  go  hand  in  hand.  From  15  to  20  per  cent, 
of  all  syringomyelias  have  laryngeal  symptoms  which  not  infre- 
quently antedate  the  other  bulbar  or  cranial  nerve  symptoms. 

Speech  disturbances  are  common,  as  one  or  other  of  the  related 
mechanisms  are  apt  to  be  involved.  Recm-rent  palsies  give  the  high, 
rasping  voice,  tongue  involvement,  the  thick,  hot-potato  speech  of 
the  bulbar  paretic;  palatal  palsies  cause  a  "nasal"  voice;  occasion- 
ally a  scanning-like  speech  is  observed. 

Tachycardias,  bradj'cardias,  and  dyspnea  are  among  the  rarer 
sjinptoms. 

Apoplectiform  or  epileptiform  attacks  have  been  described,  and  are 
sufficiently  frequent  to  attract  attention.    They,  however,  are  usually 


SYRINGOENCEPHALOMYELIA  501 

accompanied  by  signs  of  vestibular  involvement — rotatory  vertigo, 
nystagmus,  vomiting,  etc. 

Mental  Symptoms. — Syringomyelia  is  often  associated  with  chronic 
hydrocephalus  which  two  conditions  arise  simultaneously.  Here  defect 
symptoms  in  the  form  either  of  debility  or  imbecility  are  present.  Other 
patients  are  reticent,  surly  and  vindictive.  INIany  patients  come  to 
develop  a  totally  perverted  feeling  about  their  anesthesise,  analgesise, 
etc.,  and  cannot  be  convinced  regarding  its  true  character. 

Course  and  Progress.- — The  great  multiplicity  of  s^-mptoms,  and  the 
almost  fortuitous  situation  of  the  tumor  and  cavity  formation  make  it 
inadvisable  to  generalize  too  sharply  about  the  symptom  groupings, 
but  in  general  one  can  outline  four  more  or  less  classical  types.  These 
are  the  bulbar,  the  cervical,  the  dorsolumbar,  and  the  sacrolumbar. 

1.  Bulbar  Types. — Syriiigohilbia. — If  restricted  solely  to  the  med- 
ullary lesions,  this  is  the  least  common  of  the  types  (Schlesinger) . 
Bulbar  symptoms,  however,  are  very  often  found  in  the  other  types. 
In  this  type  the  lesion  is  limited  to  the  cranial  nerves.  The  laryngeal 
palsies  are  prominent;  diflficulties  in  swallowing  are  present,  either 
transitory  or  persistently,  and  cause  death.  Atrophy  of  the  tongue, 
and  paresis  of  the  ocular  muscles  are  frequent.  Sensory  disturbances 
in  the  region  of  the  trigeminus  are  frequently  found. 

Initial  syringobulbias,  in  contrast  to  those  developing  with  or  fol- 
lowing spinal  signs  are  not  as  dangerous  to  life  per  se. 

2.  Cervical  Type. — This  is  the  commonest  and  best  known.  The 
patients  first  complain  of  weakness  in  the  small  muscles  of  the 
hand  with  clumsiness  for  finer  movements.  Paresthesise  and  pains 
are  frequent  in  the  hands  and  arms.  The  patients  frequently  have 
severe  sores  on  the  hands  from  being  wounded  or  burned,  and  then 
note  the  advancing  analgesia,  muscular  wasting,  and  loss  of  ability  to 
tell  hot  from  cold,  analgesise,  total  or  partial,  with  intact  touch  sense. 
The  difficulties  are  first  unilateral,  and  later  spread  asjinmetrically. 
The  knee-jerks  are  apt  to  be  exaggerated.  Advancing  disease  shows 
itself  in  the  greater  atrophy,  with  claw-hand,  preacher-hand,  monkey- 
hand,  and  the  whole  shoulder-girdle  may  show  involvement.  The 
knee-jerks  are  increased,  clonus  and  Babinski  may  be  present.  There 
may  be  beginning  scoliosis  or  kyphosis.  Trophic  disturbances  in  the 
upper  extremities  appear. 

3.  Dorsolumbar  Type. — Strictly  localized  dorsolumbar  types  are 
rare.  Here  the  typical  sjTingomyelic  sensory  anomalies  are  met  with. 
Paresthesise,  pains  followed  by  analgesia,  thermo-anesthesia,  preserva- 
tion of  touch,  kyphoscoliosis  are  frequent.  The  muscles  of  the  pelvic 
girdle  become  involved  and  the  lower  extremities  show  atrophies  and 
deformities.  The  Klumpke  type  of  sympathetic  involvement  is  seen 
here  as  well  as  in  the  preceding  type,  i.  e.,  unequal  papils,  inequality 
in  the  palpebral  fissure,  recession  of  the  eyeball.  The  gait  becomes 
spastic  paretic,  the  tendon  reflexes  usually  increased — at  times  lost. 
Girdle  sensations  and  involvement  of  the  bladder  and  rectimi  are  not 
infrequent.    Secretory  and  trophic  anomalies,  already  noted,  point  to 


502  LESIONS  OF   THE  SPINAL  CORD 

the  localization  of  the  process.  ]\Iany  of  these  cases  closely  resemble 
tumors  of  the  cord. 

4.  Sacrohimhar  Types. — These  are  rare  types,  and  are  characterized 
by  muscle  atrophies,  especially  of  the  lower  extremities,  and  the  smaller 
muscles  of  the  feet.  The  glutei  may  also  be  involved.  Segmental 
sensory  disturbances  of  the  perineum  and  genital  regions  of  the  dis- 
sociated type  referred  to  are  present.  Trophic  disturbances  are  usually 
profound  and  extensive,  such  as  fractures,  ulcerations,  running  sores, 
etc.  Vasomotor  disturbances  are  present.  The  tendon  reflexes  are 
usuall}^  increased.  Babinski  may  be  present.  Bladder  and  rectum 
are  usually  involved.  Contractures  are  common,  and  kyphoses  and 
scolioses  occur. 

Characteristic  Groiqnngs. — Xot  only  do  certain  types  stand  out, 
conditioned  by  the  topography  of  the  intramedullary  lesion,  but 
certain  cases  show  predominant  forms  of  lesion,  such  as  motor,  sensory, 
trophic,  or  secretory.  Thus  certain  cases  resemble  amyotrophic  lateral 
sclerosis  very  closely;  others  again  have  the  general  features  of  a 
spastic  spinal  paraplegia,  others  again  show  a  characteristic  Aran- 
Duchenne  scapulohumeral  atrophy.  A  few  cases  of  general  anesthesia 
are  on  record,  and  certain  sensory  types  may  be  confused  with  hysteria. 
Trophic  cases  with  an  isolated  picture  of  Morvan's  disease  are  striking, 
and  tabetic-like  forms  are  likewise  puzzling.  Schlesinger  also  calls 
attention  to  a  pachj-meningitic  type. 

The  illustrative  case  of  Schmitt  and  Baraban,  which  is  not  unique, 
shows  the  striking  variability  that  may  be  present  in  the  symptom- 
atology of  this  disorder.  At  various  times  this  patient  was  diag- 
nosed by  competent  authorities  as  tabes,  chronic  diffuse  myelitis, 
amyotrophic  lateral  sclerosis,  and  spastic  paraplegia  of  unknown 
causation. 

Differential  Diagnosis. — The  chief  disorders  that  come  in  review 
are  atypical  multiple  sclerosis,  amyotrophic  lateral  sclerosis,  tumor  of 
cord  with  spastic  paraplegia,  diffuse  sclerosis,  progressive  muscular 
atrophy,  central  myelitis,  syphilitic  meningomyelitis,  polyneuritis, 
leprosy,  pellagra  and  ergotism. 

The  course  of  the  disease  is  very  chronic.  Large  cavity  formations 
involving  areas  controlling  the  functions  of  the  intestines  and  bladder 
soon  lead  to  death  in  from  four  to  six  years;  whereas  if  the  lesion 
does  not  compress  the  entire  cord  and  lies  outside  of  the  more  vital 
nuclei  the  patients  may  live  thirty  to  forty  years.  Dejerine  has 
reported  a  case  of  fifty  years'  development. 

The  patients  die  more  often  of  intercurrent  disease;  tuberculosis 
in  particular.  Bladder  sepsis,  with  kidney  complications,  is  also 
frequent. 

The  patients  frequently  show  an  up-and-down  course,  quite  analo- 
gous to  the  course  seen  in  multiple  sclerosis. 

Pathology. — Syringomyelia,  pathologically  speaking,  is  due  to  a 
cavity  in  the  cerebrospinal  axis  not  in  genetic  relation  to  the  central 
canal— dilatations  of  this  latter  structure  are  better  termed  hydro- 


S  YRINGOENCEPHALOM  YE  LI  A 


503 


FiQ_  297. Partly  schematic  representation  of  a  widespread  syringomyelia,  showing  the 

lesion  throughout  the  cord  and  at  various  cross  levels  of  the  same.     (J.  Hoffmann.) 


504  LESIONS  OF  THE  SPINAL  CORD 

myelias.  Cavities  due  to  hemorrhage  are  recognized  as  being  different 
from  those  in  syringomyeha  proper  and  are  classed  with  the  hemato- 
myehas.  On  opening  the  spinal  canal,  which  is  rendered  difficult  by 
reason  of  the  vertebral  deformities,  the  diu^a  is  usually  found  to  be 
normal;  occasionally  a  pachymeningitis  complicates  the  picture. 
The  pia  is  usually  thickened,  and  evidences  of  swelling  or  of  some 
internal  irregularity  are  seen  either  as  paths  of  grayish  degeneration, 
or  cystic-like  irregularities,  with  flattening  in  places.  The  posterior 
fissure  frequently  appears  deeply  sunken  in  the  cord,  causing,  as 
Thomas  says,  a  double-barrelled-shotgun  appearance.  The  cord 
collapses  on  cutting  and  clear  fluid  escapes.  Repeated  section,  best 
made  after  hardening  of  the  cord,  shows  the  existence  of  one  or  several 
cavities,  of  variable  length  and  diameter,  and  occupying  various 
situations  in  the  cord.  Its  most  frequent  site  is  behind  the  neighbor- 
hood of  the  central  canal,  usually  involving  the  posterior  commissure, 
and  with  a  tendency  to  reach  backward  more  than  forward.  The  cavity 
seems  to  have  a  special  fibrous  wall,  which  is  well  limited,  smooth 
or  papillated;  often  a  gliomatous  mass  fills  the  lower  end  of  the  canal. 
In  the  hydromyelic  type  the  cavity  is  round  and  usually  occupies  the 
center  of  the  cord.  The  cavity  is  lined  first  by  a  layer  of  epithelium, 
and  is  surrounded  by  a  gliomatous  wall. 

In  the  syringomyelic  type  there  are  also  some  ependymal  cells, 
but  they  are  less  regularly  arranged.  These  are  interspersed  with 
neuroglia  cells  and  rest  upon  a  solid  wall  of  glia  cells,  many  of  which 
are  in  process  of  disintegration.  Small  vessels  are  frequent,  among 
which  may  be  found  many  undergoing  hyaline  degeneration.  The 
picture  is  different  at  every  level,  and  in  many  sections  the  cavity 
has  no  lining  at  all  in  places,  bordering  directly  upon  the  nervous 
tissues.  Sections  through  the  glioma  show  perhaps  no  cavity  at  all. 
Fresh  hemorrhagic  remams  of  old  hemorrhagic  foci  are  frequent 
findings. 

The  glioma  may  be  sharply  delimited — central  gliosis  or  the 
neurogliar  tissue  may  infiltrate  the  cord  in  all  directions — diffuse 
gliosis. 

Secondary  degeneration  in  the  parts  impinged  upon  or  invaded 
takes  place  by  process  of  atrophy  and  then  of  tissue  replacement  with 
characteristic  neurophagia.    Regenerated  fibers  are  also  encountered. 

In  cases  complicated  with  pachymeningitis  one  finds  the  lesions  of 
this  process,  and  in  the  traumatic  forms  one  usually  finds  the  remains 
of  an  ancient  fracture,  with  inflammatory  thickening  of  the  meninges 
and  pia.  The  cavity  is  usually  posterior,  may  traverse  the  entire  cord, 
midbrain,  and  even  be  found  in  the  cerebral  hemispheres,^  constituting 
a  true  syringomyeloencephalacia. 

Pathogeny. — No  unanimity  of  opinion  has  yet  been  reached.  The 
present  general  trend  is  to  regard  the  process  as  degenerative 
rather  than  one  resulting  from  a  productive  inflammation. ^     The 

>  Spielmeyer:    Ztschr.  N.  u.  P.,  xxv,  543.     Spiller: 
*  Spiller:     Jour.  Nerv.  and  Ment.  Dis.,  1916. 


SYRINGOENCEPHALOMYELIA  505 

general  hj-potheses  are:  (1)  it  is  clue  to  a  defect  of  development; 
(2)  it  is  due  to  an  intramedullary  tumor  formation  which  later  breaks 
down;  (3)  it  is  the  left-over  remains  of  an  inflammatory  (chronic 
myelitis)  or  hemorrhagic  process  (hematomyelia) ;  (4)  it  is  due  to  a 
modification  of  pressure  in  the  ependymal  canal,  brought  about  by  a 
trauma,  a  compression.  In  general  it  seems  that  no  one  hj^othesis 
can  explain  all  of  the  cases. 

Thus,  according  to  Schlesinger,  one  divides  the  cavity  formations 
that  may  occur  in  the  spinal  cord  as  follows : 

1.  Cystic  formation,  after 

(a)  Traumatic  destruction  of  tissue, 
(6)  Traumatic  hematomyelia, 
(c)  Non-traumatic  hematomyelia. 

2.  Softening  of  an  inflammatory  or  non-inflammatory  nature  with 

short  course. 

3.  SjTingomyelias : 

(a)  True  hydromyelia  (as  malformation), 
(6)  True  tumor  with  cavity  formation, 

(c)  Syringomyelic  gliosis, 

(d)  Syringomyelia  from  vessel  disease  "without  gliosis, 

(e)  Pachymeningitis  and  leptomeningitis  with  cavity  formation. 
In  a  similar  manner  the  ca^'ity  formations  of  the  medulla  may  be 

classified  as  follows : 

1.  Cysts  following  softening  or  hemorrhage. 

2.  Softening  of  inflammatory  natiu-e  with  acute  course. 

3.  Cavity  formation  following  degeneration  of  tumors. 

4.  True  sjTingobulbias  and  sjTingoencephalias  (in  tjT^ical  localities). 

(a)  Embryonal,  lying  in  the  center,  and  in  combination  with 

hydromyelia  or  sjTingomyelia. 
(6)  Without  combination  with  hydromyelia  or  s^Tingomyelia. 
(c)  Arising  in  later  life  and  lymg  laterally  as  continuation  of  a 
syringomyelia  due  to  circumscribed  bulbar  or  cerebral 
changes. 
Treatment. — Since  hemorrhage  into  the  glial  cavities  is  a  frequent 
occurrence  in  s^nringomyelia,  it  is  advisable  to  avoid  hard,  muscular 
work,  since  such  seems  to  favor  bleeding.     Severe  muscular  effort 
also  aids  in  spontaneous  fractures.    Occupations  involving  heat  are 
to  be  avoided  because  of  the  danger  from  burning. 

For  the  pains,  the  treatment  is  symptomatic.  Here  the  various 
analgesic  antipyretics  may  be  employed.  Aspirin,  acetanilid,  anti- 
pyrine  in  5-grain  doses  are  sufficient. 

Specific  therapy  is  as  yet  unknown.  One  is  justified  in  recom- 
mending a  spinal  cord  operation  in  the  case  of  large  hemorrhages  with 
sudden  increase  in  pressure  symptoms.  Here  the  principle  of  open 
expression  of  the  clot  can  be  practised  to  advantage  (Elsberg) . 

Certain  advantageous  results  have  been  reported  from  the  use  of 
high-frequency  currents.  These,  however,  have  not  been  employed 
long  enough  to  determine  their  absolute  efficiency.     Beaugard  and 


506  LESIONS  OF  THE  SPINAL  CORD 

Lhermitte^  recommend  weekly  applications  of  penetration  rays,  79 
(radio  chronometer  of  Benoist),  dose  of  3  H,  at  a  distance  of  15  per 
cent,  between  anticathode  and  the  skin.  Twenty-six  seances  are 
recommended. 

MULTIPLE   SCLEROSIS. 

Historical. — Because  of  the  striking  variability  in  the  symptom- 
atology of  this  disorder,  it  is  not  altogether  sm*prising,  historically 
considered,  that  the  first  clues  to  its  final  delimitation  should  have 
been  gained  from  the  pathological  rather  than  from  the  clinical  side. 

Under  the  older  nosological  schemes  one  finds  these  patients  grouped 
as  epilepsy,  paraplegia,  tremor,  chorea,  dance  of  St.  Guy  chronica, 
and  a  host  of  other  conditions.  Although  Sylvius  de  la  Boe  recognized 
an  intention  tremor  as  different  from  other  t^^'pes,  it  cannot  be  said 
that  the  clinical  conception  assumed  its  modern  form  until  the  work 
of  Vulpian  (1862),  Ordenstein  (1868)  and  Charcot.  The  initial 
pathological  notion  was  given,  however,  by  CruveiDiier  (1832-1845) 
in  his  famous  case  of  Darges  (in  1840),  a  cook  in  the  Salpetriere,  a 
sketch  of  whose  cord  is  here  reproduced.  Carswell's  picture,  appear- 
ing in  1838,  is  probably  the  very  first  illustration  of  this  condition  on 
record.  From  this  time  on,  brain  and  spinal  cord  sclerosis  became 
an  active  subject  of  discussion,  in  which  the  work  of  Frerichs^  and 
Valentiner^  stand  out  prominently.  The  former  made  a  diagnosis  of 
brain  sclerosis  during  life  and  found  multiple  sclerotic  patches  at  an 
autopsy,  and  tried  to  erect  a  clinical  entity  with  differential  patho- 
logical diagnosis,  which  later  was  amplified  by  his  student,  Valentiner, 
who  collected  a  series  of  cases  resembling  those  of  Frerichs,  and  who 
also  gave  a  remarkable  summary  of  the  s\Triptoms.  When  these 
reports  are  read  it  may  be  seen  that  they  would  not  be  included  within 
the  modern  concept,  yet  Valentiner  picked  out  the  facts  of  involve- 
ment in  early  years,  the  marked  motor  disturbances,  hemipareses, 
tremors,  speech  disturbances,  the  remissions,  the  unilateral  onset,  the 
greater  involvement  of  motor  than  sensory  function,  the  involvement 
of  the  cranial  nerves,  the  long  com-se  and  bad  prognosis.  The  mental 
symptoms  as  outlined  by  Valentiner  are  not  as  characteristic.  A 
diplegic  idiot,  for  instance,  is  included  in  his  series  of  15  cases. 

The  first  real  foundation  of  the  sjonptomatology  may  be  said  to 
have  been  laid  down  by  these  two  authors,  and  little  practical  progress 
was  made  until  the  work  of  Vulpian,  Ordenstein  and  Charcot  (1862- 
1869)  and  his  pupils,  as  summarized  in  the  monograph  of  Bourneville 
et  Guerard  (1869),  when  the  multiple  sclerosis  of  the  present  day 
assumed  definite  form.  As  early  as  1862  Vulpian  and  Charcot 
brought  the  classical  triad,  scanning  speech,  nystagmus,  and  intention 
tremor,  into  prominence  as  diagnostic  of  the  condition,  and  in  the 

'  Sem.  med.,  1907. 

*  Ueber  Hirnsclerose,  Arch.  f.  d.  ges.  Med.  Haeser,  1849,  p.  334. 

'  Ueber  die  Sclerose  des  Gehirns  und  Riickenmark,  Deutsch.  Klinik,  1856,  p.  147. 


MULTIPLE  SCLEROSIS 


507 


later  studies  of  the  Charcot  school  the  clinical  and  pathological  foun- 
dations were  laid  much  as  they  were  held  up  to  within  recent  times. 


■ 


/ 

Fig.  298  Fig.  299 

Figs.  298  aud  299. — Early  sketches  by  Carswell  and  Cruveilhier  (1838  and  1840)  of  the 
sclerotic  patches  in  multiple  sclerosis. 

It  is  becoming  more  and  more  evident,  however,  that  the  picture 
insisted  upon  by  Charcot  is  not  a  satisfactory  one.     Sensory  pictures, 


508  LESIONS  OF   THE  SPINAL  CORD 

neglected  by  him,  had  to  be  reckoned  with  in  the  later  studies;  also 
interference  with  the  bladder.  Decubitus  as  a  symptom  occurs. 
Then  cerebellar  syndromes  were  recognized;  then  Erb's  spastic  par- 
alysis fell  largely  into  the  multiple  sclerosis  group,  then  this  author's 
chronic  dorsal  myelitis  with  optic  changes.  Hemiparesis  not  due 
to  embolism  or  thrombi  became  recognized.  Alternating  Millard- 
Gubler  tjpes  were  described.  Then  many  bulbar  palsies  passed  into 
its  confines,  pontine  encephalic  pictures,  pure  ophthalmoplegias  and 
occasional  forms  with  painful  attacks,  resembling  s\Tphilitic  meningo- 
myelitis.  Amyotrophic  lateral  sclerosis  (Dejerine)  and  pseudoparesis 
were  later  diagnostic  possibilities,  showing  the  extreme  multiplicity 
of  forms  under  which  this  disorder  may  show  itself.  The  best  recent 
monograph  of  real  value  is  that  of  Miiller,  1904.  The  more  recent 
literature  is  gathered  by  Wohlwill^  and  Marburg^  while  the  histology 
has  been  exhaustively  treated  by  Dawson,^  Klingman  and  Spiller.'* 

At  the  outset  it  is  desirable  to  call  attention  to  the  fact  that  there 
exists  a  multiple  sclerosis  due  to  a  fairly  definite  pathological  process, 
and  other  clinical  t\T)es  resembling  the  former  so  clearly  as  to  be  clinic- 
ally indistinguishable,  but  in  which  one  finds  multiple  tumors,  multiple 
endarteritic  lesions  (sj^philitic,  arteriosclerotic),  multiple  encephalo- 
myelitic  inflammatory  processes,  etc.  These  will  be  discussed  with 
the  pathology. 

Primary  multiple  sclerosis  then  is  a  disease  essentially  of  adolescent 
or  young  adults,  beginning  very  gradually  for  the  most  part,  advancing 
slowly,  initially  very  varied,  but  ultimately  assuming  a  very  charac- 
teristic, almost  stereotyped  character.  It  is  characterized  by  begin- 
ning muscular  weakness,  with  spasticity,  by  disturbances  in  speech, 
nystagmus,  intention  tremor,  by  forced  laughing  or  crying,  and  by 
changes  in  the  fundus — temporal  pallor.  Sensory  symptoms  may  be 
present  but  are  not  usually  prominent,  bladder  symptoms  are  not 
uncommon,  while  vasomotor  and  trophic  disturbances  are  compara- 
tively infrequent.  The  process  persists  for  many  years,  shows  strik- 
ing remissions — noted  by  Valentiner  in  1856 — and  the  patients  usually 
die  of  exhaustion  or  intercurrent  disorder.  Occasionally  the  process 
comes  to  a  standstill.  In  rare  instances  the  disorder  is  character- 
ized by  running  a  very  rapid  coiu-se — acute  disseminated  sclerosis  of 
Marburg, 

Multiple  sclerosis  is  a  relatively  infrequent  disease.  Of  18,000  cases 
of  nervous  disease  at  Vanderbilt  Clinic  it  was  diagnosed  as  occurring 
twenty-seven  times,  i.  e.,  a  percentage  of  0.001  +  per  cent.  In  Euro- 
pean clinics  it  would  appear  to  be  more  frequent.  B  ram  well-William- 
son show  2  per  cent.  Possibly  better  diagnoses  account  for  these 
differences,  since  the  American  statistics  are  obtained  from  polyclinic 
material.     These  are  not  checked  by  autopsy  findings,  and  are  sug- 

» Ztschr.  f.  N.  u.  P.,  ref.,  1913.  vol.  vii. 

^  Lewandowsky's  Handbuch,  1911,  vol.  ii. 

^Proc.  Royal  Soc,  Edinburgh,  1916. 

<  Archives  of  Neur.  and  Psychiat.,  1919,  i,  193  and  219'. 


MULTIPLE  SCLEROSIS  509 

gestive  rather  than  conclusive  of  its  frequency.  Personal  figures  show 
1  patient  in  200  of  somatic  nervous  disorder,  0.5  per  cent. 

Etiology. — A  definite  position  regarding  this  cannot  yet  be  taken. 
Practically  all  of  the  infections  have  been  held  responsible.  Certain 
metallic  poisons,  zinc,  copper,  manganese,  give  rise  to  clinical  pictures 
at  times  closely  resembling  multiple  sclerosis.  Trauma  has  been  held 
responsible;  so  also  have  sexual  excesses,  cold  and  wet  and  child-bearing. 

Heredity  may  be  an  important  factor,  according  to  many  (Pelizeus 
and  others),  and  Striimpell  contends  that  the  essential  feature  is  an 
abnormal  congenital  factor,  which  is  made  to  develop  by  any  one  of  the 
other  causes  here  enumerated.  Miiller's  critical  summary  would  seem 
to  exclude  practically  all  the  exogenous  causes  since  they  occur  in  but 
a  very  small  proportion  of  his  cases.  He  allows  that  a  secondary 
multiple  sclerosis,  in  the  sense  already  outlined,  may  possibly  follow 
infections,  but  that  multiple  sclerosis,  in  the  narrow  sense,  develops 
only  on  the  basis  of  a  congenital  predisposition. 

Sex  plays  practically  no  part.  Both  the  results  of  Charcot's  and 
WTiitoff's  studies,  which  spoke  for  greater  frequency  in  female  and 
male  material  respectively  are  due  to  their  particular  clientele.  Poly- 
clinic statistics  nearly  always  show  a  greater  frequency  of  women, 
because  they  are  freer  to  visit  dispensaries,  and  usually  go  to  all  of 
them  in  a  big  city.  The  only  reliable  statistics  are  those  of  ]\Iuller, 
which  were  controlled  by  autops}'.   They  show  practically  no  difference. 

v4gre.— Three-fourths  of  the  carefully  observed  cases  occur  between 
the  years  of  twenty  and  forty.  In  our  o^\^l  statistics^  two-thirds  of 
the  patients  were  under  forty.  Cases  have  been  reported  in  children 
as  young  as  five  months,  and  in  adults  as  old  as  seventy-five  to  eighty 
j'ears,  but  for  both  extremes  diagnostic  mistakes  are  not  ruled  out. 
Marie's  view  that  multiple  sclerosis  is  common  in  children  has  not 
withstood  the  severe  critique  of  autopsy  material,  and  Miiller  takes 
the  stand  that  the  disease  is  found  extremely  seldom  in  very  early 
youth.  The  initial  stages,  however,  may  be  traced  very  often  to  the 
years  of  adolescence  or  early  adult  life.  Occupation  plays  no  role 
that  is  yet  kno\\Ti. 

The  possibility  of  its  being  a  definite  infection,  possibly  of  a  proto- 
zoan nature,  is  coming  to  the  fore  on  the  basis  of  the  histology  of  the 
lesion  and  serum  reactions.- 

Symptoms. — The  accidental  features  of  the  distribution  of  the 
sclerotic  patches  hi  this  disorder  make  it  possible  for  almost  any 
combination  of  neurological  signs,  and  the  greater  the  number  of  cases 
studied  the  richer  has  become  the  symptomatology.  Certain  patients 
show  comparatively  few  symptoms  for  years;  others  show  additions 
almost  from  month  to  month,  until  they  become  veritable  neurological 
museums,  with  signs  of  involvement  from  the  frontal  poles  to  the 

'  .IcllifTe:     Jour,  of  Ncrv.  and  Ment.  Dis.,  1904,  p.  446. 

2  Kuhn  V.  Steiner,  Med.  Klin.,  1917,  No.  3,  and  Sienierling,  Bcrl.  klin.  Wchnschr., 
1918,  No.  12,  claimed  to  have  found  spirochetes  in  the  multiple  sclerosis  nodules.  Other 
observers  are  not  prepared  to  accept  these  findings.  Striimpell,  Neurol.  Centralbl.,  1918, 
among  them.     See  Abst.,  Jour.  Nerv.  and  Ment.  Dis.,  Sept.,  1919. 


510  LESIONS  OF  THE  SPINAL  CORD 

tip  of  the  Cauda  equina.  No  two  patients  are  alike,  yet  most  seem  to 
attain  the  same  level  in  the  end  and  almost  come  to  present  a  stereo- 
tjTDed  picture. 

In  this  picture  the  most  striking  feature  is  the  involvement  of  the 
motor  system,  giving  rise  to  muscular  weakness,  hypertonus  and 
general  spastic  phenomena,  both  in  the  cranial  and  spinal  paths. 
The  extreme  range  is  the  feature  that  characterizes  multiple  sclerosis 
in  its  fully  developed  form.  One  finds  isolated  or  group  phenomena 
all  over  the  body  just  in  the  same  proportion  as  the  accidental  dis- 
tribution of  the  sclerotic  patches  is  isolated  or  diffuse.  For  this  reason 
it  has  been  thought  advisable  to  begin  the  description  of  the  symptoms 
with  those  of  the  cranial  nerves,  and  proceed  systematically  through- 
out the  nervous  system.  The  older  and  classical  triad  of  Charcot  is 
only  partially  true,  and  today  a  multiple  sclerosis  may  be  diagnosed 
from  a  temporal  pallor  of  the  optic  disks  combined  with  bladder  incon- 
tinence in  the  absence  of  nystagmus,  intention  tremor,  or  scanning 
speech. 

The  classical  picture  of  Charcot  may  be  found  in  not  more  than 
15  per  cent,  of  all  the  cases,  at  a  period  when  the  presence  of  other 
symptoms  permits  one  to  make  a  diagnosis  of  multiple  sclerosis.  If 
one  waits  for  the  "classical"  picture,  one  may  have  to  wait  for  years. 
Some  patients  with  true  multiple  sclerosis  never  develop  the  Charcot 
"triad." 

Olfactory. — Hallucinations  of  smell  are  occasionally  found  but  belong 
to  the  rarer  and  infrequently  recorded  psychical  signs.  Bilateral 
anosmia  has  been  recorded. 

Optic. — The  researches  of  Uhthoff  have  emphasized  the  frequent 
occurrence  of  changes  in  the  optic  disks.  About  50  per  cent,  of  the 
patients  show  changes  in  the  papillae,  and  it  is  striking  that  these 
changes  occur  early.  They  are  in  the  nature  of  partial  atrophies, 
and  show  themselves  for  the  most  part  as  a  simple  atrophic  pallor 
of  the  disk — usually  most  marked  temporally.  True  optic  atrophy 
is  found  in  some  of  the  cases,  and  again  in  still  fewer  a  true  papillo- 
edema  or  choked  disk.  This  usually  recedes  and  either  leaves  no 
sign  or  an  atrophy.    A  great  number  of  variations  are  known. 

The  sight  is  frequently  affected,  sometimes  blindness,  partial  or 
complete,  unilateral  or  bilateral,  is  an  initial  symptom.  This  not 
infrequently  clears  up.  Complete  blindness  with  double-sided  atrophy 
is  extremely  rare  (Gnanck-Uhthoff ) .  Parinaud  has  made  three  class- 
ical tjTpes,  but  later  studies  have  shown  that  there  is  no  uniformity, 
and  the  variations  in  sector  blindness  and  dimness  are  very  many. 
The  loss  or  diminution  in  sight  in  multiple  sclerosis  is  characterized 
by  its  advancing  and  receding  character,  and  by  the  fact  that,  as  a  rule, 
the  ophthalmoscopic  picture  is  usually  more  pronounced  in  severity 
than  the  s^^mptoms,  and  offers  no  satisfactory  register  of  the  variability 
in  visual  acuity. 

The  fields  of  vision  more  often  show  central  scotomata  with  periph- 
eral clearness  than  peripheral  scotomata.  Unilateral  scotomata,  quad- 


Left  eye  vision  20/30 


Left  eye  vision  15 '40 


Left  eye  vision  15/30 


Fig.  301 


Fig.  302 


Fig  303 


Right  eye  vision  20/30 


Right  eye  vision  15/40 


Right  eye  vision  15/50 


Left  eye  vision  20/40  Right  eye  vision  20/30 

Field  of  vision White  -.-.-.-.  Blue  Green  for  5  mm.  □ 

Relative  scotoma  ^ 

Figs.  300  to  303. — Irregular  scotomata  picture.s  seen  in  multiple  sclerotic  patients. 

(Klingmann.') 


'  Jour.  Nerv.  and  Mental  Disease,  1910, 


512  LESIONS  OF   THE  SPINAL  CORD 

rant  scotomata,  and  a  great  variety  of  other  forms  are  known  as  well 
as  a  certain  amount  of  variability  from  time  to  time  in  the  individual 
ease.  The  color  fields  show  similar  variations  and  variabilities.  Optic 
hallucinations  also  occur.     (See  Figs.  300  to  303.) 

Ocular  Symptoms. — ^The  characteristic  phenomenon  here  is  nystag- 
mus. Its  occurrence  as  a  symptom  of  "brain  sclerosis"  was  first 
pointed  out  by  Valentiner  (1856)  and  Charcot  overvalued  its  diagnostic 
importance.  True  central  nystagmus  is  rare,  but  nystagmoid  move- 
ments on  lateral  motion  of  the  eye  occurs  in  from  70  to  80  per  cent,  of 
the  cases,  especially  in  the  later  stages  of  the  disease.  Vertical, 
oblique,  rotary  nystagmus  is  included  with  the  other  forms.  In 
cases  with  pronounced  cerebellar,  involvement  the  nystagmus  is  of 
this  type,  and  rotation  of  objects  with  subjective  rotation  and  forced 
positions  are  to  be  expected.  These  eye  movements  may  be  sum- 
marized as:  (1)  continued  rhythmical  oscillations  (true  central  nys- 
tagmus) analogous  to  the  continuous  movements  of  the  head  and 
body;  (2)  rhythmic  oscillations  set  up  on  movement  of  the  bulb  in  any 
direction,  analogous  to  the  intention  tremor  of  the  hands;  (3)  nystag- 
moid movements  on  extreme  lateral  or  vertical  movements,  analogous 
to  fatigue  (paresis)  movements;  (4)  ataxic  movements.  Of  these 
(3)  is  the  most  frequent;  practically  all  are  conditioned  by  central 
disease,  although  peripheral  involvements  are  described. 

Eye  Muscle  Palsies. — ^These  are  important  in  diagnosis,  and  present 
themselves  infrequently  as  isolated  or  complete  palsies,  transitory 
or  persisting.  Paralysis  of  convergence  is  more  often  found.  In 
general,  from  17  to  46  per  cent,  of  the  patients  show  palsies,  the 
statistics  varying  according  to  the  more  or  less  strict  interpretation  of 
paralysis.  Uhthoff  demands  "double  vision"  as  a  criterion,  which 
symptom  is  not  an  infrequent  early  occurrence  in  multiple  sclerosis. 
Miiller,  on  the  other  hand,  admits  milder  palsies  to  his  statistics  (46 
per  cent.) 

Ptosis  is  less  frequently  found  (8  to  15  per  cent.),  usually  one-sided, 
occasionally  double,  usually  incomplete  and  ephemeral.  One  also 
meets  with  external  ophthalmoplegia,  abducens  and  trochlearis 
palsies.  Divergent  palsy  has  been  noted  by  Bielschowsky.  Complete 
internal  ophthalmoplegia  has  not  yet  been  recorded. 

Pupillary  inequalities  are  not  infrequent  (24  per  cent. — ^Miiller), 
and  these  are  changeable  and  frequently  early.  They  may  represent 
spasms  or  paresis  of  muscles.  Miosis  is  frequent  in  the  later  stage 
(Parinaud),  but  the  reflexes  are  usually  normal.  Pupillary  unrest 
(hippus)  is  found  (Frankl-Hochwart) .  Argyll-Robertson  pupil  was 
found  only  four  times  in  364  cases.  One  case  of  Uhthoff  was  con- 
trolled by  autopsy;  2  cases  by  Rad,^  Wassermann  control. 

V.  Trigeminus. — ^Facial  neuralgia  has  been  observed  as  the  first 
symptom  of  a  multiple  sclerosis  (Oppenheim),  but  mvolvement  of 
both  the  sensory  and  the  motor  branches  is  rare. 

1  Neurol.  Centralbl.,  1911,  xxx,  584. 


MULTIPLE  SCLEROSIS  513 

VII.  Facial. — Facial  palsies,  usually  associated  with  other  central 
(hemiplegic)  pontine  (alternating  palsy),  or  pressure  symptoms,  occa- 
sionally coming  on  very  acutely,  are  not  infrequent  (15  per  cent.) ;  as 
an  isolated  palsy  it  is  rare.  Like  other  palsies  their  fluctuating  char- 
acter is  striking,  the  severe  permanent  types  being  rare  (Bouchaud), 
as  are  also  bilateral  palsies. 

VIII. — Hearing  is  rarely  affected.  Complete  or  partial  deafness 
has  been  recorded,  as  well  as  the  occurrence  of  noises.  Auditory 
hallucinations  are  possible,  but  not  anatomically  proved.  Hyper- 
esthesise  are  frequent. 

The  vestibular  nerve  of  the  eighth  pair  is  not  infrequently 
involved  in  its  cerebellar  connections.  An  explanation  of  some  of  the 
more  severe  nystagmus  movements  may  be  found  here.  Giddiness 
and  dizziness  are  very  prominent  symptoms,  and  occur  either  from 
involvement  of  this  nerve  or  other  sensory  cerebral  or  spinal  tracts. 
Rotatory  giddiness  with  tendency  to  fall,  revolving  of  objects,  etc.,  is 
present  in  a  few  cases  of  multiple  sclerosis,  and  certain  Meniere  cases 
belong  here. 

X. — Pneumogastric  involvement  is  rare.  Miiller  reports  a  case  of 
paroxysmal  tachycardia  in  multiple  sclerosis.  Dyspnea  is  also 
known,  but  is  infrequent. 

The  taste  has  been  modified  in  a  few  cases. 

Speech  Disturbances. — Charcot's  t}T)ical  speech  disturbance  was  of 
a  slow,  monotonous,  and  scanning  character.  This  special  tj'pe  is 
present,  however,  in  only  a  small  proportion  of  the  cases,  and  then 
usually  only  in  the  later  stages.  Miiller 's  autopsy-controlled  material 
(81  cases)  gave  only  25  per  cent,  of  this  character,  whereas  patients 
with  other  types  of  speech  disturbance  run  higher — 25  to  30  per  cent. 
— making  about  half  of  all  multiple  sclerosis  cases  with  some  disorder 
of  speech.  Easy  fatigability,  with  increasing  unsteadiness;  stuttering, 
with  accentuated  mouth  movements,  are  other  tj^pes. 

Singers  soon  notice  these  alterations,  especially  the  fatigability, 
and  an  increasing  inability  to  modulate  the  tones.  Dysarthria  and 
explosive  speech  are  obtained  in  later  stages.  Articulatory  distiu"b- 
ances  with  repetition  of  syllables,  or  words,  is  frequent  in  later  stages 
■ — r,  I,  y  and  g  are  particularly  difficult.  Aphonias  or  other  laryngeal 
complications  are  occasionally  met  with  (Rethi).  The  tongue  occa- 
sionally shows  mild  transitory  palsies,  with  mild  atrophies,  usually 
unilateral  and  fibrillary  twitchings.  Ataxic  movements  of  the  tongue 
are  frequent  in  the  later  stages.  Occasionally  subjective  sensations 
of  fulness,  thickness  and  difficulty  in  movement  are  observed. 

Chewing  and  swallowing  are  involved,  the  former  rarely,  the  latter 
not  infrequently,  less  often  transitorily  as  an  early  symptom,  more 
often  as  a  permanent  terminal  sign.  Excessive  salivation  seems  very 
infrequent. 

Motor    Disturbances. — The  most  characteristic  are  the  intention 
tremor,  ataxias,  and  palsies  with  later  developing  atrophies  and  con- 
tractures. 
33 


514  LESIONS  OF  THE  SPINAL  CORD 

Ataxia. — By  the  finger-nose  test  or  the  finger-finger  test,  and  by  the 
static  position  one  can  demonstrate  an  ataxia  in  the  upper  extremities, 
separate  from  an  intention  tremor,  in  a  large  number  of  the  cases 
(Miiller,  70  per  cent.).  A  similar  ataxia  may  be  shown  in  the  lower 
extremities  in  even  a  more  striking  manner  in  testing  by  the  knee-heel 
test.  The  ataxic  movements  usually  precede  the  development  of  the 
intention  tremor,  and  are  later  often  covered  up  by  the  same.  Occa- 
sionally the  ataxias  are  increased  on  closure  of  the  eyes,  occasionally 
not,  showing  that  at  least  two  types  are  to  be  observed.  Recent  find- 
ings in  the  sphere  of  deep  sensibility,  position,  sense,  etc.,  afford  an 
interpretation  of  these  ataxias.  They  vary  clinically  from  the  tabetic 
ataxia,  particularly  in  the  increased  tempo  of  the  individual  move- 
ments and  the  irregular  contraction  of  the  agonist  muscles.  The 
usual  hypertonus  of  the  multiple  sclerosis  patient  is  in  marked  contrast 
to  the  hypotonus  of  the  tabetic.     Crossed  hemiataxias  are  recorded. 

Intention  Tremor. — Present  in  from  50  to  75  per  cent,  of  the  cases, 
this  symptom  is  one  of  the  most  striking  in  well-advanced  multiple 
sclerosis.  It  may  be  absent  for  a  number  of  years,  and  yet  a  diagnosis 
may  be  made  on  other  grounds.  Considerable  care  must  be  exercised 
in  not  confusing  an  intention  tremor  with  an  ataxia,  and  vice  versa. 
Certain  shell-shock  tremors  observed  in  warfare  have  closely  resembled 
this  intention  tremor.  Miiller  has  laid  considerable  stress  upon  this 
point,  and  because  of  his  more  rigid  criteria  regarding  the  differences, 
states  that  real  intention  tremor  was  present  in  only  25  per  cent,  of  his 
cases.  This  intention  tremor  is  increased  by  prolonged  effort  and 
by  emotional  disturbances.  It  is  not  markedly  increased  by  shutting 
the  eyes. 

It  develops  gradually,  occasionally  suddenly  after  an  apoplectic 
attack,  is  more  often  bilateral,  corresponding  closely  with  the  pareses. 
The  arms  are  more  frequently  involved,  then  the  trunk,  and  then  the 
head ;  rarely  the  chin  and  muscles  of  the  face. 

Only  exceptionally  are  the  legs  markedly  aff,ected  by  an  intention 
tremor.  In  the  trunk  this  disturbance  gives  rise  to  a  type  of  con- 
tinual balancing  or  rocking  motion;  it  also  involves  the  muscles  sup- 
porting the  head,  causing  a  nodding  and  swaying  series  of  movements. 
Passive  tremors  of  the  hands  when  at  rest  are  also  observable. 

The  precise  anatomical  reasons  for  the  intention  tremor  are  not 
completely  analyzed.  Spinal  cases  do  not  show  it,  and  the  greatest 
probability  is  that  the  lesions  causing  the  intention  tremor  are  those 
that  cut  off'  portions  of  the  cerebello-rubro-thalamic  tracts.  A  com- 
plete analysis  of  the  fiber-tract  involvements  in  multiple  sclerosis  has 
yet  to  be  made  before  all  questions  relating  to  localization  can  be 
answered.  (See  discussions  of  athetoid  tremors,  thalamic  syndrome 
tremors,  cerebellar  tremors,  paralysis  agitans  tremors  in  their  respec- 
tive chapters.) 

Writing  shows  characteristic  changes  flue  to  both  the  intention 
tremor  and  the  ataxia.  The  strokes  are  irregular — too  thick  or  too 
thin — and  the  excursions  in  forming  the  letters  irregular. 


MULTIPLE  SCLEROSIS  515 

Motor  Power. — ^IVIotor  weakness  is  usually  the  earliest  symptom 
noted  in  multiple  sclerosis.  It  may  be  the  arms,  oftener  the  legs, 
or  occasionally  in  some  special  group  of  muscles;  larynx  in  singers, 
hand  and  arm  in  painters  or  players,  occasionally  bladder  or  rectum. 
Marked  fatigability  is  an  important  symptom,  and  may  precede  the 
other  symptoms  for  years. 

Spastic  paresis,  with  hypertonus,  then  develops,  and  though  vary- 
ing considerably  in  intensity,  often  dominates  the  picture,  leading 
later  to  the  most  severe  forms  of  contractures.  The  type  may  be 
hemiplegic  or  paraplegic,  and  usually  indicates  involvement  of  the 
pjramidal  tracts.  The  lower  extremities  are  involved  much  oftener 
than  the  upper.  Irregular  Brown-Sec^uard  paralysis  is  occasionally 
present.  Oppenheim  has  recorded  a  bilateral  Brown-Sequard  syn- 
drome. 

Gait  disturbances  are  common  and  variable.  They  may  be  purely 
spastic,  or  in  the  cerebellar  cases,  spastic  ataxic.  Pure  ataxic  gaits 
are  very  rare.  A  spastic  paretic  gait  marks  the  pre-bed  stage.  Occa- 
sionally one  finds  the  tumbling  or  reeling  gait  of  cerebellar  tract 
implication. 

Certain  motor  anomalies  are  encountered  as  the  patients  rise  or 
sit  down.  A  Romberg,  which  is  not  much  modified  on  closing  the 
eyes,  is  not  uncommon. 

Muscular  Atrophies. — ^These  occur,  but  are  rare. 

Sensory  Phenomena. — Marie's  earlier  contention  that  sensory  dis- 
turbances do  not  belong  to  multiple  sclerosis  has  been  definitely 
dispro^'ed.^  On  the  contrary,  they  are  very  frequently  found,  but 
largely  by  reason  of  their  fluctuating  character,  both  as  to  intensity 
and  extent  are  overlooked  or  falsely  interpreted.  The  frecj[uently 
made  false  diagnosis,  "hysteria,"  is  usually  founded  on  these  fluctu- 
ating, sensory  signs.  Hofl'man  and  Freund  found  sensory  disturbances 
in  from  70  to  90  per  cent,  of  their  cases.  Miiller  found  them  in  76 
per  cent.  Pains  are  not  frequent;  paresthesise,  on  the  other  hand,  are 
very  common.  Anesthetic  or  hyperesthetic  spots  are  frequent;  the 
patients  frequently  complain  of  dead  or  numb  fingers.  Tactile  anes- 
thesia is  not  infrequent,  occasional  tactile  agnosia  is  present.  The 
sense  of  localization  is  frequently  faulty,  and  bony  sensibility  is  also 
often  involved,  with  diminution  of  position  sense  and  disturbance  of 
thermal  sensibilities.     Thermal  changes  are  also  observed. 

Skin  Reflexes. — The  abdominal  and  epigastric  reflexes  are  almost 
invariably  modified  (80  per  cent.).  There  may  be  unilateral  diminu- 
tion or  loss,  or  more  often  bilateral  diminution  or  loss  (62  per  cent.). 
Thus  bilateral  loss  of  these  reflexes  constitutes  a  very  important  dift'er- 
ential  for  the  diagnosis  of  not  only  an  organic  nervous  disease,  but 
especially  for  multiple  sclerosis.  Careful  studies  by  Miiller  have 
shown  that  when  correctly  tested  they  are  modified  in  only  about  5 
per  cent,  of  normal  individuals,  and  these  occurred  most  often  in 

'  Jeremias  has  collected  the  rich  literature  bearing  on  sensory  disturbances  in  nuiltiple 
sclerosis.     Dissert.,  1898;  see  also  Miiller. 


516  LESIONS  OF  THE  SPINAL  CORD 

women  who  had  had  children,  or  in  very  obese  subjects.  The  cause 
is  to  be  sought  in  an  interruption  of  the  sensory  tracts,  either  in  the 
cord,  in  the  median  lemniscus,  in'  the  thalamic  end-stations,  or  in  the 
post-Rolandic  sensory  distributions.  The  multiplicity  of  the  sclerotic 
patches  makes  it  likely  that  these  reflex  paths  will  be  implicated 
somewhere  in  their  march  to  the  cortex. 

The  cremasteric  reflex  is  also  often  involved  unilaterally,  or  bilater- 
ally, but  less  often.     Also  the  vomiting  reflex,  though  not  so  often. 

Babinski's  phenomenon  is  very  frequent;  Miiller  says,  almost  con- 
stant. Crossed  Babinski  may  even  be  observed.  Oppenheim  reflex 
is  very  often,  though  less  frequently,  found. 

Like  all  of  the  symptoms  of  multiple  sclerosis,  the  skin  reflexes 
are  subject  to  considerable  variations.  They  infrequently  return  after 
showing  a  loss. 

Tendon  Reflexes.— In  concordance  with  the  spasticity  and  hyper- 
tonus,  increased  tendon  reflexes  are  to  be  expected.  The  upper  ex- 
tremities show  increased  triceps-jerk  and  increased  radioperiosteal 
reflexes.  The  Achilles-jerk  is  exaggerated,  unilaterally  or  bilaterally, 
as  also  the  knee-jerk,  and  usually  there  is  unilateral  or  bilateral  ankle- 
clonus.     Patellar  clonus  is  less  often  observed. 

Vasomotor  and  Trophic  Signs;  Other  Signs. — One  finds  a  number 
of  anomalies  of  this  group  in  a  large  collection  of  multiple  sclerosis 
cases,  but,  as  a  rule,  they  are  sparse  and  isolated.  Among  these  may 
be  mentioned  hyperidrosis,  angiospasms  (cyanosis),  vessel  palsies  with 
edema,  erythromelalgia-like  affections,  syringomyelia-like  trophic  dis- 
turbances, abnormal  blushing.  Hyperpyrexias  are  encountered  with 
apoplectiform  attacks.  In  the  ordinary  cases  the  patients  show 
marked  lability  of  the  body  temperature. 

Pilomotor  reflexes  are  frequently  disturbed;  thus  dermographia  is 
common,  and  may  show  unique  isolated  localizations. 

Bladder. — The  bladder  is  frequently  involved  (80  per  cent. — Oppen- 
heim; 75  per  cent. — Miiller),  often  transitorily,  and  also  it  may  be  an 
early  sjmptom.  The  patients  feel  a  sense  of  insufficiency  and  have 
to  strain  to  pass  their  water.  Complete  paresis  with  incontinence  is 
rare.  There  is  a  great  variability,  with  many  ups  and  downs  in  the 
bladder  symptoms.     Polyuria  and  glycosuria  have  been  observed. 

Obstipation  is  frequent;  loss  of  control  less  so,  but  is  present. 

Menstruation,  childbirth,  parturition,  etc.,  are  not  markedly 
affected.  Genital  hyperesthesia  is  at  times  pathologically  increased 
and  occasionally  there  is  anesthesia  with  impotence. 

Lumbar  Puncture. — ^The  experience  thus  far  gained  shows  no  appre- 
ciable changes  in  pressure.  Cytologically,  occasionally  slight  lympho- 
cytosis has  been  observed,  but  the  majority  of  the  cases  have  been 
negative.  Serologically,  some  positive  Wassermann  reactions  have 
been  recorded.^ 

1  Nonue:     Deutsch,  Ztschr.  f.  Nervenh.,  1910,  1912. 


MULTIPLE  SCLEROSIS  517 

Menial  Symptoms. — ^These  are  not  constant  nor  prominent,  and 
are  for  the  most  part  absent,  yet  careful  analysis  shows  them  to  be 
much  more  freciuent  than  is  usually  taught.  INIild  intellectual  reduc- 
tion in  the  form  of  a  dementia,  hallucinations  of  hearing,  of  sight, ^ 
hallucinatory  states  with  mild  confusion,  passing  ideas  of  reference  and 
of  persecution,  difficulty  in  thinking,  spasmodic  intermittent  alterna- 
tions in  the  capacity  for  attention  and  concentration,  lapses  of  memory, 
etc. ;  these  have  all  been  recorded.  The  latter  anomalies  are  not  infre- 
quent, and  often  are  colored  by  depression  (melancholic)  or  excited 
(manic)  states,  or  more  frequently  the  emotional  tone  is  one  of  indif- 
ference.    Pseudoparetic  states  may  be  expected  at  times. 

Involuntary  Laughing  and  Crying. — ^These  were  described  by  Cru- 
veilhier  in  his  classical  case,  and  correctly  appreciated  by  Bourneville. 
They  are  purely  neurological  signs,  forms  of  paramimia,  for  neither 
the  laughing  nor  the  crying  are  necessarily  accompanied  by  their 
usual  emotional  states,  nor  do  they  necessarily  betoken  any  intellectual 
reduction.  They  are  very  often  found,  forced  laughing  being  the 
more  frequent  (40  per  cent. — Miiller).  They  are  both  to  be  referred 
to  implication  (in  part)  of  the  corticobulbocerebellar  reflex  paths.  An 
analysis  of  all  of  the  variations  is  not  possible  here. 

Apoplectiform  and  Epileptiform  Atidcks. — These  occur  but  not  as 
frequently  as  was  held  before  one  was  in  a  position  to  rule  out  anoma- 
lous paretic  attacks.  But  the  frequent  occurrence  (20  to  25  per  cent.) 
of  mild  attacks  with  transitory  disturbances  of  consciousness,  or 
attacks  of  giddiness  or  faintness,  with  unilateral  or  bilateral  pareses, 
or  sensory  anomalies  in  the  distal  extremities,  should  be  emphasized. 
Such  mild  attacks  resembling  arteriosclerotic  attacks,  often  mark  the 
initial  stages,  or  accompanying  the  sudden  progressions  which  are 
characteristic  of  this  disorder.  Epileptiform  attacks  are  much  less 
often  observed  and  usually  speak  against  a  multiple  sclerosis,  but 
they  do  occur,  and  very  frequently  show  as  Jacksonian  attacks 
(Gussenbauer). 

Characteristic  Forms. — As  already  noted,  the  classical  signs  of 
Charcot,  with  nystagmus,  intention  tremor  and  scanning  speech,  in 
reality  are  found  in  only  a  comparatively  small  proportion  of  the 
cases  in  the  earlier  stages  of  the  disease.  In  the  interest  of  early 
diagnosis,  this  must  be  borne  in  mind,  especially  as  true  nystagmus 
is  always  rare,  nystagmoid  movements  are  to  be  judged  with  care, 
and  the  tremor  and  scanning  speech  are  usually  later  symptoms. 
The  "non-typical"  cases  in  Charcot's  sense  (formes  fruste),  are  really 
more  frequently  met  with.  Any  attempt  to  state  which  special 
group  of  symptoms  occurs  with  more  or  less  frequency  is  apt  to  be 
misleading,  especially  in  view  of  the  great  variability  met  with  and 
the  inconstant  changes,  the  advance  of  certain  symptoms  and  the 
retrogression  of  others. 

1  Nonne:     Mitt.  Hamb.  Staatskrankenhaus,  1910. 


518  LESIONS  OF  THE  SPINAL  CORD 

For  purely  didactic  purposes  oue  can  divide  the  more  common 
symptom  pictures  as  follows: 

1.  Cases  that  begin  with  isolated  or  prominent  cerebral  symptoms. 
These  occur  less  frequently  than  the  spinal  cases,  perhaps  than  the 
bulbar,  but  hence  are  also  more  likely  to  be  overlooked.  Here  optic 
atrophies,  with  defects  of  vision,  ocular  palsies,  transitory  diplopias, 
crossed  eyes,  etc.,  muscular  weaknesses  are  prominent  and  occasion- 
ally associated  with  giddiness,  nausea  and  headache. 

2.  Cases  with  isolated  or  prominent  bulbar  symptoms.  These 
begin  as  mild  or  severe  bulbar  palsies,  and  are  rare. 

3.  Cases  with  irregular  and  prominent  spinal  s^onptoms,  mostly 
involving  the  lower  extremities.  Here  the  patients  complain  of  the 
legs  becoming  tired  easily,  and  after  a  time  they  stumble  or  trip 
easily.  They  then  become  somewhat  stiff  and  walking  up  stairs 
becomes  increasingly  difficult.  Mild  bladder  disturbances  may  have 
preceded  or  accompany  the  weakness  and  stiffness  in  the  legs.  Par- 
esthesia are  also  frequent.  These  patients  soon  show  spasticity, 
increased  knee-jerks,  clonus  at  times,  Babinski's  sign.  The  abdominal 
and  epigastric  reflexes  are  diminished  or  absent  unilaterally  or 
bilaterally. 

This  is  apparently  the  most  frequent  mode  of  onset,  since  very  often 
the  patients  have  paid  no  attention  to  the  rapid  passing  of  transitory 
diplopias,  slight  speech  difficulties  or  weakness  of  the  bladder,  or  mild 
attacks  of  giddiness,  yet  sharp  questioning  usually  elicits  some  of 
these  other  signs  as  having  preceded  or  accompanied  these  spastic 
paraplegic  types.  Whereas  these  signs  usually  come  on  very  gradually, 
they  may  appear  to  have  had  a  sudden  onset,  as  after  a  long  walk,  or 
slight  accident,  or  following  childbirth,  or  other  striking  intercurrent 
event. 

4.  In  the  vast  majority  of  the  cases  there  is  a  gradual  onset  of  both 
spinal  and  cerebral  symptoms.  Headaches,  diplopia,  difficulty  in 
walking,  slight  changes  in  speech,  giddiness,  abnormal  muscular  tire, 
paresthesise,  transitory  bladder  weakness,  etc.  These  s^onptoms 
progress  and  then  recede — usually  attributed  to  the  skill  of  the  phy- 
sician or  thought  of  as  hysterical — and  then  reappear  in  the  same  or 
in  an  entirely  different  order. 

5.  A  small  number  of  cases  begin  with  a  mild  initial  apoplectiform 
attack,  and  then  either  progress,  or  remain  stationary  for  some  years. 

6.  Marburg^  has  described  an  acute  disseminated  sclerosis  which 
runs  a  rapidly  fatal  course  terminating  in  from  three  to  six  months. 

Non-characteristic  Forms. — These  unusual  forms  may  be  summarized 
as  (a)  forms  which  run  a  more  distinctly  psychic  course,  with  the 
picture  of  slowly  advancing  dementia  and  pseudoparetic  forms; 
(b)  forms  that  resemble  brain  tumor — hydrocephalus;  (c)  Jacksonian 
attacks  due  to  patches  in  the  motor  area;  (d)  hemiplegic  forms  due 
to  patches  in  the  cerebral  course  of  the  pyramidal  tracts;  (e)  bulbar 

1  Loc.  cit. 


MULTIPLE  SCLEROSIS  519 

paralysis  and  pseudobulbar  paralysis  types;  (/)  sacral  forms  as  described 
by  Oppenheim,  resembling  tumor  of  the  cauda  equina;  (g)  forms  that 
give  an  acute  onset  resembling  pontine  encephalitis;  (/?)  cerebellar 
types,  resembling  paralysis  agitans  (not  infrequently  misnamed  early 
paralysis  agitans);  (y')  forms  that  give  a  picture  like  cerebrospinal 
syphilis;  (j)  progressive  muscular  atrophy  and  amyotrophic  lateral 
sclerosis-like  forms;  (A*)  tabetic-like  forms;  (/)  transverse  myelitis  and 
combined  sclerosis  types,  and  finally  (m)  latent  and  recessive  forms 
which  are  differentiated  from  irregular  psychogenic  edemas  of  the  cord, 
or  from  pure  psychological  blockings  only  after  many  years  of  the  most 
exact  scrutiny. 

Diagnosis. — Enough  has  been  said  to  indicate  how  a  multiple 
sclerosis  may  appear  under  the  guise  of  a  number  of  organic  diseases  of 
the  brain  and  spinal  cord.  The  most  important  features  in  the  diag- 
nosis concern  the  age  of  the  patients,  young  individuals,  the  usual  lack 
of  heredity,  the  failure  of  usual  external  causes  to  account  for  the 
symptoms,  absence  of  Wassermann  reactions  and  of  spinal  fluid  find- 
ings. Of  the  more  important  objective  findings  for  the  early  diagnosis 
one  counts  on  the  early  feeling  of  motor  weakness,  feelings  of  giddiness, 
the  onset  of  spasticity  with  increased  reflexes,  clonus  and  Babinski, 
the  presence  of  the  fundus  changes,  the  loss  of  the  abdominal  reflexes, 
fine  ataxic  movements  of  the  legs  in  the  knee-heel  test,  and  of  the  arms 
in  the  finger-finger  and  finger-nose  tests. 

In  the  later  stages  the  full  pictures  as  already  'outlined  appear 
and  make  a  diagnosis  certain.  One  feature  of  perhaps  the  most 
striking  importance  is  the  peculiar  advancing  and  receding  course, 
the  remissions  during  which  the  entire  picture  seems  to  fade  away 
and  which  permit  a  patient  condemned  to  a  chronic  organic  nervous 
lesion  to  turn  up  in  the  physician's  consulting-room  apparently  well. 

It  is  for  this  latter  reason  perhaps  more  than  any  other  that  the 
false  diagnosis,  hysteria,  is  so  frequently  made  in  these  cases.  Then 
there  are  in  addition  the  almost  daily  fluctuations  in  the  sensory  sphere 
which  always  suggest  hysteria,  and  which  only  a  rigid  analysis  will 
exclude.  Furthermore,  it  must  not  be  forgotten  that  not  only  does 
one  experience  the  changeability  in  symptomatology  suggestive  of 
hysteria,  but  a  helpful  optimistic  psychotherapy  can  most  markedly 
alter  the  s\Tnptoms  as  well,  causing  some  of  them  to  entirely  disappear. 
The  rise  in  the  level  of  nervous  energy  by  such  psychic  means  seems  to 
enable  the  patient  to  force  a  better  control  of  the  disturbances.  In 
the  failure  of  a  typical  hysterical  character,  as  fleterminined  by 
a  psychoanalytic  study  of  the  unconscious  svinbolizations  of  the 
symptoms  and  their  teleological  function  for  the  individual,  this 
disorder  should  not  be  diagnosed.  Finally,  one  has  always  to  reckon 
with  a  symptomatic  psychological  reduction  in  the  patient's  resistance 
due  to  the  presence  of  an  organic  lesion. 

Further,  dift'use  sclerosis,  general  paresis,  brain  tumor,  chronic 
h}'droce])halus,  arteriosclerosis,  cerebral  syphilis,  infantile  palsies, 
encephalitis  of  a  diftuse  disseminated  type,  bulbar   palsies,  chronic 


520  LESIONS  OF  THE  SPINAL  CORD 

leptomeningitis,  infantile  ataxias,  Friedreich's  ataxia  in  particular, 
disseminated  myelitis,  meningomyelitis,  Erb's  spastic  palsy,  heredi- 
tary spastic  palsies,  syringomyelia,  tabes,  spinal  cord  tumor,  paralysis 
agitans,  chronic  zinc  poisoning,  chronic  manganese  poisoning,  chronic 
mercurial  poisoning  and  spinal  cord  edemas  all  come  in  review  in  the 
differential  diagnosis  of  multiple  sclerosis. 

Pathology  and  Pathogenesis. — On  autopsy  in  the  cases  of  true  multiple 
sclerosis  one  finds,  as  a  rule,  a  normal  dura,  the  brain  itself  usually 
shows  irregular  atrophies  with  thinning  of  the  cortex  and  some  internal 
hydrocephalus.  iVt  times  one  can  observe  on  the  surface  the  irregular 
patch-like  areas  of  atrophy.  These  usually  show  much  more  fre- 
quently on  the  surface  of  the  pons,  medulla  and  cord,  as  Carswell  and 
Cruveilhier  showed  early.  On  section  of  the  brain  one  finds  few  (five 
or  six)  or  many  (several  hundred)  irregular  sclerotic  patches,  which 
are  usually  isolated  one  from  another. 

^Microscopic  examination  invariably  reveals  many  not  seen  by  the 
naked  eye.  They  vary  in  size  from  that  of  a  pin-head  to  5  or  6  cm., 
which  larger  patches  are  usually  made  up  of  several  smaller  ones. 
The  general  color  tone  is  gray.  The  reddish  patches  usually  belong  to 
the  secondary  encephalomyelitides.  The  plaques  either  rise  slightly 
from  the  surface  or  are  level  or  show  a  slight  depression;  the  tissues 
about  are  slightly  edematous.  At  times,  particularly  in  the  optic 
nerves,  there  is  distinct  shrinkage  in  the  tissues.  The  consistency 
is  usually  tough,  or  almost  leathery.  The  soft  plaques  are  apt  to 
resemble  disseminated  myelitis,  encephalomyelitis,  etc.  The  patches 
are  very  sharply  differentiated  from  the  surrounding  tissues.  As 
to  localization,  they  may  be  anj^vvhere;  in  the  brain,  cord,  medulla, 
pons,  cerebellum,  the  roots  of  the  cranial  or  spinal  nerves,  within  the 
optic  nerve  itself.  As  a  rule  they  are  found  in  both  brain  and  spinal 
cord,  and  seem  to  have  a  special  fondness  for  places  particularly  rich 
in  neuroglia,  and  there  is  a  certain  grade  of  asymmetrical  symmetry 
in  their  localization. 

In  the  cord,  the  median  line,  and  the  pyramidal  tract  region  are 
favorite  localizations,  and  in  the  white  matter  more  than  the  gray. 
Central  gliosis  seems  rare.  In  the  medulla  the  olivary  region,  the 
floor  of  the  fourth  ventricle,  and  the  posterior  aspects  are  favorite 
sites,  while  in  the  cerebellum  the  dentate  nucleus  is  usually  most 
often  implicated. 

Histologically  the  patches  are  made  up  of  masses  of  glia  fibers. 
There  is  no  areolar  composition  and  the  glia  nuclei  do  not  appear 
prominent.  Small  holes  may  be  found  within  the  plaques,  around 
which  the  glia  fibers  cluster  as  about  a  bloodvessel.  Bielschowsky 
preparations  show  the  partially  intact  nerve  axones  passing  through 
the  glia  masses  in  much  reduced  numbers;  the  medullary  sheaths  are 
partly  modified  in  their  qualities.  Charcot  first  called  attention  to 
the  persisting  axis-cylinders.  The  immense  mass  of  glia  fibrils  is  an 
entirel}'  new  growth,  apparently  not  to  take  the  place  of  destroyed 
nervous  tissue,  but  a  pure  addition  product,  a  proliferative  inflamma- 


MULTIPLE  SCLEROSIS  521 

tion,  in  which  one  finds  the  almost  normal  elements  still  present,  for  a 
time  at  least.  Secondary  degeneration  outside  of  the  plaques  is  not 
usually  present.^ 

WTiat  relation  the  disease  has  to  the  vessels  is  still  far  from  clear, 
but  it  seems  certain  that  primary  disease  of  the  vessels  is  not  a  part  of 
true  multiple  sclerosis.  The  present  trend  is  to  view  the  process  as 
related  to  a  non-purulent  encephalomyelitis,  allied  to  a  poliomyelitis, 
rabies,  Borna's  disease,  etc.,  the  exciting  agent  being  some  specific 
organism  as  yet  not  isolated.     (Spielmeyer,  loc.  cit.;  Schol.^) 

Dawson's  monumental  study  seems  to  show  that  the  sequence  of 
the  pathological  processes  in  the  disease  is  somewhat  as  follows:  (1) 
A  commencing  degeneration  of  the  myelin  sheath  and  a  simultaneous 
reaction  of  the  glia  in  the  immediately  adjacent  perivascular  tissue; 
(2)  an  increasing  glia-cell  proliferation  and  a  commencing  fat-granule- 
cell  formation;  (3)  the  stage  of  so-called  "fat-granule-cell  myelitis;" 
(4)  a  commencing  glia-fibril  formation;  (5)  an  advancing  and  (6)  a 
complete  sclerosis.  Histological  study  has  given  overwhelming  evi- 
dence that  sclerotic  areas  in  the  disease  arise  on  the  basis  of  this 
evolution  through  a  stage  of  fat-granule-cell  formation,  and  Dawson 
concludes  that  the  underlying  process  is  a  subacute  disseminated 
encephalomyelitis  which  terminates  in  disseminated  areas  of  actual 
and  complete  sclerosis.  There  is  much  to  favor  the  view  that  true 
disseminated  sclerosis  is  due  to  a  specific  morbid  agent;  other  diffuse 
affections  of  the  central  nervous  system  (arteriosclerosis,  syphilis, 
acute  encephalomyelitic  processes)  may  give  rise  to  a  clinical  symptom- 
complex  very  like  that  of  disseminated  sclerosis;  they  differ,  however, 
in  the  characteristic  remissions  and  relapses.  According  to  Dawson, 
the  anatomical  expression  of  these  remissions  must  naturally  be  the 
gradual  clearing  up  of  the  cell  exudation  and  a  sclerosing  of  the  tissue 
with  a  retention  of  the  axis-cylinders.  There  is  no  adequate  evidence 
to  distinguish  between  a  microbic  and  a  toxic  agent.  It  is  possibly 
the  latter.  The  causal  agent  is  probably  of  the  nature  of  a  soluble 
toxin,  which  is  conveyed  to  the  nervous  tissues  probably  through  the 
blood  channels.  The  suggestion  made  is  that  the  restriction  is  in 
some  way  related  to  the  selective  action  of  the  toxm  in  certain  areas 
of  the  blood  supply,  or  that  unknown  factors  determine  an  irregularly 
distributed  paralytic  dilatation,  with  an  increased  infiltration  through 
the  vessel  walls.  Remissions  and  relapses  necessitate  the  assumption 
of  the  latent  presence  of  the  morbid  agent  in  the  body — either  the 
intermittent  evolution  of  a  toxin,  or  its  accumulation  from  deficient 
elimination. 

This  whole  picture  suggests  that  the  vegetative  control  of  the  spinal 
cord  metabolism  is  primarily  at  fault,  which  in  part  would  aid  in  the 
understanding  of  the  marked  influence  of  psychogenic  factors  in  this 
disease. 

The  differentiation  from  disseminated  myelitis,   encephalomyelitis 

•  See  Dawson's,  and  Klingman's  complete  histological  studies,  loc.  cit. 

'  Gesellsch.  f.  Naturf.,  Dresden,  February  24,   1914,  and  citations  already  made. 


522  LESIONS  OF  THE  SPINAL  CORD 

and  other  secondary  affections  which  may  give  rise  to  partial  multiple 
sclerosis  pictures  must  be  sought  in  special  works  on  pathology. 

Prognosis. — In  general  the  disorder  is  progressive,  yet  there  are 
many  stationary  cases,  and  some  few  that  apparently  recover;  these 
are  possibly  mistakes  in  diagnosis,  but  such  mistakes,  in  view  of  the 
great  number  of  similar  affections,  are  almost  impossible  to  avoid. 
Many  patients  extremely  ill  and  bed-ridden,  following  one  of  the  acute 
advances  of  the  disease,  recover  almost  completely;  but  usually  there 
is  another  acute  advance  and  then  others.  The  time  between  advances 
or  the  length  of  remissions  cannot  be  stated;  they  have  varied  from 
six  months  to  ten  years;  some  very  rare  observations  show  a  period 
of  twenty  years. 

A  few  patients  die  rapidly  of  the  disorder  (six  weeks  to  six  months) 
— acute  multiple  sclerosis  of  Marburg,  Frankl-Hochwart  and  others — 
but  the  majority  live  for  many  years,  and  die  usually  of  intercurrent 
disorders,  pyelitis,  tuberculosis,  pneumonia,  etc.  ^liiller's  average 
was  four  years;  Charcot's,  six  to  ten  years. 

Therapy. — Specific  therapy  is  not  yet  known.  Prophylaxis  also 
seems  difficult  to  grasp  since  no  definite  exciting  cause  is  known. 
Relative  prophylaxis  in  the  shape  of  advising  against  marriage  for 
young  affected  individuals,  or  against  child-bearing  in  the  married  is 
necessary.  Childbirth  would  seem  to  be  an  exciting  cause  for  an 
active  exacerbation. 

Rest  in  bed  is  more  than  desirable  in  acute  stages,  it  is  imperative. 
The  rest  must  be  absolute,  and  should  include  eye  rest.  Active 
remedial  treatment — massage,  hydrotherapy,  electrotherapy — are  all 
disadvantageous.  Warm  baths  for  short  periods  are  not  contra- 
indicated.  Definite  motor  quiet  and  sensory  quiet  as  well  is  desir- 
able. Counter-irritation  in  those  cases  which  show  sharp  myelitic 
symptoms  is  to  be  tried. 

Pharmacotherapy  has  not  yet  devised  any  specific  remedies.  Arsenic 
is  used  on  empirical  grounds  and  because  of  the  possible  relation  of 
the  toxin-producing  agent  to  a  spirochete  (positive  Wassermann  in 
some  cases,  non-syphilitic)  and  in  combination  with  quinine,  ergot, 
strychnine  and  iron  does  some  service. 

Mild  work  in  the  open  air,  gardening,  etc.,  is  advisable.  Psycho- 
therapy is  never  to  be  forgotten,  and  a  healthy  optimism  helps  these 
patients  enormously. 

Some  very  anomalous  and  extraordinarily  good  results  have  followed 
the  opening  of  the  spinal  canal  (Elsberg).^ 

'  Surgery  of  the  Spinal  Cord,  Saunders,  1916. 


CHAPTER  VII. 

LESIONS  AT  THE  LEVEL  OF  THE  MEDULLA,  PONS, 
BRAIN-STEM  OR  MIDBRAIN. 

Lesions  involving  the  medulla,  pons,  brain-stem  or  midbrain  do  not 
differ  essentially  in  their  kind  from  those  already  discussed  as  involv- 
ing the  spinal  cord.  Softening  and  hemorrhage,  however,  are  more 
frequent  from  lesions  of  the  vertebrals,  basilar  and  circle  of  Willis's 
vessels  and  their  branches.  As  has  been  pointed  out,  many  of  the 
syndromes,  such  as  for  instance  those  known  as  multiple  sclerosis,  as 
sjTingomyelia,  poliomyelitis,  s^-philis,  etc.,  may  involve  the  entire 
cerebrospinal  axis.  This  involvement  may  be  more  or  less  instan- 
taneous. Such  is  the  case  in  poliomyelitis,  which  in  one  and  the 
same  individual  may  within  three  or  four  days  develop  a  meningitis 
(cerebral),  a  choreo-athetoid  movement  (midbrain),  an  eye  or  other 
cranial  nerve  palsy  (medulla),  paralysis  of  some  of  the  muscles  of  the 
arm  (cervical  spinal  cord),  atrophic  changes  in  skin  and  bone  of  any 
extremity  (sympathetic  of  spinal  cord),  and  finally  a  palsy  of  one 
or  more  leg  muscles  (lumbar  cord).  On  the  other  hand,  in  the  case 
of  a  smngo-encephalomyelia,  a  multiple  sclerosis,  or  a  cerebrospinal 
syphilis,  the  complete  syndrome  may  need  ten  to  twenty  years  to 
reach  a  complete  development  from  brain  to  lumbar  cord. 

Furthermore,  certain  of  these  processes  may  limit  themselves  entirely 
to  one  group  of  structures.  Thus  a  pontine,  medullary,  midbrain  or 
fourth  ventricle  tumor,  a  midbrain  arteriosclerosis,  a  medullary  polio- 
myelitis, a  quadrigeminal  syphilis,  or  thalamic  arteriosclerotic  softening 
may  limit  the  syndrome  within  narrower  confines.  In  such  an  event 
the  topical  as  well  as  the  etiological  factors  become  paramount. 

The  present  chapter  intends  to  deal  with  some  of  the  more  important 
problems  chiefly  with  reference  to  localization.  These  localizing  signs 
are  extremely  intricate  and  proper  diagnosis  can  be  arrived  at  only 
through  a  complete  knowledge  of  the  anatomy  of  these  parts.  Such  an 
anatomical  knowledge  can  be  gained  only  by  study  of  serial  sections  of 
these  regions.  By  reason  of  the  compactness  of  the  structures  the 
many  complicated  connections,  and  the  as  yet  imperfectly  analyzed 
dynamics  of  the  mechanism  within  the  pons,  brain-stem  and  midbrain, 
the  study  of  disturbance  in  these  regions  is  particularly  fascinating 
and  fruitful.  Here  correlating  and  coordinating  dynamic  forces  are 
represented  at  a  maximum,  so  far  as  sensorimotor  functions  are  con- 
cerned. While  they  are  infinitely  less  complex  than  the  adjusting 
symbolic  dynamics  handled  by  the  cerebrum,  yet  they  are  sufficiently 
complex  to  make  this  field  of  sensorimotor  neurology  most  bewildering. 

(523) 


524      LESIONS  OF  THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 


The  anatomical  plotting  of  most  of  the  fiber  tracts  and  the  chief 
synapses  are  known.  They  are  given  for  the  most  part  in  various 
chapters  in  this  work  and  chiefly  figured  in  plates  VII,  X,  XI  and  in  the 
group  of  figures  given  in  the  pages  immediately  following.  All  of  the 
clinical  pictures  resulting  from  lesions  in  these  regions  cannot  possibly 
be  given  in  a  work  of  this  kind,  hence  only  those  most  frequently  met 
with  in  practice  are  taken  up,  while  a  tabular  summary  of  these  and  a 
number  of  others  is  given  at  the  end  of  this  chapter. 


Part  of  marginal  convolution;  superior 
and  middle  frontal  convolutions;  upper 
part  of  ascending  frontal  convolution. 


Corjms  ca/7os»»i;  gyrus fornicatus:  inner 
surface  of  first  frontal  convolution; 
upper  part  of  asc.  frontal  convolution. 


Third  front,  convolution;  outer 
part   of  orbital  surface  of 
frontal  lobe. 


Asc.  parietal  convolution;  lower 
part  of  sup. parietal  convolution. 


Supramarginal  gyrus;  first  temp, 
convolution :  part  of  second  temp, 
convolution;  angular  gyrus. 


Ext.  occip.  convolution; 
third  temp,  convolution. 


fost.  Inf.  Cerebellar.  K"-^'  '^^f'^  of  cerebellum.  \- 


FiG.  304. — Circle  of  Willis  and   branches,  with  indication  of  distributions.     Regions 
A,  B  most  frequent  sites  for  hemorrhages.     (Greenacre,  from  Fursac.) 

Medullary  Syndromes. — Lesions  of  the  medulla,  usually  depending 
upon  vascular  disorders  of  the  branches  of  the  vertebrals  and  basilar 
produce  marked  initial  symptoms  of  an  apoplectiform  nature  and 
definite  residual  signs  depending  upon  their  location. 

Among  the  initial  signs  are  the  usually  rapid  and  severe  onset  of 
coma  from  cutting  off  of  the  cerebral  pathways  (diaschisis).  Not 
infrequently  there  are  generalized  tonic  convulsive  movements  of  the 
extremities  and  often  of  the  facial  musculature  with  the  exception  of 


MEDULLARY  SYNDROMES 


525 


those  muscles  whose  nuclei  may  be  implicated.  Flaccidity  of  the 
lower  extremities  from  diaschisis  of  the  p^Tamidal  tracts  with  initial 
loss  of  joint  reflexes  which  later  are  increased.  Involvement  of  vege- 
tati^'e  pathways  may  cause  vasomotor,  respiratory,  pupillary  and 
gastro-intestinal  symptoms,  vomiting,  diarrhea.  Profound  sensory 
loss  also  may  be  present. 


NUCLEUS  AH8I6UUS 
(I2*-»I) 


JJUCIEUS  OF 
UTARY  TRACT 


Fig.  305. — The  cranial  nen'e  nuclei  schematically  represented  in  a  supposedly 
transparent  brain-stem,  dorsal  view. 

The  residual  symptoms  will  vary  greatly  according  to  the  area 
destroyed.  If  recovery  takes  place,  which  is  comparatively  rare  in 
lesions  at  the  level  of  the  pyramidal  decussation,  at  least  three  sepa- 
rate clinical  pictures  are  recognizable.  If  the  lesion  occupies  the 
lower  side  of  the  medulla  implicating  the  pyramidal  tracts  one  observes 
a  more  or  less  continuous  spastic  paralysis  of  all  four  extremities  usually 


526      LESIONS  OF   THE  PONS,  BRAINSTEM  OR  MIDBRAIN 

more  marked  on  the  side  opposite  the  lesion;  if  it  impinges  further 
forward,  involving  the  nucleus  of  the  spinal  accessory,  there  is  added 
to  the  spastic  diplegia  a  flaccid  palsy  of  the  muscles  of  the  accessorius. 
Such  clinical  pictures  result  from  arterial  lesions,  chiefly  of  the  anterior 
spinal  just  below  the  juncture  of  the  vertebral  arteries  to  form  the 
basilar. 

A  lesion  at  the  side  of  the  medulla  here  (meningitis,  tumor,  gumma, 
tuberculosis)  involving  the  tractus  cerebellospinalis  would  cause  a 
cerebellar  ataxia. 

Lesions  slightly  higher  up  in  the  medulla,  at  the  level  of  the  oHve, 
for  example  and  involving  the  lemniscus  crossing  fibers  produce  a 
dissociated  disturbance  of  sensation  of  the  entire  half  of  the  body 
omitting  the  trigeminus  regions.  If  the  lesion  is  below  and  impinging 
on  the  pyramidal  region  (Fig.  306)  there  is  a  spastic  paresis  of  the 
opposite  half  of  the  body  with  atrophic  paralysis  of  the  same  side  of 
the  tongue,  which  shows  fibrillary  twitching,  deviates  to  the  non- 
paralyzed  side  and  gives  reaction  of  degeneration. 

Following  lesions  of  the  anterior  spinal  artery  the  lesion  may  impli- 
cate a  long  strip  up  and  down  the  medulla.  Thus  in  the  patient 
(Fig.  306)  inferior  alternate  hemiplegia  from  anterior  pyramidal  and 
interolivary  lesions,  the  picture  just  described  occurs,  with  addi- 
tion of  a  hemianesthesia  of  the  same  side.  There  are  no  deep  sensi- 
bility signs  (hemianalgesia)  nor  hemithermo-anesthesia.  Should  the 
lesion  cut  off  the  circulation  on  both  sides,  as  it  not  infrequently 
happens  in  anterior  spinal  artery  occlusion  from  vertebral  thrombi,  the 
picture  seen  in  Fig.  307  occurs.  Here  there  is  a  double  hemiplegia  and 
hemianesthesia  with  alternate  paralysis  of  the  tongue,  more  marked 
on  the  left  side.  There  is  atrophy  of  the  right  half  of  the  tongue  with 
fibrillary  twitching  and  reaction  of  degeneration. 


General  Legend  Abbreviations  of  Medullary,  Pontine,  Peduncular  and 
Midbrain  Syndromes. 

In  all  of  these  hemiplegia  is  indicated  by  oblique  lines;  hemianesthesiis  by  dots  and 
alternate  paralyses  by  gray  network.  The  lesions  involve  the  cerebral  peduncles,  and 
the  pons  at  different  levels. 

Abbreviations.  Aq.,  aqueduct  of  Sylvius;  BrQp,  peduncle  of  posterior  corpus 
quadrigeminum ;  Crst,  corpus  restiforme  and  inferior  cerebellar  peduncle;  Fee,  central 
tegmental  tract;  Fes,  internal  semicircular  fibers  of  the  cerebellum;  Flp,  posterior 
longitudinal  fascicidus;  FPoa,  FPop  anterior  and  posterior  pontine  fibers;  HC,  cere- 
bellar hemisphere;  Le,  locus  ceruleus;  LN,  locus  niger;  Lig,  lingula  of  superior  vermis 
of  cerebellum;  ND,  Deiters'  nucleus;  Np,  nuclei  of  pons;  NR,  red  nucleus;  NRl,  nucleus 
of  lateral  lemniscus;  Nrt,  reticular  nucleus  of  the  tegmentum;  NIII,  nuclei  of  third 
nerve;  NtnV,  motor  fifth  nucleus;  NsV,  sensory  fifth  nucleus;  NVI,  nucleus  of  sixth 
nerve;  NVII,  nucleus  of  the  facial  nerve;  NVIII,  anterior  nucleus  of  the  acoustic 
(cochlear);  Oc,  cerebellar  olive;  Os,  superior  or  pontine  olive;  P,  lower  stage  of  cere- 
bral peduncle;  Pern,  middle  cerebellar  peduncle;  Pes,  superior  cerebellar  peduncle;  Pu, 
pyramidal  tract  in  its  pedunculopontine  region;  Qa,  corpus  quadrigeminum  anterior; 
r,  raphe;  Rin,  median  lemniscus;  Rl,  lateral  lemniscus;  SAij,  gray  substance  of  the 
aqueduct  of  Sylvius;  SgR,  substance  of  Rolando;  Tpo,  tenia  pontis;  Tr,  trapezoid  body; 
Va,  fourth  ventricle;  Vs,  superior  vermis  of  the  cerebellum;  VV,  valve  of  Vieussens; 
///,  root  filjcrs  of  the  third  nerve;  V,  trigeminus;  Vc,  descending  motor  root  of  the 
fifth;  Vds,  descending  sensory  root  of  the  fifth;  VI,  root  fibers  of  the  sixth;  VII,  rVII, 
root  fibers  of  the  facial:    VI Ig,  knee  of  the  facial;  VII Iv,  vestibular. 


MEDULLARY  SYNDROMES 


527 


Lesions  of  the  vertebral  (usually  thromboses)  which  involve  the 
anterior  spinal  artery,  and  the  posterior  cerebellar  arteries  produce  an 
involvement  of  most  of  one-half  of  the  medulla  (Fig.  308) .  This  causes 
the  so-called  Syndrome  of  Avellis.  The  patient  shows  the  usual  apo- 
plectic initial  symptoms.  The  residuals  show  a  hemiplegia  and  hemi- 
anesthesia on  the  side  opposite  to  the  lesion  and  a  palsy  of  the 


NCB 


Fig.  306. — Antero-intemal  bulbar  syndrome.  Alternate  hemiplegia  of  the  hypo- 
glossus  by  thromlxjsis  of  the  left  anterior  spinal  arter>'.  The  lesion  involves  the  anterior 
pyramid,  the  reticular  formation  of  the  bulb,  the  root  fibers  of  the  hypoglossus,  but  not 
the  XII  nucleus. 

On  the  right  side  there  is  hemiplegia  of  the  extremities  and  hemianesthesia  especially 
of  deep  sensibility,  without  hemianalgesia  or  hemithermo-anesthesia. 

On  the  left  side  there  is  hemiatrophy  of  the  tongue  with  R.  D.  (Dejerine.) 


tongue  on  the  side  of  the  lesion.  I'here  is  a  trigeminal  anesthesia  of 
the  involved  side,  Oculopupillary  signs  are  present,  narrowing  of  the 
palpebral  fissure  on  the  side  of  tlie  lesion.  There  is  also,  from  implica- 
tion of  the  cerebellar  pathways,  a  cerebellar  asynergia.  There  is  also  a 
paralysis  of  the  soft  palate  and  vocal  cord  of  the  same  side  as  the  lesion. ' 

'  Sijillcr:     Jour.  Nerv.  and  Ment.  Dis.,  June,  1919. 


528      LESIONS  OF  THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 

This  clinical  pictiire  may  be  encountered  also  in  syringomyelia, 
multiple  sclerosis  or  tabes  where  it  is  of  gradual  development. 

Partial  clinical  pictures  of  the  syndrome  of  Avellis  have  been  termed 
the  syndromes  of  Schmidt  and  of  Jackson.  In  the  Syndrome  of 
Schmidt  there  is  a  unilateral  paralysis  of  the  soft  palate,  of  the  vocal 


ixa      Nxp  mo 


NCB 


^f///;? 


Fig.  307. — ^Antero-internal  bulbar  syndrome  from  occlusion  of  the  anterior  spinal 
artery  from  the  right  vertebra.  There  results  a  double  hemiplegia  with  alternate 
paralysis  of  the  hypoglossus.  The  lesion  more  marked  on  the  right  side  involves  both 
pyramidal  tracts  (Py)  in  the  bulb,  the  interolivary  paths  (Rm)  and  the  formatio  reticu- 
laris (rCl,  Co).  On  the  right  side  the  lesion  cuts  off  the  root  fibers  of  the  hypoglossus. 
There  is  hemiplegia  and  hemianesthesia  of  all  four  limbs,  more  marked  in  the  left  side 
The  sense  of  position  is  specially  involved.  On  the  right  side  there  is  hemilingual  atrophy 
with  R.  D.  (Dejerine.) 


cord,  of  the  sternocleidomastoid  and  of  the  trapezius.  The  spinal 
vagus  nucleus  and  the  spinal  accessory  nucleus  or  radicular  fibers  are 
involved. 

In  the  syndrome  of  Jackson  there  is  a  hemiparesis  of  the  soft  palate, 
the  vocal  cord,  the  sternocleidomastoid,  with  hemiparesis  of  the  tongue 
with  atrophy. 


MEDULLARY  SYNDROMES 


529 


NCB 


Fig.  308. — Retro-olivary  bulbar  syndromes.  Hemianesthesia  of  syringomj-elic  type 
with  alternate  paralysis  of  the  descending  root  of  the  trigeminus  and  of  the  spinal 
vagus  (syndrome  of  Avellis)  and  hemiparesis  of  the  pharyngo-larj-ngo-velo-palatine 
with  (a)  or  without  (b)  oculopupillary  vegetative  signs;  without  (a)  or  with  (6)  con- 
comitant alternate  paralysis  of  the  XII,  and  of  the  lower  portions  of  the  XI  pairs 
(syndrome  of  Jackson).  There  is  also  lateropulsion,  cerebellar  hemiataxia  and  hemi- 
asynergia  from  lesion  of  the  bulboretro-olivary  tegmentum  of  the  right  side  supplied 
by  the  inferior  and  posterior  cerebellar  artery.  There  is  no  crossed  hemiplegia  of  the 
limbs  nor  tactUe  nor  deep  hemianesthesia  because  of  the  integrity  of  the  pyramids 
and  of  the  reticular  formation,  and  particularly  of  the  interolivary  paths. 

On  the  left  side  there  is  analgesia  and  thermo-anesthesia  of  the  extremities,  of  the 
neck  and  the  back  of  the  head  from  lesion  of  the  crossed  secondary  sensory  paths  of 
the  retro-olivarj-  reticular  formation. 

On  the  right  there  is  hemiataxia  and  hemiasynergia,  more  marked  in  the  lower  extrem- 
ity with  lateropulsion  from  extension  of  the  lesion  to  the  restiform  body;  pharyngo- 
laryngo-velo-palatine  palsy  from  lesion  of  the  spinal  vagus;  anesthesia  of  the  face  from 
lesion  of  the  descending  root  of  the  trigeminus. 

In  a  there  are  vegetative  oculopupillarj'  signs  from  involvement  of  the  sympathetic 
fibers  in  the  lateral  reticular  formation  and  trigeminus.  In  h  the  sympathetic  fibers 
are  not  involved  but  the  XII  fibers  are  implicated,  hence  the  paralysis  and  atrophy 
of  the  tongue;  extending  lower  down  in  the  medulla  the  inferior  spinal  accessory  fibers 
being  caught  in  the  lesion  there  is  paralysis  of  the  external  branch  supplying  the  trapezius 
and  sternocleidomastoid.  (Dejerine.) 
34 


530      LESIONS  OF   THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 

These  bulbar  palsies  are  observed  in  vascular  lesions,  in  tabes,  pro- 
gressive bulbar  palsy,  multiple  sclerosis,  syringomyelia  and  occasionally 
in  poliomyelitis. 


NCB 


^f///^7 


Fig.  309. — -Antero-internal  retro-olivary  bulbar  syndrome.  Crossed  hemiplegia  and 
hemianesthesia  with  alternate  paralysis  of  the  hypoglossus,  the  descending  fifth  (syn- 
drome of  Avellis),  oculopupillary  vegetative  signs,  cerebellar  hemiasynergia  from  throm- 
bosis of  the  left  vertebral  before  the  giving  off  of  the  anterior  spinal  and  inferior  and 
posterior  cerebellar  arteries. 

On  the  right  side  there  is  crossed  hemiplegia,  pontine-bulbar  type,  of  the  trunk  and 
the  extremities  from  pyramidal  lesion;  hemianesthesia,  for  all  forms  of  sensibility, 
especially  for  pain  and  heat,  of  the  trunk,  neck,  extremities  and  back  of  the  head  from 
lesion  of  the  reticular  formation  (Rm,  rCl)  white  and  {SR)  gray. 

On  the  left  there  is  cerebellar  hemiasynergia  with  lateropulsion  and  hcmiataxia. 
There  is  hemilingual  atrophy  from  lesion  of  the  root  fibers  of  the  hypoglossus,  pharyngo- 
laryngo-velo-palatine  palsj'  (syndrome  of  Avellis),  with  dysphagia,  dysphonia,  rough- 
ening and  loss  ot  voice  from  lesion  of  the  root  fibers  and  anterior  nucleus  of  the  spinal 
vagus  (NXa),  myosis  and  narrowing  of  the  palpebral  fissure  from  sympathetic  oculo- 
pupillary fibers  in  the  lateral  reticular  formation  (SR);  hemianesthesia  of  the  face 
from  descending  fifth  lesions.      (Dejerine.) 

A  related  syndrome  due  to  lesions  in  this  general  region  is  that 
of  Babinski  and  Nageotte.^    These  patients  show  acute  or  subacute- 


1  Nouv.  Iconog.  de  la  SalpStri^re,  1902,  xv,  492. 


LESIONS  OF  THE  PONS  531 

involvement.  If  not  comatose  there  may  be  dizziness.  The  residual 
picture  is  that  of  a  crossed  hemiparesis,  hemianesthesia  with  deep 
sensory  losses,  and  difficulty  in  swallowing  (Fig.  296) .  These  patients 
have  to  be  supported  when  they  walk  with  legs  widespread.  There 
is  marked  cerebellar  asynergia  with  lateropulsion  to  the  side  of  the 
lesion.  The  abdominal  reflexes  are  not  apt  to  be  involved.  The  other 
reflexes  follow  the  usual  hemiplegic  type.  Irregularity  of  the  pupils, 
myosis  of  affected  side,  enophthalmos,  syringomyelic  dissociation  at 
times  occur. 

Cestan  and  Chenais  have  described  a  variant  which  is  a  combina- 
tion of  this  syndrome  with  the  palatovocal  palsies  of  the  syndrome  of 
Avellis. 

Lesions  of  the  Pons. — Softenings,  hemorrhages,  tumors,  gummata, 
multiple  sclerosis,  syringomyelia,  poliomyelitis,  tabes  produce  isolated 
or  complicating  lesions  of  the  pons,  which  varying  with  their  location 
and  size,  give  rise  to  a  bewildering  array  of  clinical  pictures  which 
merge  one  into  another  and  almost  defy  analysis.  A  careful  study  of 
the  motor  nuclei  and  of  the  motor  and  sensory  tracts  will  enable  the 
student  to  differentiate  these  pontine  s}^ldromes,  which  when  due  to 
lesions  implicating  the  seventh  nerve  nuclei  or  structiu-es  below  or  of 
its  branches  cause  the  various  typical  inferior  alternate  paralyses. 

The  most  classical  of  these  syndromes  are  those  of  Millard-Gubler, 
Foville  and  Gubler- Weber  combinations. 

Reference  to  Plate  I  (pp.  48,  49)  shows  that  the  pontine  fibers  com- 
mence to  cross  about  the  upper  level  of  the  tenth  and  twelfth  nerve 
nuclei.  The  syndromes  just  described — Schmidt,  Avellis,  Babinski 
and  Xageotte — result  from  lesions  just  below  this  general  area.  The 
course  of  the  corticonuclear  fibers  is  extremely  complex  throughout 
the  whole  pontine  levels  and  can  best  be  traced  from  Plate  VII,  pp. 
318, 319,  and  from  the  series  of  cross-sections  of  the  various  syndromes 
of  this  chapter. 

The  sensory  pathways  are  likewise  extremely  complicated. 

So  long  as  the  sensory  paths  were  in  their  spinal  route  they  were 
capable  of  a  certain  amount  of  isolation  either  as  they  entered  the  cord 
and  made  their  first  synapses,  or  as  they  continued  up  the  cord  in 
primary  or  secondary  paths.  But  as  these  paths  converge  to  enter 
the  brain-stem  they  become  anatomically  closer,  disease  processes  are 
apt  to  oN'errun  many  paths,  and  thus  the  analysis  becomes  increasingly 
difficult  up  to  the  entering  of  these  paths  into  the  optic  thalamus. 

The  most  searching  analyses  of  Head,  Holmes,  May,  Rothmann  and 
others  and  their  researches  tend  to  show  that  the  impulses  underlying 
sensations  of  pain,  heat  and  cold  seem  alone  to  run  unaltered,  either 
directly  or  by  intercalated  fibers  associated  with  the  ganglion  cells  of 
the  formatio  reticularis,  between  the  upper  end  of  the  spinal  cord  and 
the  optic  thalamus.  Here  are  received  the  regrouped  secondary 
•impulses  from  the  face  which  cross,  then  join  the  specific  paths  for 
pain,  for  heat  or  for  cold.  These  paths  are  so  situated  that  they  can  be 
interrupted  without  disturbance  of  any  other  form  of  sensation  of  the 


532      LESIONS  OF  THE  PONS,  BRAIN-STEM   OR  MIDBRAIN 

body,  and  the  analgesia  and  thermo-anesthesia  so  produced  resemble 
in  quality  the  loss  of  sensation  to  pain,  heat  and  cold  caused  by  a 
lesion  in  the  spinal  cord.     (See  Plates  X  and  XL) 


T>iN,ulf...L, 


ff.^t//fC 


Fig.  310. — Anterior  cephalic  pontine  syndrome.  Hemiplegia  of  cerebral  type  due  to 
thrombosis  of  the  upper  part  of  the  basilar  trunk.  The  lesion  is  unilateral,  occupying  the 
cephalad  part  of  the  pons,  or  the  anterior  portion  of  the  right  pons,  destroying  there  the 
corticospinal  pyramidal  fibers,  the  corticonuclear  fibers  of  the  facial,  the  masticators, 
and  the  hypoglossal.  It  does  not  involve  the  tegmentum  nor  the  root  fibers  of  the 
cranial  nerves. 

On  the  left  side  there  is  a  crossed  hemiplegia  of  the  trunk  and  extremities  with  con- 
tractures and  exaggeration  of  the  reflexes.  Left  inferior  facial  hemiplegia,  mild  superior 
hemifacial  paresis,  slight  widening  of  the  palpebral  fissure,  slight  drooping  of  the  external 
border  of  the  left  eyebrow,  slight  impairment  of  independent  closure  of  the  eyes;  hemi- 
paresis  of  the  masticators,  especially  of  the  internal  pterygoid.  Hemiparesis  of  the 
tongue.     Integrity  of  electrical  excitability.  • 

By  reason  of  the  predominance  of  the  muscles  on  the  sound  side  the  lips  and  jaw  are 
drawn  to  the  right  and  on  protrusion  of  the  tongue  it  points  to  the  paraly2ed  side,  due 
to  action  of  the  right  genioglossus.     (Dejerine.) 


LESIONS  OP  THE  PONS 


533 


Fig.  311. — Anterior  and  posterior  internal  pontine  syndrome.  Thrombosis  of  the 
middle  portion  of  the  basilar  artery  invohang  the  right  anterior,  mesial  basotegmental 
portion;  the  lesion  not  going  as  far  as  the  motor  and  sensory  nuclei  of  the  trigeminus 
and  avoiding  the  posterior  longitudinal  fasciculus.  There  is  contralateral  hemiplegia, 
on  the  left  side,  of  the  cerebral  type,  of  the  trunk  and  extremities  and  of  the  inferior 
facial  by  reason  of  the  lesion  of  the  corticospinal  pyramidal  and  facial  fibers  (Py) ; 
mild  hemiparesis  of  the  upper  facial,  hemilingual  paresis  and  hemiparesis  of  the  masti- 
cators (de\aation  of  the  chin  to  the  sound  side  by  implication  of  the  aberrant  pyramidal 
corticolingual,  and  corticotrigeminal  (motor)  fibers  which  travel  in  the  median  lemniscus 
(Rm)  and  the  tegmentum  (Sr)).     (See  Plate  I.) 

There  is  contralateral  hemianesthesia  for  all  kinds  of  sensibility  but  above  all  of  the 
sense  of  position  by  lesion  of  the  median  lemniscus  (Rm)  and  of  the  reticular  fornuv- 
ticn  of  the  tegmentum  {SR).  There  is  no  alternate  paralysis,  the  lesion  not  involving 
any  of  the  sensory  or  motor  cranial  nerve  nuclei.     (Dejerine.) 


534      LESIONS  OF  THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 

Thus  when  a  lesion  in  the  medulla  interferes  with  pain  pathways, 
not  only  may  the  skin  be  insensitive  to  prick,  but  the  readings  of  the 


Fig.  312. — Anterior  pontine  syndrome.  Inferior  alternate  paralysis  —  Millard- 
Gubler  type.  Contralateral  hemiplegia  of  the  trunk  and  of  the  extremities  of  the  ponto- 
medullary  type  (without  inferior  facial  palsy)  without  hemianesthesia,  and  without 
the  syndromes  of  Foville,  with  alternate  paralysis  may  be  of  the  sixth  nerve  alone  (a) 
or  of  the  sixth  and  seventh  (a  and  h) .  At  this  level  the  cortico-nucleo-facial  fibers  have 
left  the  pyramidal  tract  with  the  medullopontine  aberrant  fibers  (see  Plate  I)  and  are 
in  the  tegmentum,  hence  the  absence  of  the  inferior  contralateral  facial  palsy. 

At  the  right  there  is  hemiplegia  of  the  extremities  without  paralysis  of  the  inferior 
facial. 

To  the  left  there  is  convergent  internal  strabismus  by  paralysis  of  the  external  rectus; 
the  predominance  of  the  antagonists  carries  the  eye  to  the  left  and  up.  There  is  no 
forced  position  of  the  head,  the  associated  lateral  movements  of  the  eyes  being  possible, 
only  the  left  eye  is  involved. 

In  (b)  there  is  added  to  the  preceding  an  atrophic  facial  paralysis  with  RD  (VII), 
The  facial  folds  are  gone,  there  is  lagophthalmus,  drooping  of  the  angle  of  the  mouth  and 
paralysis  of  the  elevators.     (Dejerine.) 


LESIONS  OF  THE  PONS  535 

pressure  algometer  may  be  raised  on  the  analgesic  side.  In  the  same 
way  the  affected  area  of  the  body  may  be  insensitive  to  all  degrees  of 
heat,  and  to  all  stimuli  capable  of  evoking  normally  a  sensation  of 
cold.  In  the  medulla,  however,  in  distinction  from  lesions  of  the 
cord,  the  grosser  forms  of  pain  and  discomfort  may  traverse  other  paths 
if  the  usual  ones  are  closed,  whereas  in  the  cord  all  painful  impulses 
are  blocked  by  a  unifocal  lesion. 

At  the  medulla  and  pontine  levels,  moreover,  all  three  forms  of  sen- 
sibility' may  be  affected  together  or  any  one  may  escape  or  be  alone 
involved. 

These  impulses  of  pain,  heat,  and  cold  all  run  up  in- the  neighborhood 
of  the  fifth  nerve  nucleus,  and  in  cases  of  occlusion  of  the  postero- 
inferior  cerebellar  artery  and  from  other  destroying  lesions,  the  paths 
are  usually  implicated.  This  same  accident  may  occasion  a  dissocia- 
tion of  the  impulses  underlying  the  appreciation  of  posture  and  passive 
movement  from  those  concerned  with  spatial  discrimination. 

A  summary  of  the  findings  which  may  occur  from  the  lesions  which 
cut  off  the  sensory  pathways  between  the  nuclei  of  the  posterior 
columns  and  the  optic  thalamus  has  been  stated  by  Head  and  Holmes 
as  follows : 

1.  The  impulses  for  pain,  heat,  and  cold  continue  to  run  up  in 
separate  secondary  paths  on  the  opposite  side  of  the  nervous  system 
to  that  by  which  they  entered.  They  receive  accessions  from  the 
regi'ouped  afferent  impulses  from  the  nerves  of  the  head  and  upper 
part  of  the  neck. 

Although  these  paths  are  frequently  affected  together  they  are 
independent  of  one  another,  and  any  of  the  three  qualities  of  sensation 
may  be  dissociated  from  the  others  by  disease. 

2.  Lesions  of  the  spinal  cord  tend  to  diminish  simultaneously  all 
forms  of  painful  sensibility,  but  with  disease  of  the  brain-stem  the 
gross  forms  of  pain  and  discomfort  may  pass  to  consciousness,  although 
the  skin  is  analgesic.  This  applies  not  only  to  painful  pressure,  but 
to  the  discomfort  produced  by  excessive  heat. 

3.  The  impulses  .concerned  Avith  postural  recognition  part  company 
with  those  for  spatial  discrimination  at  the  posterior  column  nuclei. 
Up  to  this  point  they  have  travelled  together  in  the  same  column  of 
the,  spinal  cord,  but  as  soon  as  they  reach  their  first  synaptic  junction 
they  separate.  Above  the  point  where  they  enter  seconflary  paths  the 
power  of  recognizing  posture  and  passive  movements  can  be  effected 
independently  of  the  discrimination  of  two  points  and  the  appreciation 
of  size,  shape  and  form  in  three  dimensions. 

4.  It  would  seem  as  if  those  elements  which  underlie  the  power  of 
localizing  the  spot  touched  or  pricked  become  separated  oft'  from  their 
associated  tactile  impulses  before  they  have  actually  come  to  an  end 
in  the  optic  thalamus.  The  long  connection  of  localization  with  the 
integrity  of  tactile  sensibility  is  here  broken  for  the  first  time. 

All  these  changes  are  preparatory  to  the  great  regrouping  which 
takes  place  in  the  optic  thalamus. 


536        LESIONS  OF   THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 


Fig.  313. — Pontine  syndrome.  Millard-Gubler-Foville  syndrome.  Hemiplegia  of 
cerebral  type  with  hemianesthesia  of  the  extremities  and  of  the  head  of  the  side  opposite 
and  alternate  paralysis  may  be  of  the  {VI)  only  (a)  or  of  the  VI  and  VII,  (b)  by  throm- 
bosis of  the  inferior  portion  of  the  basilar  artery,  in  particular  of  the  median  pontine 
arteries  of  the  left  side.  The  lesion  occupies  the  anterior  part  of  the  pons,  destroys 
the  pyramidal  tract  (Py)  extends  to  the  tegmentum,  sections  the  median  lemniscus 
fibers  (Rm),  the  fibers  of  the  reticular  formation,  the  posterior  longitudinal  fasciculus 
(Flp),  the  root  fibers  of  the  VI  pair.  In  (b)  the  more  extensive  lesion  destroys  in  addi- 
tion the  root  fibers  of  the  facial  and  the  nuclei  of  the  VI  and  VII  and  the  fibers  of  the 
lateral  portion  of  the  reticular  formation. 

On  the  right  there  is  contralateral  hemiplegia  of  the  cerebral  type — extremities  and 
inferior  facial,  by  lesion  of  the  corticospinal  pyramidal  fibers  and  of  the  cortico-nucleo- 
facial  (aberrant -medullo-pontine  fibers).  Hemianesthesia  of  the  extremities  and  of  the 
face  in  (a)  involving  particularly  the  tactile  sensibility  and  postural  sense,  and  in  (6) 
all  modes  of  sensibility,  these  being  more  marked  in  the  head  than  in  the  extremities. 

On  the  left  side  there  is  paralysis  of  the  external  rectus  (VI)  internal  strabismus,  to 
which  there  is  added  in  (b)  a  paralysis  of  the  facial  {VI I)  inferior,  superior,  and  of  the 
platysma  myoides,  lagophthaimia,  drooping  of  the  angle  of  the  mouth,  flattening  of 
the  facial  lines. 

Furthermore,  there  is  paralysis  of  lateral  movements  of  the  eyes  toward  the  left  by 
lesion  of  the  left  posterior  longitudinal  fasciculus  (Flp).  By  reason  of  the  predominance 
of  the  antagonists  the  patient  looks  toward  the  right.     (Dejerine.) 


LESIONS  OF   THE  PONS 


537 


Inferior  Alternate  Paralysis.— Anterior  and  Posterior  Pontine  Syn- 
dromes.   ^Millard-Gubler,  Foville  Syndromes. 

Reference  to  Plate  I  and  Fig.  312,  p.  534,  shows  the  site  of  a 
lesion  which  produces  the  ^Millard-Gubler^  type  of  inferior  alternate 
paralysis.  The  lesion,  a  softening,  hemorrhage,  tabes,  isolated  tubercle, 
syringomyelia,  bulbar  palsy,  gumma,  polioencephalomyelitis,  rarely 
recurrent  palsy  in  migrainous  vascular  disturbance,  in  the  anterior  part 
of  the  pons  low  down  at  the  emergence  of  the  VI  nerve  and  internal  to 
the  peripheral  exit  of  the  VII  nerve,  causes  a  partial  paralysis  of  the 
limbs  and  trunk  on  the  opposite  side  of  the  body  to  the  lesion  and  an 
internal  strabismus  of  the  eye  of  the  opposite  side.  There  is  (a)  no 
facial  palsy  and  no  sensory  loss — hemianesthesia — the  median  lemniscus 
(Rm)  being  spared  in  this  lesion.  If  this  lesion  extends  laterally  (6)  to 
include  the  peripheral  VII  there  is  a  facial  palsy  on  the  side  of  the  lesion. 


Fig.  314. — Hemorrhage  of  pons,  causing  posterior  pontine  syndrome. 
[Compare  Fig.  316.] 

The  sixth  nerve  palsy  on  the  same  side  of  the  lesion  causes  an  internal 
strabismus.  The  seventh  nerve  palsy  is  of  the  peripheral  type  (see  6). 
There  is  reaction  of  degeneration,  atrophy  of  the  facial  musculature, 
lagophthalmos  from  levator  palsy,  the  angle  of  the  mouth  droops. 
There  are  no  sensory  disturbances. 

A  lesion  lying  internal  to  the  peripheral  emergence  of  the  VI  nerve 
can  cause  an  ordinary  hemiplegia  indistinguishable  from  a  cerebral 
palsy.    It  would  have  to  be  a  very  small  lesion,  however. 

A  still  rarer  delimitation  by  a  lesion  involving  the  VI  and  VII 
(between  a  and  b  peripherally  in  Fig.  312)  at  their  convergence  at 
this  level  will  cause  a  facial  palsy  with  an  internal  strabismus  and  no 

>  Gubler:  Memoire  sur  I'herniplegie  alterne,  Gaz.  Hebd..  1856-1859.  Prevost:  Thdse 
de  Paris,  1868. 


538      LESIONS  OF  THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 

other  symptoms.  These  are  usually  due  to  meningeal  syphilitic  lesions 
and  several  cases  have  come  under  observation.  Two  such  patients 
have  had  two  recurrences  of  this  combination  at  a  vear's  interval. 


FPoc 


Fig.  315. — Posterolateral  pontine  syndrome.  Lesion  of  the  lateral  part  of  the  left 
pontine  tegmentum,  involving  the  nuclei  of  the  trigeminus,  the  crossed  secondary  sensory 
paths  of  the  tegmentum,  partially  involving  the  superior  cerebellar  peduncle  (JPcs) 
and  the  median  lemniscus  (Rm),  and  not  involving  the  anterior  portion  of  the  pons. 

On  the  right  there  is  hemianesthesia  of  the  extremities  of  the  syringomyelitic  type, 
aboA'e  all  for  pain  and  temperature  sense. 

On  the  left  there  is  paralysis  of  the  muscles  of  mastication  (pterygoid,  masseter, 
temporal)  by  lesion  of  the  motor  nucleus  of  the  trigeminus.  There  is  slight  anesthesia 
in  the  trigeminus  region  (sensory  nucleus  V)  and  choreo-athetoid  movements  of  the 
extremities  from  involvement  of  the  superior  cerebellar  peduncle.     (Dejcrine.) 


LESIONS  OF   THE  PONS  539 

There  have  been  no  migraine  attacks  in  either.  In  one  the  Wassermann 
test  was  +  +  +  + ;  in  another  it  was  negative  and  a  beginning  multiple 
sclerosis  was  suspected. 

MiUard-Gubler-Forille  Syndrome. — ^This  syndrome  was  originally 
described  by  Foville.^  The  lesion  which  may  be  any  of  the  t^'pes 
already  cited  for  the  Millard-Gubler  syndrome,  but  is  more  apt  to  be 
vascular  (thrombus  of  vertebrals,  basilar  involving  the  middle  pontine 
vessels) ,  occupies  not  only  the  anterior  stage  of  the  pons  but  lies  along 
the  central  raphe  in  this  region  of  the  pons  (Fig.  312).  Thus  the 
median  lemniscus  (fillet)  (Rm)  fibers  are  involved  in  addition  to  the 
pyramidal  fibers,  the  fibers  of  the  reticular  formation,  and  even  the 
posterior  longitudinal  fasciculus  {Ftp) .  Thus  to  the  crossed  hemiplegia 
there  is  added  a  crossed  hemianesthesia  and  a  paralysis  of  the  associ- 
ated eye  muscles.  The  internal  rectus  of  one  side  (III  pair)  and  the 
external  rectus  of  the  opposite  side  (VI)  cannot  functionate  synergistic- 
ally.  This  oculogjTic  palsy  may  involve  one  or  the  other  side  according 
to  the  site  of  the  lesion,  the  eyes  being  turned  away  from  the  site  of  the 
lesion  (see  Plate  II).  If  the  root  fibers  of  the  facial  are  involved  (6), 
as  they  usually  are,  there  is  an  inferior  facial  palsy  of  the  same  side. 
This  type  of  paralysis  is  illustrated  in  Figs,  a  and  h  of  Fig.  313.  In  (6) 
the  facial  palsy  is  figured.  In  (a)  the  lesion  lies  internal  to  the  facial. 
The  hemianesthesia  involves  deep  sensibility  of  the  body  and  face. 
Postural  sense  is  also  involved.  The  losses  in  sensibility  are  more 
marked  in  the  face  than  in  the  extremities  and  there  is  no  marked 
affective  overresponse. 

Posterior  Pontine  Syndrome's. — Lesions  at  this  same  level  (VI  and 
VII  nerve  level — see  Plate  I)  by  lying  in  the  tegmental  or  posterior 
part  of  the  pons  and  behind  the  corticonuclear  and  corticospinal  fibers 
produce  a  combination  syndrome  if  the  lesion  lies  internal  to  the 
corpus  restiforme,  a  more  extended  sjTidrome  when  encroaching  upon 
this  structure  and  the  VIII  nerve. 

In  this  more  limited  posterior  pontine  syndrome  (usually  softening 
or  heniorrhage)  there  is  a  partial  crossed  hemianesthesia  from  minimal 
fillet  (Rm)  involvement,  alternate  paralysis  of  the  VI  and  VII  nerves, 
anesthesia  of  the  trigeminus.  The  anesthesia  is  a  syringomyelic  dis- 
sociated type  if  only  a  part  of  the  fillet  is  implicated.  Tactile  sensi- 
bility, sense  of  position,  and  stereognostic  sense  are  intact  (Fig.  315). 

A  more  extensive  lesion  (tubercle,  multiple  sclerosis,  gumma,  soften- 
ing (Fig.  316,  p.  540)  produces  a  much  greater  degree  of  crossed 
hemianesthesia  by  complete  cutting  oft"  of  fillet  fibers  (Rm).  There  is 
alternate  VI  and  VII  nerve  palsy.  There  is  conjugate  palsy  of  the  III 
and  VI  with  turning  of  the  eyes  away  from  the  side  of  the  lesion,  from 
involvement  of  the  posterior  longitudinal  fasciculus  (Flp).  Further- 
more, there  is  a  cerebellar  hemiataxia  from  the  cutting  oft'  of  fibers 
going  to  the  cerebellum  (Crst).  Involvement  of  the  spinal  vagus 
causes  a  palsy  of  the  ipsolateral  vocal  cord,  cutting  oft'  of  the  VIII 

•  Gasset:  Revue  Neurologique,  1900,  v-iii,  586,  cites  Foville,  Society  Anatomique, 
1858,  as  having  given  the  first  clear  description  of  this  syndrome. 


540      LESIONS  OF  THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 


Fig.  316. — Posterior  fcaudal)  pontine  lesion.  Contralateral  anesthesia  with  alternate 
paralysis  of  the  VI,  VII,  VIII  pair  and  of  the  spinal  vagus  (Xa)  from  a  tubercle  in  the 
inferior  (caudal)  portion  of  the  left  pontine  tegmentum.  The  tubercle  has  pressed  upon 
or  destroyed  the  secondary  crossed  paths  of  the  tegmentum  (SR) ,  the  median  lemniscus 
(Rm),  the  posterior  longitudinal  fasciculus  (Flp),  the  nuclei  and  root  fibers  of  the  exter- 
nal rectus,  of  the  facial,  the  vestibular  and  the  cochlear  branches  of  the  eighth. 

On  the  right  there  is  hemianesthesia  of  the  body  and  of  the  face  for  all  modes  of  deep 
and  superficial  sensibility.  Cerebellar  hemiataxia  and  lateropulsion  by  lesion  of  the 
cerebellar  pathways. 

On  the  left  there  is  atrophic  facial  paralysis.  (a)  superior  facial,  inferior  of  the 
platysma,  lagophthalmia,  drooping  mouth  angle,  cochlear  deafness,  vestibular  nystagmus, 
convergent  strabismus,  paralysis  of  the  lateral  associated  movements  of  the  eyes  toward 
the  left  by  lesion  of  the  internuclear  and  vestibular  left  oculorotary  fibers.  By  reason 
of  the  predominance  of  the  antagonists  the  patient  looks  to  the  right.  There  is  a  velo- 
palatine  paralysis  and  a  paralysis  tf  the  vocal  cords  (nuclei  and  root  fibers  of  the  spinal 
vagus').  Hemianesthesia  of  the  face  by  lesion  of  the  descending  root  of  the  trigeminus 
(a').     (Dejerine.) 


LESIONS  OF   THE  POXS 


541 


causes  deafness.  Vestibular  nystagmus  also  occurs  from  cutting  off  of 
vestibular  fibers.  Soft  palate  palsy  is  also  present.  Tumors  of  the 
pons  give  rise  to  a  very  varied  syndromy.^ 

Postero-inferior  Cerebellar  Syndrome.- — The  occlusion  of  the  pos- 
tero-inferior  cerebellar  artery  is  prone  to  produce  a  widespread  pontine 
s\Tidrome.  It  forms  one  of  the  apopletic  bulbar  palsies.  The  initial 
sjTnptoms  are  apt  to  be  acute  dizziness,  occasionally  Avith  transitory 
unconsciousness.  There  is  slight,  usually  passing  paresis  of  the  oppo- 
site extremity  with,  it  may  be,  transitory  motor  loss  of  fifth  of  the  same 
side.  Crossed  hemianesthesia  to  pain  and  temperature  and  ipsolateral 
trigeminus  sensory  impairment  of  the  first,  second,  or  all  three  branch 
distributions,  ipsolateral  hemiataxia  with  lateropulsion  to  the  side  of 


Fk;.   317. — Hemorrhage  of  pons.     [Compare  Fig.  314.] 

the  lesion.  Bilateral  nystagmus,  worse  on  side  looking  toward  lesion. 
Revolving  vertigo,  sometimes  headache  and  vomiting.  Avellis  syn- 
drome of  palatal  and  laryngeal  paresis  on  the  ipsolateral  side,  causing 
dy.sphagia  and  a  whispered  speech,  sometimes  taste  impairment  in 
the  anterior  two-thirds  of  the  tongue.  Occasionally  ipsolateral  sixth 
and  seventh  nerve  palsy  from  extension  of  the  softening.  Ipsolateral 
myosis,  narrowing  of  the  palpebral  fissure,  anhydrosis  and  enophthal- 
mos.     Occasionally  ipsolateral  loss  of  hearing. 

The  circulation  of  these  portions  of  the  cerebrospinal  axis  is  subject 
to  much  variation.     The  postero-inferior  cerebellar  is  usually  given 


•  Varet:     Thfese  de  Paris,  1905. 

»  Spiller:     Jour.  Nerv.  and  Ment.  Dis.,  1908,  xxxv,  365. 


542      LESIONS  OF   THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 


off  from  the  vertebrals  about  2  cm.  from  their  basilar  union.  The 
anterior  spinal  is  given  off  internally  just  below.  This  latter  enters 
the  raphe  and  is  distributed  along  the  raphe  (see  Fig.  307,  a  and  b) 
to  the  antero-olivary  bodies,  posterior  longitudinal  fasciculus  and 
twelfth  nerve  nucleus  first  on  the  floor  of  the  fourth  ventricle.  The 
postero-inferior  cerebellar  artery  is  chiefly  distributed  to  the  lateral 
parts  of  the  upper  medulla  and  lower  pons.  Branches  go  to  the  corpus 
restiforme.  (See  Fig.  316,  Crst.)  There  is  so  much  variation  in  the 
arteries  of  this  region  that  it  is  practically  impossible  to  state  always 
whether  a  clinical  symptom-complex  is  due  to  vertebral  or  to  inferior 
cerebellar  artery  disease.^ 

Peduncle  Syndromes. — Lesions  in  the  region  of  the  upper  level  of 
the  pons,  lower  border  of  quadrigemina,  exit  of  III  nerve  (see  Plate  I), 
give  rise  to  a  very  characteristic  group  of  clinical  pictures  variously 
termed  Gubler-Weber,  Weber  and  Benedict  syndromes. 


Fig.  318. — Weber's  syndrome. 


Fig.  319. — Weber's  syndrome. 


These  penduncular  syndromes  occur  from  lesions  similar  to  those 
just  discussed.  Thromboses  chiefly  of  the  basilar,  tumors,  polio- 
encephalitis, etc. 

Weber  s  Syndrome. — Superior  alternate  paralysis,  Gubler-Weber.^ 
These  patients  are  taken  acutely  ill,  are  comatose  or  unconscious  for 
the  most  part,  occasionally  as  in  Luton's  case  (earliest  recorded)  there 
is  no  loss  of  consciousness.  If  the  patient  recovers  the  residual  paralyses 
show  themselves.  These  will  vary  somewhat,  depending  upon  the 
extent  of  the  destruction  in  the  peduncles. 

1  Breuer  and  Marburg:  Obersteiner's  Arbeiteia,  1902,  ix,  181;  Blackburn:  Jour.  Compt. 
Neur.,  1907,  xvii,  493;  Duret:  Archiv.  de  Physiol,  norm.,  1873,  97;  Wallenberg:  Arch, 
f.  Psych.,  xxvii,  504. 

*Jelliffe:  Superior  Alternate  Hemiplegia,  Gubler-Weber  Type,  Interstate  Medical 
Journal,  1908,  xv.  No.  9;  Gublor:  Gazette  hebdomedaire,  1859;  Weber:  Med,  Chir. 
Transactions,  1863. 


PEDUNCLE  SYNDROMES  543 

When  the  lesion  extends  along  the  central  raphe  (as  in  Fig.  310), 
usually  from  arterial  disease,  an  acute  apoplectiform  palsy,  often  with 
transitory  or  complete  unconsciousness  results.  Many  of  these  lesions, 
especially  when  extensive,  result  in  sudden  death.  In  the  milder  forms 
the  various  multiform  after-pictures  manifest  themselves.  The  grave 
implication  of  the  interspersed  corticospinal  fibers  causes  a  contra- 
lateral hemiplegia  with  increased  tendon  reflexes,  clonus  and  Babinski 
phenomena.  Involvement  of  the  corticofacial  fibers  causes  a  contra- 
lateral lower  facial  palsy,  paralysis  of  the  tongue  muscles,  and  of  the 
motor  fifth,  deviation  of  chin  to  the  sound  side,  protrusion  of  tongue  to 
the  paralyzed  side.  Hemianesthesia  of  the  same  side  as  the  hemiplegia 
is  also  present.  It  involves  the  sense  of  position  as  well  as  all  other 
t>TDes  of  sensibility  on  the  entire  half  of  the  body.  In  middle-lying 
lesions  the  trigeminus  may  be  spared.  In  more  lateral-lying  ones, 
posterolateral  pontine  syndromes  (Fig.  315),  the  s^^ldrome  is  less 
extensive. 

In  (Fig.  320,  a)  the  lesion  occupies  the  middle  third  of  the  right 
peduncle,  cutting  off  only  a  portion  of  the  corticospinal  and  cortico- 
nuclear pathways.  There  is  a  crossed  hemiplegia  of  the  entire  side  of 
the  body,  the  extremities  from  the  cutting  off  of  the  corticospinal,  the 
inferior  branches  of  the  facial  from  the  corticonuclear  fibers.  There  are 
no  atrophies  and  no  reaction  of  degeneration,  the  paralyses  being  of 
the  cerebral  t}j>e.  The  tongue  protrudes  to  the  paralyzed  side.  In 
the  early  stages  of  a  severe  attack  with  conia  or  marked  somnolence 
there  may  be  no  h^^ertonicity  of  the  involved  hemiplegic  side,  but 
later  the  increased  knee-jerks,  ankle-clonus  and  Babinski's  phenomena 
appear.  The  lesion  being  confined  below  the  locus  niger  gives  rise  to 
no  sensory  symptoms.  In  certain  cases^  (Gruenewald,  Cestan)  the 
lesion  involves  lemniscus  fibers  (Benedict  syndrome  tj-pes)  and 
hemianesthesias  appear. 

On  the  same  side  of  the  lesion  a  III  nerve  pais}'  appears.  There  is 
ptosis  and  external  strabismus.  When  the  lesion  extends,  occupying 
two-thirds  of  the  cerebral  peduncles,  additional  symptoms  appear  (6). 
Thus  there  are  added  further  corticonuclear  involvements.  Thus  there 
is  a  conjugate  deviation  of  the  head  and  eyes,  the  head  turned  away 
from,  the  eyes  looking  toward  the  side  of  the  lesion;  there  are  motor 
fibers  involved  causing  difficulties  in  chewing,  and  further,  palsies  of 
the  soft  palate  and  pharynx  cause  dysphagia.  The  uvula  is  deflected 
to  the  sound  side  (side  of  the  lesion).  There  may  be  total  mydriasis 
with  loss  of  light  and  accommodation  reflexes. 

Some  of  these  patients  make  a  fairly  complete  recovery,  syphilitic 
arteritis  with  hemorrhage,  but  most  show  residuals  and  usually  develop 
further  attacks  which  lead  to  death. 

Benedicts  Syjidrunie. — Benedict  first  described  this  in  1872.^  These 
patients  usually  show  an  apoplectiform  onset.    Benedict's  first  case  was 

'  Jelliffe:     On   Lesions  of   the    Midbrain,   with   Special   Reference   to   the   Benedict 
Syndrome.    Interstate  Medical  Journal,  1911,  xviii.  No.  8. 
"  Jelliffe:  Loc.  cit.;  Benedict:  Nervenpathologie,  1872. 


544      LESIONS  OF   THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 


eu 


Fig.  320. — Anterior  peduncular  syndrome.  Weber's  syndrome.  Superior  alternate 
hemiplegia.    Produced  by  cerebral  peduncle  and  ///  root  fiber  lesion. 

On  the  right  side,  contralateral  hemiplegia  of  the  trunk  and  extremities  from  lesion 
of  the  corticospinal  pyramidal  fibers.  Facial  hemiplegia  inferior  branches,  hemiparesis 
of  the  tongue  from  lesion  of  the  corticonuclear  fibers  of  the  hypoglossus.  In  (o)  the 
lesion  involves  only  a  portion  of  the  corticospinal  and  corticonuclear  fibers.  In  (6)  the 
lesion  includes  all  of  the  pyramidal  corticospinal  and  corticonuclear  fibers,  even  those 
which  in  this  region  form  the  superficial  and  deep  pes  lemniscus  fibers.     (See  Plate  I.) 

In  addition  to  the  symptoms  common  to  (a)  and  (5)  indicated  in  the  illustrations,  (6) 
also  shows  a  conjugate  deviation  of  the  head  and  the  eyes  from  a  lesion  of  the  cortical 
oculorotary  fibers  destined  to  go  to  the  nuclei  of  the  oculomotorius  and  abducens 
{III-VI)\  difficulties  in  mastication  from  lesions  of  the  motor  corticotrigeminus  fibers; 
difficulties  in  swallowing,  of  phonation,  and  of  articulation  from  lesions  of  the  cortico- 
nuclear fibers  of  the  spinal  vagus. 

As  a  result  of  the  predominance  of  the  antagonists  ot  the  sound  side  (left)  in  (a)  and 
(6),  there  can  be  observed  a  deviation  of  the  mouth  (facial),  and  of  the  jaw  (masticators) 
toward  the  left,  a  de\'iation  of  the  tongue  to  the  paralysed  side  (action  of  sound  genio- 
glossus)  and  in  (6)  further  a  conjugate  de\aation  of  the  head  and  of  the  eyes  toward  the 
left  (action  of  the  external  branch  of  the  spinal  accessory  and  of  the  levorotary  ocular 
fibers)  and  a  deviation  of  the  uvula  toward  the  left  (spinal  vagus). 

On  the  left  side  there  is  a  direct  paralysis  of  the  third  (///)  nerve  with  ptosis,  divergent 
strabismus,  with  or  without  mydriasis  and  pupils  which  do  not  react  either  to  light  or 
convergence,  according  to  the  grade  of  destruction  of  the  root  fibers  of  the  oculomotorius 
(777).     (Dejerine.) 


PED UNCLE  S  YX DROMES 


545 


gradual  in  development  (multiple  tubercles).  They  may  show  the 
t;y"pical  Weber  hemiplegia  and  crossed  third  nerve  palsy  to  which  is 
added  a  hemianesthesia  and  a  choreo-athetoid-like  tremor.^    ^Yhen  the 


M>//f-tr. 


Fig.  321. — Posterior  peduncular  syndrome.  Contralateral  Benedict's  syndrome. 
Hemianesthesia  with  choreo-athetoid  movements  and  alternate  paralysis  of  the  third 
nerve  (///)  by  reason  of  lesion  of  the  right  pontine  tegmentum. 

On  the  left  side,  contralateral  hemianesthesia  of  the  extremities  and  of  the  face  from 
lesion  of  the  secondary  crossed  sensory  pathways  (Rrn  and  SR).  Choreo-athetoid  move- 
ments and  at  times  tremors  from  lesion  of  the  red  nucleus  and  of  the  superior  cerebellar 
peduncle  {Pes)  below  the  decussation. 

On  the  right  .side  paralysis  of  the  third  nerve  (HI),  with  ptosis,  external  strabismus 
(non-resistance  to  external  rectus),  with  or  without  mydriasis  and  pupils  immobile  to 
light  and  convergence,  depending  on  the  extent  of  the  lesion  of  the  root  fibers  of  the 
oculomotorius  (III).     (Dejerine.) 


lesion  fails  to  reach  the  corticospinal  or  corticonuclear  fibers  in  the 
peduncles  there  may  be  no  hemiplegia. 
Thus  in  the  case  illustrated   (Fig.  321)   there  is  a   contralateral 

'  Jelliffe:     On   Some   Obscure   Tremors   Due   to   Midbrain    Lesions,  Post  Graduate. 
1914,  756. 

dQ 


546      LESIONS  OF   THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 

Benedict  syndrome  without  any  hemiplegia.  There  is  a  complete  hemi- 
anesthesia from  implication  of  the  median  lemniscus  fibers *(/?m).  There 
is  ptosis,  external  strabismus  and  pupillary  immobility  (complete  or 
partial)  from  involvement  of  the  III  nerve.  The  choreo-athetoid 
movements  result  from  cutting  off  of  rubrospinal  (von  Monakow)  and 
rubrocerebellar  fibers. 

These  cases  are  due  to  hemorrhage,  softening,  tubercle,  multiple 
sclerosis,  s^T^hilis,  polioencephalitis,  etc.  The  course  and  treatment 
will  depend  upon  the  causative  lesion. 

Corpora  Quadrigemina  Syndromes. — General  lesions  of  the  corpora 
quadrigemina  bring  about  a  syndrome  often  called  Nothnagel's  syn- 
drome. Here  the  most  characteristic  pathological  agents  are  tumors, 
either  of  the  corpora  quadrigemina  themselves,  or  of  the  pineal  body. 
When  the  roof  of  the  midbrain  is  involved,  there  is  usually  headache 
and  vomiting  from  increased  intracerebral  pressure.  Optic  neuritis 
often  develops.  Dizziness,  staggering  and  rolling  gait,  with  irregular 
forms  of  oculomotor  palsy  are  present.  The  conjugate  vertical  move- 
ments of  the  eye  are  frequently  involved,  and  not  infrequently  there  is 
nystagmus.  The  pupils  are  usually  widely  dilated,  or  show  anomalous 
reactions  to  light  and  accommodation.  Hearing  is  modified  if  the 
posterior  quadrigeminal  bodies  are  involved.  Tumors  of  the  pineal 
body  (see  Pineal  Syndrome)  offer  special  features  for  consideration. 
These  have  been  studied  particularly  by  Bailey  and  Jelliffe,^  and 
consist  of  (1)  general  symptoms  of  intracerebral  pressure,  (2)  oculo- 
motor palsies  with  disturbed  pupillary  reactions  and  other  signs  of 
involvement  of  the  corpora  quadrigemina,  and  (3)  metabolic  symp- 
toms, due  to  the  disturbed  pineal  itself,  or  more  often  to  hydrops 
and  pressure  upon  the  pitu'tary  stalk .^  The  metabolic  symptoms 
are  interesting,  often  consisting  of  adiposity,  sexual  precocity,  with 
premature  development  of  sexual  characteristics,  and  occasionally 
cachexia.  Lesions  anterior  to  the  midbrain  involving  the  thalamus 
give  a  special  symptomatology  which  is  discussed  under  the  head  of 
Thalamic  Syndrome  (g.  v.). 

CoUiculns  Superior. — This  is  also  known  as  the  anterior  quadri- 
geminal body.  The  general  topographical  anatomy  has  been  discussed. 
The  finer  anatomy  of  the  superior  colliculus  shows  it  to  be  a  highly 
complicated  structure.  It  is  arbitrarily  separated  from  the  lower 
structures  of  the  midbrain. 

A  section  at  about  the  center  of  the  midbrain  shows  the  following 
structures:  (1)  An  outer  white  layer,  the  stratum  zonale,  made  up 
chiefly  of  fibers  derived  from  the  optic  tract.  There  are  also  fibers 
from  the  superior  quadrigeminal  brachia  and  small  horizontally  lying 
nerve  cells.  (2)  A  gray  layer,  stratum  cinereum,  made  up  chiefly  of 
ganglion  cells  whose  axis-cylinders  proceed  inward.  These  are  cells 
making  up  the  synaptic  junctures  of  the  optic  tract  fibers  and  are 
concerned  with  establishing  collateral  associations  with  other  nerve 

1  Tumors  of  the  Pineal  Body,  Archives  of  Internal  Medicine,  1911;  Kidd:  Review 
of.  Neurology  and  Psychiatry,  1912,  1913, 

2  Pell:  The  Pituitary,  1919, 


CORPORA   QUADRIGEMINA  SYNDROMES  547 

pathways,  chiefly  the  oculomotor  pathways  to  the  nuclei  of  the  eye 
muscles  and  also  to  those  of  the  neck  and  body.  (3)  A  layer  of  white 
fibers  containing  the  greater  mass  of  the  optic  tract  fibers  which  are 
terminating  about  the  ganglion  cells  of  the  third  and  the  fourth  layer. 
(4)  A  middle  gray  layer  of  cells  or  collateral  associations.  (5)  The 
lemniscus  layer  made  up  probably  of  fibers  of  the  median  and  lateral 
lemniscus.  (6  and  7)  Layers  of  ganglion  cells  with  axones  passing  to 
the  opposite  side  or  passing  ventrally  to  the  aqueduct  and  central  gray 
which  decussate  and  form  the  tectobulbopontospinal  tract  going  to  the 
anterior  columns  for  head,  neck  and  trimk  associations.  Fibers  from 
the  spinothalamic  tracts  and  from  the  temporal  and  occipital  cortex 
are  also  present. 

This  whole  structure  is  part  of  the  pathway  for  the  mechanism  of 
sight.  It  is  in  connection  with:  (a)  Centripetal  pathways:  (1)  From 
the  optic  tract  through  the  anterior  brachium  of  the  superior  colliculus. 
(2)  From  the  spinal  cord  through  the  posterior  columns,  their  nuclei, 
and  median  lemniscus.  (3)  With  the  spinal  cord,  lateral  columns, 
tractus  spinotectalis.  (4)  Occipital  lobes  through  the  internal  sagittal 
layers. 

(b)  Centrifugal  pathways :  (1)  To  the  occipital  optic  cortex  by  means 
of  the  anterior  brachiiun.  (2)  To  the  medulla  and  spinal  cord  by  means 
of  the  tectobulbospinal  tracts.  (3)  To  the  nuclei  of  the  oculomotor 
muscles,  light  reflexes,  etc. 

Thus  the  reflex  collaterals  in  the  superior  colliculus  are  homologous 
to  those  in  the  inferior  colliculus,  the  former  serving  to  join  up  light 
stimuli  with  the  rest  of  the  body — with  vegetative,  sensorimotor  and 
psychical  levels,  the  latter  subserving  homologous  functions  for  sound 
stimuli.  These  reflex  collaterals  do  not  subserve  any  sight  functions 
in  the  narrower  sense. 

From  these  anatomical  considerations  it  follows  that  a  complicated 
symptomatology  may  result  from  colliculus  lesions  which  will  vary 
according  to  which  tracts  or  groups  of  cells  are  involved.  Minute 
lesions,  as  in  poliomyelitis,  encephalitis,  cysticercus,  small  tubercles, 
miliary  aneurisms,  may  cause  very  few  syndromes,  isolated  reflex 
disturbances,  whereas  gross  lesions  from  pressure  of  tumors — pmeal, 
tubercle,  sarcoma,  etc.,  may  cause  more  widespread  symptoms.  One 
of  the  more  roughly  grouped  of  these  is  the  so-called  Xothnagel 
syndrome  just  described. 

Lesions  limited  to  the  structures  of  the  superior  colliculus  do  not 
cause  blindness  nor  hemianopsia.  The  lesion  must  extend  as  far  as  the 
geniculate  bodies  to  reach  those  optic  tract  fibers  which  are  continued 
farther  back  to  the  occipital  cortex  as  sight  fibers.  Although  fibers 
may  go  from  the  colliculus  to  the  occipital  lobes,  just  what  relationship 
they  have  to  seeing  is  still  uncertain.  The  chief  disturbances  of  pure 
colliculus  superior  disease  are  in  the  pupillary  reflexes,  eye  movements, 
and  balancing  functions  through  eye  movement. 

The  pathways  taken  by  the  pupillary  fibers  are  not  cleared  up  com- 
pletely as  yet.    They  pass  with  the  tractus  in  its  middle  portion,  pass 


548      LESIONS  OF  THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 

through  the  brachium  anterius  collicuH,  also  in  the  pulvinar  surface, 
and  end  in  part  in  the  superficial  gray  layer  of  the  superior  colliculus. 
Here  a  s;sTiaptic  junction  takes  place  to  connect  up  with  the  oculomotor. 
Lesions  limited  to  these  pathways  may  cause  inequality  of  the  pupils, 
possibly  the  Argyll-Robertson  phenomenon.  Such  may  follow  trauma, 
alcoholism,  or  syphilitic  meningeal  infiltrative  processes.  The  over- 
whelming majority  of  such  lesions  ai-e  syphilitic ;  poliomyelitis  or  infec- 
tious encephalitides  occasion  a  smaller  group  of  these  disorders.^ 

A  group  of  peculiar  associated  eye  palsies  are  found  in  lesions  affect- 
ing the  colliculus  itself  or  its  connections  with  the  eye-muscle  nuclei. 
Thus  a  break  in  the  connections  between  the  abducens  and  the  colliculus 
will  result  in  a  palsy  of  the  rectus  internus  on  looking  to  one  or  the 
other  side  or  of  only  one  side  without  any  palsy  of  the  abducens.  Inter- 
nus associated  palsy  without  loss  of  convergence  may  occur  (Fischer, 
Bielschowsky).  The  precise  anatomical  details  are  as  yet  lacking. 
Conjugate  palsy  has  been  frequently  described  as  mostly  due  to  tumors 
pressing  upon  the  colliculus  or  involving  it.  Here  the  eyes  may  be 
directed  to  right  or  left  but  cannot  be  raised  or  lowered,  or  only  one  of 
these  capacities  is  lost.  In  looking  down  the  eyelids  do  not  follow. 
Most  of  the  described  lesions  are  so  gross  that  it  is  difficult  to  localize 
the  precise  mechanisms.  Lewandowsky  regards  these  palsies  as  due  to 
a  break  in  the  pathways  going  from  the  cortex  to  the  oculomotor  nuclei. 

The  chief  producing  lesions  are  tumors  (gumma,  teratoma,  sarcoma, 
abscess,  cysticercus,  etc.),  multiple  sclerosis,  encephalitis,  arterio- 
sclerosis, etc. 

More  extensive  eye-muscle  palsies  are  referable  to  colliculus  disease, 
especially  if  the  lesion  press  farther  caudad  toward  the  nuclei  them- 
selves, or  involve  the  posterior  longitudinal  bundle. 

Ataxias  are  similarly  present  in  disorder  here  from  implication  of 
the  eye-muscle  functions  of  horizontal  vision  but  they  do  not  give  a  pic- 
ture of  pure  colliculus  disease,  neither  are  the  complex  motor  disturb- 
ances of  choreic,  paralysis  agitans,  or  athetoid  character  such  as  have 
been  described  in  von  Monakow  and  other  classics.  These  are  due 
more  particularly  to  cerebellar  and  vestibular  pathway  disturbances. 

That  the  superior  colliculus  is  associated  with  other  motor  reflexes 
is  certain,  but  no  precise  localizing  symptomatology  is  known.  The 
reflex  collaterals  with  the  vegetative  permit  the  fear  reactions  through 
sight — and  all  of  the  considerations  concerning  the  relation  of  the 
psyche  mentioned  in  the  paragraph  on  inferior  colliculus. 

CoUiciihis  Inferior.- — Also  known  as  the  posterior  quadrigeminal  body. 
Bechterew  (1885)  was  among  the  earlier  investigators  of  the  inferior 
colliculus  to  show  its  relations  to  the  cochlear  portions  of  the  acoustic 
nerve  and  hence  its  importance  in  hearing.  For  the  most  part  the 
results  obtained  by  him  in  Flechsig's  laboratory  have  been  verified  and 
amplified  by  the  work  of  Held,  Van  Gehuchten,  Lewandowsky,  Winkler, 
Spitzer  and  Karplus.    The  inferior  colliculus,  in  part  with  the  median 

1  See  Study  by  Riley:  Neurological  Bulletin,  1919,  ii,  p.  181,  on  Conjugate  Deviation 
of  the  Eye. 


CORPORA  QUADRIGEMINA  SYNDROMES  549 

geniculate  body,  form  the  two  chief  secondary  s\Tiapses  of  the  auditory 
pathway.    The  neurons  of  this  pathway  are  as  follows : 

First  Neuron:  Sensory  receptor  in  Corti's  organ,  ganglion  spirale; 
(a)  ventral  cochlear  nucleus  and  (b)  acoustic  tubercle. 

Second  Neuron:  (a)  Ventral  cochlearis  nucleus — corpus  trapezoides 
superior  olives — ventral  and  dorsal  lemniscus  nuclei  of  opposite  side; 
(6)  acoustic  tubercle  and  striae  acousticse,  superior  olive  of  both  sides. 

Third  Neuron:  Superior  olive,  lemniscus  nuclei  (lateral  lemniscus), 
colliculus  inferior  (posterior  quadrigemina) . 

Fourth  Neuron:  Inferior  colliculus,  arm  of  colliculus,  median 
geniculate  body. 

Fifth  Neuron:  ^ledian  geniculate — temporal-auditory  cortical  area. 

Cajal  assumes  that  the  cells  of  the  inferior  colliculus  are  not  direct 
but  collateral  synapses  for  shunting  auditory  stimuli  (reflexes)  to 
other  mechanisms,  ear  movements,  head  movements,  voice  production, 
eye  movements  in  part.  Anatomy  teaches  that  the  pathways  for  sound 
are  crossed  and  uncrossed  as  are  other  sensory  pathways,  hence  a 
lesion  of  one  colliculus  causes  no  marked  deafness,  although  interfer- 
ing somewhat  with  hearing  and  more  especially  with  many  auditory 
reflexes.  Experimental  stimulation  of  the  inferior  colliculus  has  brought 
about  dilatation  of  the  pupil  of  the  opposite  side — raising  of  eyebrows, 
protrusion  of  eyeballs  (autonomic  stimulation  from  fear  reactions  from 
sound  stimuli  in  normal  physiology),  turning  of  the  head  up  toward 
the  non-stimulated  side,  movement  of  ears  (in  animals)  and  a  number 
of  laryngeal  and  respiratory  stimuli  causing  voice  production  (cries, 
grunts,  etc.).  Ferrier,  who  performed  a  number  of  such  experiments, 
came  to  believe  that  a  psychical  center  was  revealed.  Prus  obtained 
somewhat  similar  results,  which,  with  the  information  available  from 
the  studies  in  the  vegetative  nervous  system,  would  tend  to  show 
that  the  auditory  synapses  in  the  inferior  colliculus  constitute  a  large 
factor  in  psychical  reactions  to  sound  stimuli  as  expressed  through 
the  autonomic  (vagus)  distribution.  Cocainization  of  these  bodies 
caused  clonic  convulsions,  raising  of  the  head,  movements  of  the  eyes 
to  the  opposite  side,  dilatation  of  the  pupils,  exophthalmos,  cardiac- 
cramp,  and  loud  crying,  i.  e.,  tj'pical  epileptiform  phenomena.  Prus 
interpreted  the  results  as  showing  a  motor  center.  Seen,  however,  from 
the  viewpoint  of  vegetative  neurology  it  would  appear  that  dishar- 
monic  vagus  activities  come  into  activity  (I^ewandowsky)  through 
cutting  off  of  psychical  (thalamic  homologue)  control,  i.  e.,  thalamic 
overresponse  occurred  in  the  sense  in  which  Head  has  described  it  for 
the  thalamic  lesions  in  which  tactile  sensibility  was  under  discussion. 
These  reflections  would  lead  to  a  review  of  the  subject  of  the  psycho- 
genic factors  in  certain  epileptic  attacks  associated  with  infantile  fear 
shock  conditioned  by  auditory  impressions.  In  this  connection  Oppen- 
heim's  statement  that  overresponse  to  sounds  is  seen  in  diplegias, 
might  have  a  wider  significance  than  he  seems  to  give  it.  Furthermore, 
the  problem  of  tone  production,  as  contrasted  with  tone  perception, 
acquires  wider  significance  than  that  given  to  it  by  the  original  Helm- 


550      LESIONS  OF   THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 

holtz  peripheral  hypothesis,  since  the  reflex  activities  of  the  tensor  tym- 
pani  are  apparently  involved  in  colliculus  inferior  disorder.  Bechterew 
has  further  shown  that  the  genital  apparatus  (erection,  contraction  of 
uterus,  stimulation  of  milk  secretion)  is  also  reflexly  aflfected  through 
the  inferior  colliculus.  As  the  autonomic  pelvic  functions  are  homo- 
logues  of  the  vagus  autonomics  this  seems  logical,  and  contributes 
further  light  on  the  psychoanalytic  hypotheses  of  repression  of  the 
sexuality  and  certain  epileptic  attacks  (Maeder).  From  the  reflex, 
somatic  side,  a  study  by  Frey  and  Fuchs  is  of  interest  on  the  subject 
of  reflex  epilepsy  and  ear  and  nose  disease,  also  the  classical  reports  of 
Hughlings  Jackson  and  Ormerod. 

Clinical  neurology  still  lags  behind  the  anatomical  and  physiological 
correlations.  Charcot  has  noted  a  tabetic  with  deafness  in  whom  the 
inferior  colliculus  was  involved.  Flechsig  has  recorded  hallucinations 
of  hearing  and  Weinland  has  called  attention  to  disturbances  of  hearing 
from  lesions  of  the  inferior  colliculus,  and  possibly  incorporating  other 
geniculate  fibers.  The  relationships  of  disturbed  autonomic  functions 
to  collicular  disease  have  not  received  sufficient  attention  from  neurolo- 
gists and  otologists.  The  literature  on  deafness  with  dumbness  has 
not  been  gone  into.  The  tinnitus  of  neurasthenia,  of  the  psycho- 
neuroses,  the  great  importance  ascribed  to  sounds  in  many  psy- 
choses, hallucinations  in  schizophrenics,  in  manic-depressives,  have  as 
yet  received  no  satisfactory  analyses  from  either  the  neurological  or  the 
psychiatric  disciplines. 

RABIES. 

Rabies  is  an  acute,  specific,  infectious  disease,  which,  after  the 
sj'mptoms  have  appeared,  is  almost,  if  not  quite  always,  rapidly  fatal. 
While  the  disease  may  occur  in  any  mammal,  it  almost  always  results 
in  man  from  the  bite  of  the  dog.  The  virus  travels  from  the  wound 
along  the  nerve  trunks  via  the  spinal  cord  to  the  medulla  and  brain 
much  in  the  same  way  as  does  the  tetanus  toxin.  It  is  contained  in  the 
saliva  of  the  rabid  dog.  For  these  reasons  wounds  upon  the  exposed 
portions  of  the  body  and  those  containing  a  large  nerve  supply  are 
most  dangerous. 

The  incubation  period  varies  within  wide  limits,  but  in  man  on  the 
average  it  is  about  forty  days,  though  it  may  be  prolonged  for  a  number 
of  months. 

Symptoms. — The  sjVTiiptoms,  appearing,  as  a  rule,  from  two  to  six 
weeks  after  the  wound,  are  at  first  pain  and  redness  in  the  wound  itself 
and  a  condition  of  depression,  restlessness,  headache,  and  insomnia — 
the  prodromal  stage.  Two  to  four  days  later  the  stage  of  excitement 
begins  with  tonic  spasms  of  the  muscles  of  the  pharynx  and  larynx, 
with  involvement  also  of  the  respiratory  muscles,  producing  difficulty 
of  respiration.  Increased  flow  of  saliva  and  severe  thirst  is  accom- 
panied with  an  inability,  to  swallow,  because  attempts  at  swallowing 
produce  spasm  of  the  muscles  of  the  pharynx.     It  is  because  of  this 


TETANUS  551 

symptom  that  the  patient  fears  to  touch  water,  thus  the  common  name 
of  the  disease — hydrophobia.  The  excitement  increases  rapidly  for 
from  one  to  three  days,  when  the  paralytic  stage  begins,  with  a  rapidly 
developmg  weakness  and  paralysis  involving  the  muscles  of  the  face, 
tongue,  and  the  eye  muscles.  Temperature  rises  and  death  occurs  in  a 
few  days. 

Pathology. — The  most  characteristic  features  of  the  pathology  are: 
(1)  the  presence  of  the  so-called  Negri  bodies  (Neurocytes  hydropho- 
bise),  which  occur  especially  in  Ammon's  horn.  The  nature  and  mean- 
ing of  these  bodies  is  not  altogether  clear.  Negri,  as  indicated  by  the 
name  above,  presumed  them  to  be  protozoa;  (2)  the  so-called  nodules 
rabiques  of  Babes,  which  are  collections  of  small  foci  of  mononuclear 
cells  about  the  spinal  cord  ner^-e  cells;  (3)  the  lesion  of  Van  Gehuchten 
and  Nelis.  These  lesions  are  found  in  the  peripheral  ganglia  of  the 
cerebrospinal  and  sympathetic  nervous  systems,  especially  in  the  plexi- 
form  ganglia  of  the  pneumogastric  and  in  the  Gasserian  ganglia,  and 
consist  of. a  proliferation  of  the  endothelial  cells  surrounding  these  gan- 
glia, the  nerve  cells  being  pushed  aside  and  even  destroyed,  the  ganglia 
finally  containing  many  round  cells. 

Treatment. — The  immediate  treatment  of  a  wound  inflicted  by  a 
suspected  animal  should  be  thorough  cauterization  by  fuming  nitric 
acid,  if  possible.  In  its  absence  the  actual  cautery  may  be  used. 
Strong  solutions  of  carbolic  acid  and  formalin  are  poor  substitutes, 
while  nitrate  of  silver  is  of  no  value. 

The  most  important  treatment,  however,  is  the  Pasteur  treatment. 
The  principle  of  this  treatment  is  the  production  of  an  active  immunity 
by  the  use  of  an  attenuated  virus.  For  the  details  of  the  treatment  the 
student  is  referred  to  special  works.  In  any  event,  the  general  prac- 
titioner cannot  apply  it,  but  must  take  his  patient  to  a  laboratory 
especially  equipped  for  the  purpose. 

The  principle  of  the  treatment  is  to  produce  immunity  before  the 
appearance  of  the  s\Tnptoms.  Inasmuch  as  the  incubation  period  is 
on  the  average  forty  days,  this  is  possible,  as  immunity  is  produced, 
by  the  injection  of  an  attenuated  virus,  at  the  end  of  two  weeks.  This 
methofl  of  treatment  has  materiall}'  reduced  the  fatality  of  this  disease. 


TETANUS. 

Etiology. — The  cause  of  tetanus  is  the  Bacillus  tetani.  This  is  an 
anaerobic  bacillus,  rather  widely  distributed,  normally  inhabiting  the 
intestinal  tracts  of  herbivora,  particularly  of  horses  and  cows,  and 
also  of  man.  It  is  a  spore-forming  organism.  The  spores  are  extremely 
resistant  to  destructive  agencies  and  are  found  in  the  soil,  especially 
soil  which  has  been  contaminated  by  the  feces  of  horses  and  of  man. 
The  symptoms  of  tetanus  are  due,  not  to  the  baci'his  directly,  but  to  a 
toxin  elaborated  by  it,  which  travels  up  the  axjs-cylinders  of  the  nerves 
to  the  cord  and   brain. 


552      LESIONS  OF   THE  PONS,  BRAIN-STEM  OR  MIDBRAIN 


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554      LESIONS  OF  THE  PONS,   BRAIN-STEM  OR  MIDBRAIN 


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The  incubation  period  varies  within  considerable  limits,  from  three 
or  four  days  up  to  as  many  as  twenty  days,  the  severity  of  the  infection 
being  fairly  well  indicated  by  the  earliness  of  the  onset  of  symptoms. 

Symptoms. — The  symptoms  of  the  disease  are  essentially  those  of 
tonic  spasmodic  contractions  of  the  voluntary  musculature.  The 
muscles  involved  at  first  feel  stiff  and  are  subsequently  thrown  into 
convulsions,  particularly  the  face,  giving  rise  to  the  characteristic 
risus  sardonicus.  Later  the  trunk  muscles  are  involved,  producing 
severe  convulsions  and  bending  of  the  body  toward  those  most  affected. 
The  muscles  of  mastication  are  early  involved,  while  the  spasm  of  the 
muscles  of  respiration  and  of  the  larynx  interfere  with  breathing  and 
hasten  exhaustion  and  the  end.  Facial  paralysis  is  an  occasional 
complication  when  the  point  of  entry  of  the  infection  has  been  the 
face.  The  mind  remains  clear,  as  a  rule,  and  there  is  no  temperature, 
except  toward  the  end.  The  convulsions  are  excited  by  slight  stimu- 
lation, such  as  noises,  much  as  in  strychnine  poisoning. 

Course  and  Diagnosis. — In  the  severe  forms  death  generally  eventuates 
in  three  or  four  days.  The  disease  must  be  differentiated  from  strych- 
nine poisoning,  tetany,  hysterical  and  epileptic  types  of  convulsions, 
hydrophobia,  and  meningitis. 

Treatment. — The  prophylactic  treatment  is  largely  surgical,  involving 
the  proper  treatment  of  wounds,  particularly  punctured,  contused, 
and  infected  wounds.  The  bacillus  appears  to  thrive  especially  in 
mixed  culture,  and  so  infected  wounds  are  especially  dangerous.  The 
specific  treatment  is  by  the  use  of  tetanus  antitoxin,  which  should 
be  administered  after  any  suspicious  wound,  without  waiting  for 
symptoms.  A  prophylactic  dose  of  about  1500  units  may  be  given. 
As  soon  as  symptoms  appear,  however,  the  larger  dose,  about  20,000 
units  should  be  administered.  It  is  well,  too,  to  inject  some  antitoxin 
into  the  large  nerve  trunks  leading  from  the  wound,  and  dry  tetanus 
antitoxin  may  be  dusted  upon  the  wound  itself.  As  the  antitoxin  is 
eliminated  in  about  two  weeks,  if  further  effects  are  desired  from  it, 
additional  injections  will  be  necessary.  After  the  toxin  has  combined 
with  the  motor  nerve  cells  it  cannot  be  displaced  or  neutralized  by 
antitoxin.  The  antitoxin  can  only  neutralize  the  free  toxin.  Good 
results  have  been  reported  by  the  intravenous  injections  o '  magnesium 
sulphate,  in  conjunction  with  the  use  of  antitoxin,  and  also  by  the 
hypodermic  use  of  magnesium  sulphate. 

The  lyrognosis  has  been  materially  improved  since  the  advent  of 
antitoxin  treatment. 


CHAPTER  VIII. 

PARALYSIS  AGITANS,  LENTICULAR  DEGENERATION, 
CHOREA,  AND  RELATED  SYNDROMES. 

PARALYSIS  AGITANS  GROUP. 

History. — ^The  clinicians  of  the  times  immediately  preceding  Parkin- 
son grouped  the  paralysis  agitans  cases  of  the  present  day  in  several 
different  categories.  Galen  had  noted  the  characteristic  tremor,  and 
the  class  of  tremors  of  this  kind;  the  "tremblement  palpitant"  of 
Preysinger  was  part  of  the  earlier  imhnos  of  Galen.  Francis  de  la 
Boe  was  shrewd  enough  to  notice  the  difference,  afterv\'ard  forgotten, 
between  the  tremor  produced  by  attempts  at  motion  and  the  tremors 
present  while  the  limbs  were  at  rest,  and  his  term  tremor  coactus, 
for  the  tremors  of  paralysis  agitans,  was  utilized  up  to  Parkinson's 
time.  Juncker  had  also  described  a  paralytic-like  tremor,  tremores 
paralytoidei,  which  included  some  of  these  patients. 

Not  only  was  the  tremor  appreciated,  but  the  clinicians  of  the 
eighteenth  century  (Gaubius,  1751)  had  called  attention  to  the  pro- 
pulsion of  these  patients,  and  Sauvages  groups  them  in  his  choreas, 
as  Scelotrybe  precipitee  (Danse  de  St.  Guy  precipitee,  L.). 

Parkinson,  in  his  famous  thesis  on  the  "Shaking  Palsy,"  London, 
1817,  made  a  synthesis  of  several  of  these  conditions,  and  erected  a 
new  clinical  form  to  which  he  gave  the  name  shaking  palsy  (paralysis 
agitans),  and  gave  the  following  short  and  striking  description: 
"Involuntary  tremulous  motion,  with  lessened  muscular  power,  with 
a  propensity  to  bend  the  trunk  forward,  and  to  pass  from  a  walking 
to  a  running  pace,  the  senses  and  the  intellect  being  unimpaired." 

All  of  the  case  histories  cited  by  Parkinson  were  type  cases  of  our 
present-flay  limited  paralysis  agitans  syndrome,  but  the  group  as 
then  understood  still  contained  certain  of  the  chronic  choreas,  and 
certain  cases  of  multiple  sclerosis,  possibly  certain  thalamic  cases,  etc., 
which  later  clinicians  have  agreed  to  separate.  The  chronic  choreas 
were  definitely  excluded  by  the  work  of  the  Gennain  See,  1851,  and  the 
researches  of  B.  Cohn,'  Ordenstein,-  and  Charcot  finally  separated  the 
multiple  sclerosis  syndrome. 

Within  recent  years  a  large  medley  of  closely  related  s\nidromes 
has  been  described  in  all  of  which  certain  resemblances  to  the  more 
classical  types  of  Parkinson's  syndrome  may  be  recognized.  Many 
have  been  named;  thus  the  pseudosclerosis  of  Westphal,  Striimpell's 
pseudosclerosis,  certain  forms  of  Huntington's  chorea,  Wilson's  len- 

•  Wien.  med.  W'chnschr.,  No.  18.  2  Thfese  de  Paris,  1868. 

(557) 


558     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

ticular  degeneration,  Cecil  Vogt's  syndrome  of  the  corpus  striatum, 
Bechterew's  hemitonia  apoplectica,  Hunt's  syndrome  of  the  globus 
pallidus,  Oppenheim's  dystonia  musculorum,  Spiller's  family  forms  of 
pseudosclerosis,  etc.^  Some  of  these  trends  have  been  sufficiently 
isolated  to  permit  short  descriptions  in  this  work,  but  the  interested 
student  is  referred  to  the  bibliographic  notes  here  appended  for  fur- 
ther investigation  of  what  constitutes  a  most  profitable  field  for 
research . 

Charcot's  studies,  the  monographs  of  Wollenberg,  Heimann,  Man- 
schot  (Winkler)  (1904),  Zingerle  (1910),  Mendel  (1911),  Wilson 
(1913),  Spiller  (1916)  and  Hunt  (1917),  contain  the  chief  steps  made, 
showing  the  steadily  advancing  trend  to  regard  the  Parkinsonian  type 
not  in  the  light  of  a  functional  disturbance,  as  Charcot  taught,  but 
as  a  closely  related  group  of  somatic  s^nadromes,  due  to  the  involve- 
ment, either  circumscribedly  or  more  diffusely  of  certain  cerebellar, 
thalamic  and  lenticular  pathways,  and  of  certain  synapses  in  the  motor 
and  vegetative  cells  of  the  globus  pallidus  and  related  basal  ganglia. 

W^e  shall  first  discuss  the  more  classical  type  as  more  sharply  delim- 
ited by  Parkinson,  again  reminding  the  student  that  names  stand  for 
general  categories,  rather  than  for  specific  things.  The  splitting  up 
of  this  large  group  is  still  going  on  and  the  details  must  be  consulted 
in  special  monographs.^ 

Etiology. — The  changes  of  age  seem  to  be  the  most  striking  etio- 
logical factors.  The  majority  of  the  patients  are  between  fifty  and 
seventy — although  cases  of  patients  of  nineteen,  fifteen,  twelve,  ten, 
and  three  years  of  age  are  recorded,  some  of  which  have  been  possibly 
faultily  diagnosed,  the  vast  majority  of  these  being  multiple  sclerosis, 
encephalitis  or  poliomyelitis.  Hereditary  factors  may  play  a  role, 
probably  through  vascular  disease.  Berger,  Gowers,  Borgherini, 
Clerici  and  Medea  and  others  have  reported  cases  occurring  in  two 
generations  or  in  more  than  one  member  of  the  same  generation,  and 
Erb  reports  that  in  15  per  cent,  of  his  cases  the  parents  or  grandparents 
suffered  from  the  same  disease.  Concerning  indirect  heredity,  the  less 
said  the  better,  as  the  studies  available  are  entirely  too  conflicting, 
and  for  the  most  part  inapplicable. 

Emotional  disturbances  are  held  accountable  by  many  writers;  it 
is  difficult  to  determine  here  whether  one  is  concerned  with  cause  or 
effect.  Sorrow,  worry  and  emotional  distress  are  such  universal  all- 
pervading,  environmental  factors,  that  too  much  stress  must  not  be 
placed  upon  them.  Sudden  shock  may  perhaps  stand  in  an  accen- 
tuating, accidental  relationship. 

Trauma  stands  in  a  possibly  closer  relationship.  It  is  highly  improb- 
able as  a  direct  cause,  but  it  may  be  a  sufficiently  exciting  cause  to 
bring  the  symptoms  of  a  slumbering  paralysis  agitans  to  the  surface, 

1  See  Hunt,  J.  R.:  Brain,  1917,  for  complete  bibliography.  Spiller:  Jour,  of 
Nervous  and  Mental  Disease,  1916,  p.  23.  Wilson:  Brain,  vol.  xxxv,  p.  36.  Vogt: 
Jour,  of  Psych,  u.  Naur.,  1917,  vol.  xviii.    Westphal:  Arch.  f.  Psychiatrie,  1913,  vol.  li. 

*  Wilson,  1.  c;  Hunt,  1.  c;  Tilney,  1.  c. 


PARALYSIS  AGITANS  GROUP  559 

or  those  of  a  mild  case  rapidly  to  a  severe  stage.  In  the  recent  great 
war  shell  shock  has  been  prolific  in  bringing  certain  paralysis  agitans 
and  multiple-sclerosis-like  tremors  to  the  fore.  All  of  these  have  not 
been  tremophobias  as  ]\Ieige  has  termed  them,  nor  hysterias  as  diag- 
nosed by  others,  but  probably  many  are  the  results  of  somatic  lesions. 

Physical  stress  is  a  factor  which,  bearing  upon  arteriosclerosis,  may 
be  an  accompanying  factor  in  certain  cases.  Toxic  factors  of  them- 
selves are  not  kno\Mi  to  play  any  necessary  role.  Their  coincidental 
occurrence  is  frequently  reported;  the  same  may  be  said  of  infections. 
Cold,  exposure  to  wet,  and  other  actors  are  probably  more  accidental 
than  vital;  they  may  augment  the  action  of  an  underlying  factor,  as 
yet  unknowai ;  they  may  represent  purely  coincidental  features. 

Arteriosclerosis  is  the  chief  factor  in  bringing  about  the  presenile 
syndrome.  The  central  features  of  the  syndrome  are  matters  of  locali- 
zation in  the  implication  of  certain  pathways  by  the  sclerosing  process. 

The  hypothesis  that  the  disorder  is  due  to  a  hyperfunctioning  of 
the  parathyroid"  (Lundborg)  is  still  purely  suggestive  of  the  general 
problem  of  motor  activity  in  relation  to  the  effect  that  calcium  metab- 
olism has  upon  motor  impulse  conduction.    (See  chapter  on  Tetany.) 

Symptoms. — These  develop  for  the  most  part  very  slowly,  although 
occasionally  patients  are  seen  who  show  fulminating  types,  and 
although  atypical  developments  are  known,  the  regularity  and  uni- 
formity iii  the  development  is  very  striking. 

The  vast  majority  of  the  patients  show,  on  close  analysis,  prodromal 
symptoms  which  are  chiefly  sensors',  in  contrast  to  the  sensorimotor 
symptoms  of  the  more  advanced  stages  of  the  picture.  The  more 
characteristic  of  these  prodromata  are  fugitive,  irregular  pains  of  a 
sharp,  lancinating  character,  frequently  found  in  the  extremities  first 
to  be  affected  by  the  motor  disturbances,  and  usually  ceasing  as  these 
latter  advance.  Paresthesise  are  also  frequent,  causing  sensations 
of  tickling,  cold  spots,  hot  spots,  gastric  distress — almost  crisis-like 
attacks,  with  diarrhea  and  colicky  disturbances  in  the  large  intestine. 

General  mialaise  with  headache,  sweating,  mild  vertigo,  palpitation, 
sialorrhea,  anxiety,  pressure  of  blood  in  the  head,  easy  excitability, 
are  general  symptoms  accompanying  many  senile  and  presenile 
conditions,  but  are  so  frequently  found  as  forerunners  of  the  motor 
symptoms,  and  persist  with  such  marked  increase  of  severity  through- 
out the  disorder  that  their  appearance  is  to  be  regarded  as  more  than 
coincidental. 

The  symptoms  of  the  more  classical  syndrome  may  be  grouped  as 
follows:  (1)  The  main  group  of  sensorimotor  disturbances,  varying 
in  intensity  aiKl  location  in  different  individuals.  (2)  A  number  of 
sensorv',  vasomotor,  trophic  and  secretorj'  disturbances  already  indi- 
cated as  often  in  part  occurring  as  prodromes.  (3)  Psychical  symptoms 
which  are  somewhat  variable  and  possibly  not  essentially  related  to 
the  disorder  per  se. 

The  sensorimotor  disturbances  are  predominantly  increase  of  mus- 
cular tonus,  with  rigidity,  and  resulting  contractures,  and  motor  dis- 


560     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

turbances   with   tremor,    compulsory   gait,   forced   attitudes,    forced 
movements,  and  loss  of  mimetic  expression. 

An  increase  in  the  muscular  tonus  is  a  most  fundamental  feature 
in  the  concept  paralysis  agitans.  As  a  result  of  it  there  follows 
the  rigidity,  the  mask-like  countenance,  and  the  contractures.  The 
increase  in  the  muscle  tonus  usually  is  a  very  early  sign,  although 
positive  traces  of  rigidity  may  not  appear  until  later.  It  is  practica  ly 
always  found,  whereas  there  are  some  patients  who  have  little  or  no 
tremor^  and  yet  the  name  paralysis  agitans  is  properly  used.  Asso- 
ciated with  the  hypertonus  and  the  rigidity  there  is  a  slowness  of 
movement,  and  a  steadily  increasing  stiffness,  and  also  retardation 
of  the  motor  and  ideational  impulses. 


Fig.  322. — Attitude  of  paralysis  agitans  xjatient.      (Tilney.) 

The  muscular  rigidity  varies  w^idely  in  its  situation  at  the  beginning. 
Practically  the  symptoms  first  become  manifest  on  one  side  of  the 
body,  and  the  severity  of  the  symptoms  usually  predominates  on  one 
side,  it  may  be,  for  years.  In  the  well-developed  syndrome  the  rigidity 
involves  the  muscles  of  the  neck  and  trunk;  particularly  the  patient 
assumes  the  bent-over  attitude,  such  as  one  naturally  assumes  when 
shivering  from  the  cold  and  the  face  is  mask-like  (corrugators)  and 
staring,  the  eye  muscles  also  sharing  in  the  rigidity  with  Stelwag's 
sign. 

Whereas  the  muscles  of  the  neck  and  back  are  most  affected,  almost 
any  group  of  muscles  may  be  involved.    The  arms  and  legs  are  almost 

'  fur§tner,  et  ai,;  see  Ziiigerle, 


PARALYSIS  AGITAKS  GROUP  561 

always  implicated,  and  so  also  are  the  muscles  of  the  face.  Occasion- 
ally there  is  ptosis,  or  the  patients  open  their  eyes  with  difficulty  after 
closing  them.  One  of  us  has  seen  this  as  an  initial  symptom.  The 
patients  read  with  difficulty  because  of  the  stiffness  of  the  ocular 
movements.  Ocular  palsies  may  result — pseudo-ophthalmoplegias. 
Slow  pupillars'  reactions  are  occasionally  found.  The  pharAiigeal  and 
larsTigeal  muscles  being  involved,  as  others  in  the  body,  results  in 
slow,  difficult  speech,  becoming  fainter  and  fainter  as  the  years  go 
by,  until  finally  the  patient,  in  addition  to  being  unable  to  move,  to 
dress  himself,  eat  without  help,  finally  is  unable  to  talk  or  to  swallow. 

The  h^-pertonus  and  rigidity,  however,  is  not  associated  with  the 
usual  increased  reflex  signs  of  pyramidal  tract  involvement,  the 
reflexes  are  either  normal,  or  only  slightly  exaggerated,  no  clonus, 
Babinski,  Oppenheim,  etc.,  and  the  contractures  may  be  easily  over- 
come by  passive  movements,  in  marked  contrast  to  the  contractures 
of  psychomotor  cortical  origin.  The  muscular  power  is  also  not  so 
involved,  the  patients  show  muscular  weakness,  but  not  paralysis. 
There  is  a  striking  contrast  between  the  strength  of  active  movements 
and  that  of  resistance  movements.  The  former  are  weak,  the  latter 
rarely  less  than  normal. 

A  few  cases  of  extensor  rigidity  are  recorded,  but  the  involvement  is 
predominantly  of  the  flexor  muscles. 

With  the  rigidity  there  is  a  feeling  of  tension  which  the  patients 
dislike  usually  much  more  than  the  almost  universal  tremor.  This 
causes  them  to  feel  as  though  they  were  bound.  Their  motor  impulse 
seems  interfered  with.  This  may  even  involve  their  urination  and 
defecation,  and  their  deglutition. 

Attitude. — This  is  that  of  a  decerebellate  rigidity  in  contrast  to  a 
decerebrate  rigidity. 

Tremor. — In  the  majority  of  the  cases  this  objective  sign  seems  to  be 
the  first,  although,  in  reality,  vasomotor  signs  precede  as  a  rule.  It 
was  the  symptom  first  noted  by  Galen.  It  is  characterized  by  its 
uniformity  and  steadily  increasing  severity,  both  in  point  of  advance 
of  the  disease,  and  also  during  the  movements  themselves.  It  is  a 
tremor  that  Franciscus  Sylvius  first  noted  way  present  while  the 
limb  was  at  rest.  It  ceases  during  mov^ement,  especially  if  the  move- 
ment is  rapid,  and  in  the  beginning  of  the  disease.  In  the  later  stages 
it  becomes  continuous,  and  some  patients  with  paralysis  agitans  show 
some  intention  tremor.  Again,  a  certain  number  of  patients  show 
little  or  no  tremor. 

The  tremor  is  characterized  by  the  uniformity  of  its  excursions, 
which  are  at  first  small,  slow,  and  rhythmical.  They  average  about 
three  to  five  in  a  second,  according  to  the  muscles  involved.  Tremor 
in  the  muscles  of  the  thumb,  which  is  often  an  early  sign,  gives  rise  to 
the  well-known  "pill-rolling"  movements.  Similarly,  one  has  the 
movement  of  "beating  the  drum,"  and  other  attitude  types  when  the 
larger  muscles  of  the  arms  are  implicated.  The  muscles  inxolved  in 
the  early  tremors  vary  considerably;  usually  the  upper  extremity  is 
36 


562     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

involved  before  the  lower,  the  hand  particularly;  but  with  the  prog- 
ress of  the  disease  the  tremor  tends  to  become  widespread,  almost 
universal;  most  diverse  localizations  are  on  record,  anything  is  to  be 
expected.  There  is  no  great  preponderance  of  one  hand  over  the  other, 
although  there  is  a  marked  tendency  to  disproportion  in  the  severity, 
and  a  hemiplegic  type  of  onset  and  persistence  is  frequent,  if  not 
characteristic.     Monolimbic   types   are   encountered. 

At  first  the  tremor  is  absent  during  sleep,  but  in  the  advanced 
stages  it  frequently  persists  and  constitutes  one  of  the  factors  in 
sleeplessness  which  finally  exhausts  the  patient. 

Motion  tends  to  diminish  the  tremor,  as  also  does  attention;  emo- 
tional disturbances  and  cold  increase  it  markedly.  Grasping  the 
tremulous  member,  touching  it,  or  changing  its  position  results  in  a 
temporary  cessation  of  the  tremor.  The  restlessness  of  these  patients 
is  largely  dependent  upon  the  constant  and  continual  shifting  of  the 
body,  in  order  to  obtain  comfort,  i.  e.,  before  they  become  too 
rigid. 

Statistical  studies  show  that  tremor  may  be  absent  in  as  high  as 
20  per  cent,  of  the  cases — a  much  greater  proportion  than  show  absence 
of  rigidity,  which  also  may  be  absent  or  scarcely  noticeable.  These  are 
variations,  in  precise  pathway-blocking,  the  lesions  showing  slight  vari- 
ability in  localization. 

Disturbajices  of  EquUihrium. — Propulsion,  which  apparently  was 
first  noted  by  Gaubius  (1751),  is  one  of  the  cardinal  symptoms  in 
Parkinson's  original  definition.  The  patient  on  walking  tends  to  fall 
fonvard,  and  in  his  effort  to  keep  his  equilibrium  goes  faster  and  faster, 
until  he  either  falls  or  stops  himself  by  grasping  a  support.  Latero- 
pulsion  and  retropulsion  are  also  present.  These  are  due  either  to 
the  stiffness  with  the  slowness  of  muscular  movement,  or  to  a  central 
disturbance  of  equilibrium,  which  latter  is  perhaps  the  preferable 
explanation,  since  exquisite  examples  of  gait  disturbances  are  known 
without  any  marked  stift'ness  or  rigidity.  In  a  few  cases  the  loss  of 
equilibrium  in  one  direction  alters  to  that  of  another  during  the  course 
of  the  disease. 

Secretory,  Vasomotor,  Trophic  Symptoms  of  the  Vegetative  Level. — 
These  make  up  the  second  category  of  symptoms  almost  universally 
found  to  a  greater  or  less  degree  in  paralysis  agitans.  As  noted,  many 
are  prodromal  symptoms.  What  relation  these  symptom  s,  all  of  which 
have  some  definite  relation  to  the  vegetative  nervous  structures,  pos- 
sibly at  lenticular  levels,  bear  to  the  almost  universally  present  arterio- 
sclerosis is  not  yet  apparent. 

The  most  important  of  the  secretory  changes  are  increased  perspira- 
tion— sometimes  unilateral,  increased  salivation — one  of  the  most 
distressing  of  the  symptoms — and  polyuria,  with  occasionally  diarrhea. 

Among  the  vasomotor  changes  are  rushes  of  blood  to  the  head, 
reddening  of  the  face,  cyanosis,  tachycardia,  acroparesthesia,  with 
hot  and  cold  spots,  alteration  of  temperature — sometimes  unilaterally 
disposed,  and  dermographia,  which  is  almost  constant, 


PARALYSIS   AGITANS  GROUP  563 

Trophic  changes  m  the  skin,  such  as  atrophy,  thickening,  edema,  are 
among  the  rarer  findings. 

Psychic  Disturbances. — These  probably  do  not  constitute  an  essen- 
tial part  of  the  disorder,  but  represent  almost  normal  psychological 
reactions  to  a  most  distressing  and  hopeless  situation.  Depression, 
anxiety,  ideas  of  self-destruction,  scornfulness,  savage  raillery,  sarcastic 
pessimism,  euphoric  compensation  and  sublimation,  resignation  to  the 
will  of  God,  etc.,  these  are  but  a  few  of  the  innumerable  attitudes  which 
these  patients  show  at  one  or  another  time  during  their  long  period  of 
almost  unbearable  suffering. 

Parkinson  said  the  "senses  and  the  intellects  are  not  impaired," 
and  intelligence  tests  bear  this  out.  In  some  patients  one  naturally 
finds  a  senile  deterioration,  and  for  most,  in  the  later  stages,  the  mental 
signs  of  an  arteriosclerotic  deteriorating  process  are  present.  Acute 
exhaustion,  delirious  states,  often  close  the  sad  chapter;  but  these 
are  not  a  part  of  the  paralysis  agitans. 

Sensory  Symptoms. — Tactile,  thermal  or  pain  disturbances  are  not 
definite.  They  are  not  often  striking  features  of  the  disorder.  The 
early  pains  ar^  usually  fugitive,  and  apart  from  the  dull  and  most 
oppressive  sensation  due  to  the  tension  and  stiffness,  pain  is  not 
prominent.  Irregular  anesthesise,  hyperesthesise,  paresthesife  are 
frequently  found,  but  are  so  inconstant  that  one  can  say  that  definite 
sensory  disturbances  rarely  belong  to  the  paralysis  agitans  picture. 
When  present  in  striking  fashion  they  are  probably  due  to  lenticular 
or  thalamic  involvement  of  deep  sensibility  pathways. 

The  reflexes  a:re  not  markedly  disturbed.  Considerable  variation 
exists,  but  there  is  no  constant  picture  as  yet  known  which  is  pathog- 
nomonic of  the  condition.  Reflex  activities  due  to  pyramidal  tract 
involvement  are  occasionally  found.  Increased  knee-jerks,  clonus,  and 
Babinski  phenomena  are  at  times  found,  but  they  are  not  constant, 
and  represent  occasional  rather  than  essential  features. 

Loss  of  the  Achilles  reflex  is  a  not  infrequent  symptom  the  signifi- 
cance of  which  is  as  yet  not  definitely  placed. 

The  abdominal,  cremasteric,  epigastric,  and  anal  reflexes  are  not 
involved. 

Other  clinical  findings  are  inconstant.  The  blood  practically  shows 
nothing,  some  anemia  at  times,  but  nothing  striking;  the  cerebro- 
spinal fluid  is  practically  negative.  The  urine,  apart  from  a  frequent 
polyuria,  shows  no  quantitative  or  qualitative  anomalies,  beyond  the 
excessive  phosphate  elimination,  which  is  indicative  of  the  exhaustion. 

Course  and  Progress. — Rudimentary  forms  are  not  unknown.  Many 
senile  patients  show  conditions  closely  approaching  the  milder  grades 
of  paralysis  agitans,  and  intermediary  stages,  with  muscular  stifl'ness, 
slowness  of  movement,  retardation  of  motor  impulses,  etc.,  are  not 
infrequent.  Certain  stationary  cases,  non-progressing  for  twenty -five 
years,  are  also  knowii,  and  infrequently  patients  make  partial  recov- 
eries. These  are  possibly  syphilitic  cases  showing  the  syndrome. 
But  the  usual  course  of  the  more  frequent  arteriosclerotic  cases  is  a 


564     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

long,  slow,  and  gradual  progression,  lasting  over  many  years  with 
annoyance,  inconvenience,  discomfort,  distress,  and  agony  until  life 
becomes  a  burden.  Remissions  and  exacerbations  belong  to  almost 
every  case. 

Patients  with  little  hypertonus  and  muscular  rigidity  seem  to  pro- 
gress less  rapidly,  and  many  cases  beginning  in  younger  individuals 
do  fairly  well.  Emotional  disturbances  act  badly,  almost  invariably 
causing  marked  progress  of  the  disease. 


,  > 


1/'     *■' 


V-V 


'■■A  / 


'^  fr' 


Fig.  323. — Primary  atrophy  of  the  pallidal  system.  Progressive  atrophy  of  the 
globus  pallidus.  Juvenile  paralysis  agitans.  Section  through  the  globus  pallidus,  show- 
ing atrophy  and  diminution  ot  the  large  motor  cells  of  the  globus  pallidus,  with  increase 
of  glia  nuclei.     (Ramsay  Hunt.) 

The  majority  of  the  patients  get  worse  gradually.  The  increasing 
weakness  and  stiffness  limits  them  more  and  more  in  their  work,  until 
that  becomes  impossible.  Then  the  walking,  to  which  they  are  uncon- 
sciously attracted,  becomes  more  and  more  difficult.  They  are  then 
confined  to  their  chairs  for  a  few  years,  or  to  bed,  and  finally  come  to 
be  helpless  invalids,  who  must  be  fed,  turned  over,  attended  to  like 
living  rigid  statues,  which  in  the  presence  of  relatively  intact  intelli- 
gence, though  robbed  of  the  power  of  expression,  reading,  writing, 


PARALYSIS   AGITANS  GROUP  565 

even  pantomime,  constitutes  one  of  the  most  ghastly  afflictions  in  the 
entire  realm  of  nervous  disorders. 

Decubitus,  pneumonia,  exhaustion  delirium,  and  starvation,  are  the 
usual  precursors  to  the  end  of  a  disorder  whose  prognosis  is  bad. 
Recoveries  there  are  none,  although  stationary  cases  are  occasionally 
seen. 

Differential  Diagnosis. — ^The  diagnosis  of  the  classical  Parkinsonian 
t^-pe  is  rarely  difficult.  The  attitude,  gait,  facial  expression,  and  tremor 
are  so  characteristic  as  to  stamp  the  patient. at  once.  In  the  initial 
period,  before  the  stiffness,  tremor,  pulsions,  etc.,  have  developed,  the 
diagnosis  may  be  difficult,  but  after  its  classical  development  it  cannot 
be  mistaken  for  anything  else. 

Hysteria  sometimes  comes  into  review,  but  here  the  character  of 
the  hypertonus  is  quite  different,  the  tremor  is  rarely  classical,  and 
can  be  more  readily  influenced  by  diversion  and  distraction.  The 
exaggeration  of  a  paralysis  agitans  s\Tnptom-picture  is  characteristic 
of  the  hysterical  type. 

^Multiple  sclerosis  of  the  cerebellar  type  frequently  shows  the  classical 
paralysis  agitans  picture,  plus  the  evidence  of  pyramidal  tract  involve- 
ment, nystagmus,  bulbar  speech,  etc.,  of  this  disease.  It  is  usually 
present  in  younger  individuals.  Some  of  the  so-called  juvenile  Park- 
inson cases  are  cerebellar  types  of  multiple  sclerosis. 

The  chief  development  of  recent  years  have  been  toward  the  sepa- 
ration of  juvenile  types  of  the  syndrome  as  an  expression  of  an 
involvement  of  the  globus  pallidus  (Hunt),  Cecile  Vogts  syndrome, 
characterized  by  a  spastic  diplegia  wdth  pseudobulbar  palsy  and 
athetosis,  with  necrotic  lesions  in  the  caudate  nucleus  and  putamen; 
Wilson's  lenticular  degeneration  and  various  admixtures  of  these  syn- 
dromes. Their  discussion  opens  up  a  large  chapter  bearing  on  the 
distribution,  localization  and  dynamic  interrelationships  of  the  group- 
ing of  the  sensory,  motor  and  vegetative  pathways  in  the  basal 
ganglionic  synaptic  junctions.  This  is  too  detailed  and  complicated 
to  be  entered  into  here. 

Pathology  and  Pathogeny. — A  dogmatic  presentation  of  the  causes 
for  paralysis  agitans  is  not  yet  available.  The  trend  of  opinion  is 
that  it  represents  a  senile  or  presenile  degeneration  of  certain  mid- 
brain pathways,  and  these  are  mostly  concerned  within  the  cerebellar, 
thalamic,  and  lenticular  mechanisms.  Whether  they  are  confined  to 
the  midbrain  regions  is  not  proved — neither  are  these  mechanisms 
— but  the  evidence  points  in  that  direction.  The  increased  tonus 
resembles  cerebellar  and  not  cerebral  tonus.  The  rigidity,  attitude, 
slowness  of  motor  impulses,  tremor,  has  its  analogies  in  disorders  of 
the  frontocerebellar,  cerebello-rubro-spinal  and  thalamic  systems. 
Hunt^  would  localize  this  motor  part  of  the  syndrome  to  disease  of 
motor  cells  in  the  globus  pallidus,  a  view  closely  related  to  that  of 
Winkler  elaborated  in  Manschot's  thesis  (1904).    The  disturbances  of 

1  Huut,  J.  R.:  loc.  cit. 


566     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

equilibrium  are  suggestive  of  the  cerebellar  type.  The  vasomotor,  se- 
cretory, and  trophic  symptoms  represent  central  vegetative  disorders, 
which  are  referred  with  greatest  probability  to  those  hypothalamic 
nuclei  other  than  the  nuclei  which  are  known  to  be  the  synapses  of  the 
chief  sensory  pathways.  Thus  the  automatic  proprioceptive  tonus 
impulses  passing  over  impaired  cerebellar  pathways  are  not  counter- 
balanced by  the  influence  of  the  corticomedullary  pyramidal  systems. 
Because  of  the  lesions  in  the  globus  pallidus  with  the  degenerations  in 
the  ansa  lenticularis,  there  are  not  enough  centripetal  cortex  impulses 
to  act.  The  rigidity,  forced  attitude  of  the  head  and  trunk  possibly 
may  "be  so  interpreted. 

The  recent  review  of  Zingerle  (loc.  cit.),  Jelgersma,^  and  of  Hunt^ 
brings  these  features  to  the  fore.  Few  complete  series  of  microscopic 
sections  through  the  cerebellum  and  midbrain  region  have  as  yet 
been  studied,  loss  of  motor  cells  in  the  globus  pallidus  and  distinct 
atrophies  in  the  ansa  lenticularis,  in  thalamic,  and  lenticular  regions 
are  present  in  those  cases  studied  by  the  serial  section  method. 

The  muscular  hypotheses,^  which  are  many,  are  totally  inadequate, 
as  is  also  the  parathyroid  hypersecretion  theory  upheld  by  Lundborg. 
There  are  a  number  of  pathological  processes  operative.  Arteriosclero- 
sis is  the  chief  factor  thus  far  observed. 

Therapy. — Notwithstanding  the  gloomy  outlook,  much  can  be  done 
to  relieve  the  patients.  They  must  be  guarded  against  cold,  and  as 
far  as  possible,  from  emotional  disturbances  and  mental  and  physical 
strain. 

They  should  live  in  warm,  dry,  sunny  rooms  if  possible,  be  much 
in  the  open  air,  eat  a  full,  mixed  diet,  and  possibly  a  minimum  of 
purin-containing  substances  is  needed.  Alcohol  and  coffee  may  be 
used  in  moderation.  Tobacco  is  not  necessarily  taboo;  two  or  three 
cigarettes  or  cigars  a  day.  The  regimen  should  under  no  circumstances 
be  so  strictly  adhered  to  as  to  cause  the  patients,  already  suffering 
from  irritating  conditions,  to  become  further  annoyed  thereby.  Diet 
has  relatively  little  power  to  modify  the  trouble  and  fussy  dietaries 
are  superstitious  nonsense  for  the  most  part. 

The  greatest  relief  from  rigidity  comes  from  the  regular  use  of 
passive  movements.  The  Zander  apparatus  can  be  utilized.  Working 
with  carpentry  or  garden  tools  is  often  very  helpful.  Heat  is  grateful 
and  helpful  and  passive  motion  combined  with  warm  (not  hot)  baths 
is  particularly  gratifying.    A  few  patients  react  badly  to  baths. 

Such  attempts  at  occupation  therapy  must  be  carefully  dosed. 
Fatigue  must  be  avoided.  Five  to  ten  minutes  is  sufficient  in  the  early 
stages.    Such  therapy  is  solely  of  value  from  a  psychical  standpoint. 

Drug  therapy  is  at  times  of  doubtful  service  in  controlling  the 
tremor.  The  remedies  are  those  with  marked  action  on  overaffective 
autonomic  stimuli — notably  the  alkaloids  of  the  belladonna  group,  of 

'  Verb.  d.  Gesellschaft  deut.  Naturforscher,  1908,  80,  ii,  p.  383. 

2  Hunt,  J.  R.:  Brain,  1917,  complete  bibliography. 

3  Camp:    Jour.  Am.  Med.  Assn.,  1910. 


PROGRESSIVE  LENTICULAR  DEGENERATION  567 

which  hyoscine,  duboisme,  scopolamine  and  atropine  are  the  most 
available.  In  view  of  the  chemical  uncertamties  concerning  the 
alkaloids  of  this  group  one  should  obtain  good  products  and  try  the 
different  derivatives.    The  dosage  must  be  tested  with  each  case. 

The  analgesics,  particularly  in  combination  with  salicylates,  are  use- 
ful in  relieving  the  muscular  soreness  and  pain  of  tension — phenacetin, 
aspirin,  acetanilid  combinations,  etc.  * 

For  sleep,  the  best  form  of  hypnotic  is  not  yet  known.  Bromides 
are  at  times  available,  at  other  times  the  alcohol  hypnotics — trional, 
sulphonal,  again  urea  substitutes,  as  veronal — are  useful.  One  should 
avoid  morphin  as  much  as  possible,  particularly  bearing  in  mind  that 
the  emotional  h^'peractivity  may  have  little  real  feeling  behind  it. 
It  is  often  mostly  mimicry  which  is  uncontrollable  because  of  the 
motor  defect. 

PROGRESSIVE  LENTICULAR  DEGENERATION. 

Wilson^  has  described  this  syndrome,  to  which  he  applies  the  term 
progressive  lenticular  degeneration. 

The  disease  is  familial,  in  the  sense  that  frequently  more  than  one 
member  of  a  family  is  affected  with  it,  but  it  is  not  hereditary;  it  may 
also  occur  sporadically.  It  occurs  in  young  people,  either  in  an  acute 
or  a  chronic  form.  As  far  as  is  knowTi  it  is  progressive  and  invariably 
fatal,  its  duration  ranging  from  six  months  or  a  year  to  as  long  as 
four  or  five  years. 

S3nnptoms. — The  clinical  symptoms  consist  of  involuntary  move- 
ments, nearly  always  a  bilateral  tremor  of  both  upper  and  lower 
extremities,  the  head  and  trunk  also  being  sometimes  involved.  The 
tremor  is  usually  rhythmical  but  occasionally  irregular,  and  increas- 
ing with  volitional  movement;  there  is  pronounced  spasticity  of  the 
limbs  and  of  the  face,  the  latter  being  usually  set  in  a  spastic  smile, 
while  in  the  latter  stages  contractures  of  the  limbs  develop;  there  is 
dysphagia  and  dysarthria,  the  latter  eventually  degenerating  into 
complete  anarthria;  there  is  also  spasmodic  laughing  and  emotional- 
ism. As  a  result  of  the  extraordinary  degree  of  stiffness  of  the  muscu- 
lature there  is  considerable  difficulty  in  maintaining  equilibrium. 
Little  or  no  true  paresis  or  paralysis  occurs,  however,  inasmuch  as 
most  ordinary  movements  can  be  executed,  although,  it  may  be, 
slowly  and  feebly.  In  spite  of  the  great  degree  of  motor  weakness  and 
helplessness,  in  a  pure  case  the  abdominal  reflexes  are  present  and  a 
double  flexor  response  is  obtained.  In  other  words,  this  affection, 
where  it  occurs  in  an  uncomplicated  form,  is  an  extrapyramidal  motor 
disease,  the  importance  of  which  is  apparent  by  reason  of  the  light  it 
sheds  on  such  a  process  as  paralysis  agitans. 

Pathology. — The  chief  j)ath()logical  feature  of  the  disease  from  the 
neuroanatomical  point  of  view  is  a  bilateral  symmetrical  degeneration 

•  Progressive  Lenticular  Degeneration.  A  Familial  Nervous  Disease  Associated  with 
Cirrhosis  of  the  Liver,  Brain,  1913.     Tilney:  Neurological  Bulletin,  1918. 


568     PARALYSIS  AGITANS,  CHOREA   AND  RELATED  SYNDROMES 


of  the  piitamen  and  globus  pallidus,  in  particular  the  former.  This 
degeneration  is  thought  by  Wilson  to  be  a  sequel  to  the  selective 
operation  of  some  toxic  agent  on  the  cells  and  fibers  of  the  putamen 
and  lenticular  nucleus  generally.  The  caudate  nuclues  is  often  some- 
what degenerated,  but  never  to  the  same  extent,  while  other  large 

collections  of  gray  matter  in  the 
immediate  neighborhood  of  the 
lenticular  nucleus — e.  g.,  the  optic 
thalamus,  which  has  partially  the 
same  blood  supplv — are  not  affected 
at  all  in  a  pure  case  unless  it  be 
indirectly,  and  to  a  very  slight  ex- 
tent. A  constant  essential,  and  in 
all  probability  primary  feature  of 
the  pathology  of  the  disease  is 
hypertrophy  of  the  liver,  not 
syphilitic  or  alcoholic;  it  is  multi- 
lobular or  mixed  in  type,  always 
pronounced,  but  presenting  a 
varying  pathological  picture  of 
necrosis,  fatty  degeneration,  and 
regeneration. 

As  yet  no  valid  correlations  have 
been  established  between  these 
isolated  happenings.  Our  attitude 
is  that  this  disorder  is  a  type  syn- 
drome of  disordered  vegetative 
pathways,  chiefly  of  those  con- 
cerned in  the  dynamic  maintenance 
of  proper  hepatic  function.  The 
synapses  of  the  higher  vegetative 
(motor)  pathways  are  located  we 
believe  in  the  corpora  striata.  The 
analysis  of  these  pathways  is  still  in 
its  infancy.  (See  discussion  of 
vegetative  pathways  in  section  on 
Visceral  Neurology).  This  syn- 
drome as  well  as  that  of  paralysis 
agitans  and  related  striated  syn- 
dromes offers  excellent  material  for 
the  analysis  of  these  higher  vege- 
tative pathways  and  synapses.^ 


eration. 


aticular  degen- 
(Tilney.) 


DYSTONIA  MUSCULORUM  DEFORMANS. 

Under  this  term  Oppenheim  includes  a  peculiar  syndrome  first 
called  attention  to  bv  Ziehen  as  a  tonic  torsion  neurosis.     Flatau  and 


^  See  Malone:    Studies  on  the  Vegetative  Synapse.s  of  the  Diencephalon. 


DYSTONIA  MUSCULORUM  DEFORMANS 


569 


Sterling  term  it  a  progressive  torsion  spasm.  It  is  preeminently  a 
disorder  of  children,  most  of  the  observed  patients  having  been  between 
eight  and  fourteen  years,  and  almost  all  of  the  Jewish  race.  Apparently 
there  are  no  sex  differences.  Oppenheim's  cases  did  not  lead  him  to 
any  light  on  the  disorder  as  regards  heredity.  Three  of  Ziehen's  cases 
were  brothers  and  sisters.  Exciting  or  other  causative  factors  are 
not  known. 

The  illness  comes  on  apparently  gradually  and  subtly  in  one  arm 
or  in  both  arms,  occasionally  first  in  the  legs  or  in  the  spine,  but  in 
progression.  The  patient  twists  the  spine  in  a  peculiar  fashion,  tilting 
the  pelvis,  and  bringing  about  a  marked  torsion  of  the  entire  vertebral 


Fig.  325  Fig.  326 

Figs.  325  and  326. — Attitudes  in  dystonia  musculorum  cases.     (Flatau.) 

axis,  with  lordosis,  scoliosis,  and  tilting  of  the  pelvis,  the  arms  and 
legs  moving  in  a  peculiar  manner.  The  mode  of  progression  at 
times  resembles  that  of  astasia  abasia.  The  whole  musculature,  when 
in  action,  is  extremely  stiff  and  h^•]Dertonic;  when  at  rest,  h\']3otonia 
is  apparent.  In  the  general  attitude  of  the  patient  in  walking  one  is 
inclined  to  regard  the  whole  matter  as  one  of  extreme  suggestibility; 
a  psychogenic-hysteriform  affair.  The  movements  of  the  hip  are  very 
typical.  It  is  tilted  or  thrust  forward  or  backward  in  an  awkward 
manner.  Oppenheim  has  likened  it  to  a  dromedary  in  some  of  the 
positions  assumed.  Walking  seems  to  fatigue  the  patients  greatly. 
They  perspire,  get  red,  and  show  signs  of  fatigue,  getting  out  of  breath, 
and  one  of  our  (J.)  patients  grunted  involuntarily.     One  of  Oppen- 


570     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

heim's  patients  could  walk  backward  better  than  he  could  walk  for- 
ward. On  sitting  down  or  lying  down  the  movements  cease  (Bieling's 
case),  or  are  much  reduced  in  frequency  and  in  clownishness.  Ziehen's 
cases  were  at  times  continually  in  motion,  and  had  to  be  kept  in  a 
special  bed,  against  which  they  frequently  bruised  themselves  by  their 
impulsive  movements.  The  peculiar  activities  come  into  play  as  soon 
as  there  is  an  attempt  to  make  any  voluntary  movement.  Writing 
becomes  difficult  or  impossible.  There  is  no  paralysis.  Oppenheim 
speaks  of  a  dystonia,  Ziehen  of  a  hypertonia.  The  movements  are  not 
athetoid  nor  choreic.  They  are  wide,  irregular,  and  yet  partake  of 
the  nature  of  both,  and  at  times  resemble  those  of  Huntington's  chorea. 

Tonic  and  clonic  extension  of  the  muscles,  particularly  of  the  biceps 
and  rotators  of  the  thigh,  were  marked  in  Oppenheim's  cases.  Thus 
there  is  an  alternation  in  tonicity  of  the  muscle. 

The  knee-jerks  are  apt  to  be  much  diminished,  coming  out  in 
Oppenheim's  cases  only  by  Jendrassik  reinforcement. 

The  relationships  are  difficult  to  state.  Hysteria  should  be  excluded. 
Double  infantile  athetosis  shows  a  similar  picture.  These  cases  have 
been  studied  especially  by  Lewandowsky.^  Cecile  Vogt  and  Oppen- 
heim have  reported  on  infantile  pseudobulbar  palsy,  which  also  is 
to  be  considered  in  this  connection.^  The  patients  apparently  hold 
their  own  for  some  time,  and  but  little  is  known  of  the  development 
of  the  disorder.  As  yet  no  pathological  reports  are  available.  No 
known  method  of  treatment  seems  useful.  Suggestion  is  of  no  value, 
nor  are  bromides.    The  movements  cease  during  sleep. 


THE  CHOREAS. 

The  somatic  choreas  are  due  to  definite  but  usually  recoverable  brain 
conditions,  chiefly  located  in  and  about  the  cerebral  and  cerebellar 
cortex  and  the  static  motor  pathways.  Meningitides,  encephalitides, 
new  growths,  severe  concussions,  hemorrhages,  toxemias,  and  marked 
fatigues,  etc.,  are  among  the  causative  factors.  They  are  a  vast 
conglomeration  of  conditions,  certain  trends  of  which  have  been 
separated  out  under  a  variety  of  types. 

The  detailed  history  of  this  sorting  process  would  lead  too  far.  The 
beginning  of  the  present-day  groupings  are  to  be  found  in  the  classic  of 
Germain  See  (1S50).  The  chief  trends  center  about  the  study  of  the 
movements  which  are  present.  These  are  of  three  main  forms:  (a) 
spontaneous  movements,  (b)  coordination  disturbances  and  (c)  dis- 
turbances of  purposeful  movements.  In  most  choreas  the  three  forms 
are  present  but  in  varying  proportions. 

The  chief  nosological  entities  which  have  been  created  are: 

1.  Chorea  Minor  or  Sydenham's  Chorea. — The  most  widespread  and 
frequent  of  the  trends,  usually  found  in  children  or  young  adults, 

1  Deutsche  Zeitsch.  f.  Nervenheilk.,  xxxix,  1908. 

2  Journal  f.  Neurologie,  xviii,  1911. 


THE  CHOREAS  571 

which  is  usimlly  recoverable  and  hence  erroneously  spoken  of  as 
functional.    Only  psychogenic  choreas  may  thus  be  termed. 

2.  Chorea  Chronica. — A  stationary  form  of  the  former,  or  when 
occurring  in  old  age,  chorea  senilis. 

3.  Chorea  Huntingfonii. — A  chronic  progressive  type  with  certain 
definite  hereditary  factors  and  one  showing  a  vast  variety  of  other 
choreic  anomalies  in  the  "  non-Huntington  chorea"  members, 

4.  Chorea  degenerans  of  Brissaiid,  occurring  as  a  result  of  presenile 
breakdown  in  unstable  neuropathic  individuals. 

5.  Chorea  electrica  of  Diibini,  an  acute,  usually  fatal  disturbance, 
often  occurring  with  epileptiform  attacks,  paralysis  and  death. 

6.  Chorea  electrica  of  Bergeron  and  Henoch,  occurring  in  young  per- 
sons, seven  to  fourteen  years,  with  rhythmic  lighting-like  movements 
of  the  neck,  shoulders,  and  upper  arms.  It  has  allies  in  certain  epileptic- 
like choreas. 

7.  Chorea  epileptica,  continuous  with  the  preceding  or  related  to 
cortical  epilepsies.     (See  Epilepsy.) 

8.  Choreas  of  general  paresis,  in  which  spontaneous  choreiform 
movements  occur.     (See  Paresis.) 

9.  Choreas  of  many  Psychoses. — IMotility  psychoses  of  Wernicke, 
chiefly  schizophrenic  individuals  in  whom  Kleist  has  endeavored  to 
show  an  involvement  of  the  cerebro-rubro-cortical  tracts. 

10.  Choreas  of  Congenital  or  Infantile  Cerebral  Palsies. — (See  Hemi- 
plegia, Thalamic  Syndrome.) 

11.  Chorea  Postapoplectica.  Posthemiplegic  Choreas. — (See  Hemi- 
plegia). ■  .  ,  . 

12.  Chorea  Thalamica. — (See  Thalamic  Syndrome.) 

13.  Choreas  dne  to  Disorder  Involving  the  Superior  Cerebellar  Pedun- 
cles.— -(See  Cerebellar  Syndromes.) 

14.  Choreas  of  Cerebellar  Origins. — (See  Cerebellar  S\Tidromes.) 

15.  Chorea  tabica  in  tabes  with  choreic  crises  with  or  without  pain. 

16.  Psychogenic  Choreas. — (See  Psychoneuroses,  Hysteria,  Com- 
pulsion Neurosis.) 

Chorea  Minor  (Sydenhains  Chorea,  St.  Vitus'  Dance)  is  the  most 
frequent  of  these  disturbances  of  spontaneous,  purposeful  and  coordi- 
nating movements,  which,  occurring  in  children,  usually  recovers. 

Infection  and  maldevelopment  are  the  most  frequent  etiological 
factors.  The  most  widespread  infecting  agents  are  various  strep- 
tococci (tonsillitis),  infectious  diseases,  measles,  whooping-cough,  etc., 
hence  the  frequent  complications  of  infectious  arthritides,  endocarditis, 
with  a  mild  meningitis  which  is  usually  present. 

Girls  are  apparently  less  resistant  following  these  infections,  and 
hence  show  a  higher  i)ercentage  of  choreic  attacks. 

Symptoms. — These  show  considerable  variation,  ranging  from  slight 
motor  inirest  and  irritability  to  marked  motor  disturbances,  with 
corresponding  modifications  of  conduct  and  emotional  response. 
The  latter  at  times  are  so  severe  as  to  constitute  a  psychosis  (choreic 
mental  disease,  q.  v.).     The  motor  symptoms  are  best  grouped  as 


572     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

pyramidal  or  extrapyramidal  tract  and  cerebellar  disturbances,  either 
or  both  occurring  in  most  patients.  They  are  the  results  of  definite 
irritations,  occasionally  of  defect  (diaschisis)  lesions.^ 

The  spontaneous  movements  are  quick  and  show  comparatively  wide 
excursions.  In  the  distal  extremities  cramps  of  single  muscles  or  groups 
of  muscles  occur,  with  complete  rest  following  the  movements.  There 
is  great  irregularity  in  the  affected  groups;  there  will  be  one  or  two 
movements,  which  are  followed  by  opposite  muscle  action. 

In  mild  cases  these  movements  are  limited  to  the  face  and  to  single 
muscles.  In  the  more  severe  ones  the  entire  body  musculature  is 
involved. 

The  arms  are  flung  about,  the  legs  are  wobbled  and  pulled,  walking 
is  impossible,  the  larynx,  lips,  and  eyes  are  in  activity.  The  patients 
gasp,  snort,  and  groan.  In  the  mild  cases  the  movements  may  cease 
during  sleep;  in  the  more  severe  ones  the  movements  are  continuous. 

Any  sensory  stimulus  may  increase  these  spontaneous  movements. 
They  do  not  behave  like  willed  movements.  Hence  extrapyramidal 
systems  are  also  involved. 

Pyramidal  Trad  Disturbances. — Certain  choreic,  jerky  movements 
are  observed  apart  from  the  more  usual  ataxias  and  incoordinate  move- 
ments. The  latter  diminish  with  rest,  quiet,  and  relaxation;  the 
former  do  not  seem  to  diminish  as  muscle  activity  is  withdrawn. 

A  group  of  minor  signs  appear  on  close  examination.  One  of  these  is 
the  Babinski  hand  sign  (dysmetria;  see  Fig.  333).  When  the  choreic 
patient  slowly  raises  the  arms  in  front  of  the  body,  palm  down,  one 
side,  that  most  affected,  has  a  tendency  to  sag.  Or  if  the  hands  are 
hanging  by  the  side,  the  more  affected  hand  shows  a  position  half-way 
between  pronation  and  supination,  whereas,  since  the  normal  muscle 
tonus  is  greater  in  the  pronators,  the  more  healthy  side  is  held  more 
pronated.  This  is  in  accord  with  the  general  tendency  for  one-half 
of  the  body  to  be  more  affected  than  the  other.  The  affected  side  is 
hypotonic;  the  shoulder  droops  more.  There  is  apt  to  be  exaggerated 
flexion  or  extension  of  the  arms.  In  the  lower  extremities  hyper- 
flexions  of  the  leg  on  the  thigh  occur.  Since  h^'potonicity  is  char- 
acteristic of  underdeveloped  psychomotor  integration,  younger 
children  do  not  bring  these  contrasts  into  relief. 

If  the  patient  lies  flat  upon  the  back  and  attempts  to  sit  up,  the 
arms  being  folded,  the  leg  that  is  most  affected  is  flexed  on  the  thigh 
and  raises  from  the  bed,  the  healthy  side  remains  flat.  (Babinski 
thigh  sign — Hoover).  Similarly,  if  the  patient  lying  on  the  back  raises 
the  legs  simultaneously,  the  sound  side  rises  higher  than  the  other, 
or  if  one  leg  is  raised  and  then  the  other,  the  sound  side  is  raised  higher 
and  the  choreic  side  also  falls  more  readily. 

The  Hoover  procedure  by  measuring  the  pressure  of  the  leg  on  the 
heel  during  the  movements  just  mentioned  shows  diminished  pressure 
on  the  weak  side.     (See  sjTiergistic  tests  in  chapter  on  Examinations.) 

1  Forster:   Volkmann  klin.  Vort.,  382. 


THE  CHOREAS  573 

Forster  showed  that  in  choreics,  particularly  in  the  more  unilateral 
cases,  the  closing  of  the  fist  of  the  sound  side  called  forth  an  associated 
closure  of  the  fist  of  the  affected  side,  but  not  vice  versa.  Similar 
associated  abductor  or  adductor  movements  usually  occur  in  the  lower 
limbs. 

The  Babinski,  Oppenheim,  Gordon,  and  Chaddock  great-toe  exten- 
sion sign  is  very  frequent  in  chorea,  and  should  be  looked  for. 

Striimpell's  contraction  of  the  tibialis  anticus  occurs  when  the 
patient,  lying  on  the  back,  attempts  to  flex  the  leg  on  the  thigh  against 
passive  resistance  applied  to  the  thighs.  The  foot  assumes  the  equino- 
varus  position. 

The  tendon  reflexes,  patellar  and  Achilles,  are  apt  to  be  variable  and 
often  delayed. 

These  signs  are  all  suggestive  of  mild  t^'pes  of  hemiplegia,  and  have 
been  C3llected  by  Lhermitte  under  the  title  of  the  "Little  Signs  of 
Hemiplegia."^  Some  one  or  all  may  be  present  in  even  mild  cases  of 
chorea,  especially  when  the  disturbance  is  at  its  height  and  particularly 
in  the  severe  infectious  t^'pes.  Their  disappearance  often  takes  place 
with  the  stage  of  recovery.  Mild  cases  may  show  only  the  most 
passing  signs,  or  very  mildly  developed  ones. 

Cerebellar  Sigm. — These  are  chiefly  adiadokokinesia  and  asynergia. 
The  former  is  frequently  found,  especially  on  the  more  hypotonic 
side.    It  is  sometimes  complicated  by  the  choreic  movements.^ 

Asynergia  is  the  usual  choreic  type  of  movement.  There  is  a  jerky, 
irregular  movement  of  the  muscles  which  fail  to  perform  well-adapted 
movement.  TJius  the  patients  drop  things,  lurch,  stumble  or  fall, 
spill  their  food,  or  speak  in  a  jerky,  at  times  mumbling,  manner.  They 
are  incapacitated  from  writing,  playii^g  on  the  piano,  or  for  making 
any  finely  adaptive  movements.  The  finger-nose  and  finger-finger 
tests  show  this  asynergia  by  the  overshooting  of  the  mark,  pseudo- 
ataxia.  Attempting  to  grasp  an  object,  the  choreic  opens  the  affected 
hand  over-widely;    the  pencil  test  also  shows  similar  overshooting. 

Leukocytes  are  usually  increased  in  the  cerebrospinal  fluid.  (See 
Meningeal  Inflammation.) 

Etiology  and  Pathogenesis. — A  too  narrow  view  of  the  causation  of 
chorea  has  prevailed.  While  many  patients  undoubtedly  develop  a 
chorea  following  streptococcic  or  other  infection,  this  whole  group 
should  be  looked  at  from  the  developmental  point  of  view  as  well.  This 
renders  fatigue  factors  comprehensible. 

The  so-called  hereditary  factor  is  probably  dependent  upon  an 
inferior  or  slowly  developing  psychomotor-cerebellar  integration.  In 
certain  patients  the  rapidly  developing  body  calls  for  a  higher  grade 
of  motor  adaptation  than  the  developing  motor  integration  can  sub- 
sen^e.  This  is  seen  normally  in  the  cerebellar  static  apparatus  in  what 
is  so  widely  called  the  awkward  age,  or  in  the  "pu})py"  stage,  where 
the  analogues  to  mild  choreic  affections  are  obvious.    Chorea  may  thus 

'  JellifiFe:    Little  signs  of  Hemiplegia,  Post-Graduate  Medical  Journal,  1912. 
■  Grenet  et  Loubet:    Rev.  Xeur.,  December  15,  1912,  p.  632. 


574     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

develop  in  adolescents  from  the  mildest  of  infections  or  even  from 
excess  of  motor  activity.    Thus  chorea  may  be  a  fatigue  symptom. 

Ancestral  syphilis  is  responsible  for  a  small  percentage  of  choreas, 
especially  as  affecting  the  complete  development  of  the  nervous 
system,  thus  allying  such  choreas  with  related  spinal,  cerebellar,  and 
cortical  ageneses  (Friedreich,  etc.).  Hence  the  Wassermann  test 
should  be  applied  in  chorea  diagnosis.  Milian  has  found  from  60  to 
70  per  cent,  of  fifteen  choreics  examined  to  show  positive  Wassermann 
reaction.!  Other  investigators  have  found  much  smaller  percentages. 
This  may  account  for  the  good  results  from  salvarsan  in  the  treatment 
of  some  choreas. 

Acute  rheumatic  infection  loses  much  of  its  so-called  specificity. 
Tonsillitis  is  assuredly  not  the  universal  malefactor.  A  large  number 
of  other  infections,  measles,  scarlet  fever,  whooping-cough,  typhoid 
fever,  infected  teeth  (Streptococcus  mridans),  tonsils,  intestines  are 
important  as  reducing  physiological  efficiency,  by  interfering  with 
fiber-carrying  capacity  (through  inflammatory  exudates  at  times), 
especially  in  some  cases  where  meningitis  is  obvious,  of  an  insufficient 
or  tardily  maturing  and  integrating  motor  apparatus,  especially  in  its 
cerebellar  static  portion. 

Diagnosis. — The  chief  disorders  to  be  excluded  are  hysteria,  fre- 
quently from  imitation,  and  the  more  persistent  and  milder  of  the 
compulsive  tics.  Occasionally  the  choreic  movements  of  myoclonus, 
multiple  sclerosis,  and  explosion  shock  concussions  are  to  be  separated. 
The  organic  signs  enumerated  will  be  found  in  the  majority  of  choreics, 
if  carefully  looked  for.  Lumbar  puncture  may  be  resorted  to  in 
difficult  cases,  although  those  with  definite  leukocytosis  are  apt  to 
show  the  organic  signs.  In  certain  obscure  subinfections,  teeth, 
tonsils,  intestines.  Streptococcus  mridans  may  be  recovered  from  the 
blood.     Streptococcus-fixation  tests  are  also  of  value  in  the  diagnosis. 

The  diagnosis  of  hysteria  will  depend  upon  the  finding  of  definite 
conversion  mechanisms,  whil^  that  of  the  tics  will  on  analysis  show  the 
typical  substitution  mechanisms,  especially  the  infantile  reproaches, 
in  the  unconscious.  The  movements  in  both  may  be  similar  to  those 
called  choreic,  but  in  both  hysteria  and  in  tics  hypotonus  is  less  liable 
to  show.  There  is  a  greater  likelihood  that  so-called  hysterical  move- 
ments will  turn  out  to  be  choreas  than  the  reverse,  especially  in  younger 
children.  In  older  children  or  in  young  adults  the  opposite  may  be  more 
seriously  entertained. 

Treatment. — The  best  treatment  is  rest  in  bed,  with  partial  isolation, 
no  playing,  local  treatment  of  infected  throat,  tonsils,  teeth,  full  diet, 
with  increased  fatty  ingredients  (milk  and  eggs). 

The  rest  in  bed  should  be  practically  absolute  for  six  weeks,  and  if 
anemia  is  present,  and  it  usually  is,  iron  and  arsenic  are  useful. 

One  may  start  with  milder  arsenical  preparations  in  less  severe 
attacks,  and  in  those  where  sudden  disproportionate  growth  seems  to 

»  Milian:   Soc.  Med,  d'Hop,,  November  29,  1912. 


THE  CHOREAS  '^(^ 

play  a  larger  role,  Fowler's  solution  TTl  v-x  or  the  acidi  arsenosi  may 
be  used,  either  alone  or  in  pleasing  vehicles. 

In  severe  and  protracted  cases  the  intravenous  use  of  doses  of  0.05 
to  0.2  gm.  of  salvarsan,  according  to  age,  once  a  week  for  four  weeks, 
is  advantageous.  •     i     i 

Most  of  the  mild  cases  recover  on  prolonged  rest  m  bed,  without 
medication,  if  on  a  full  diet  with  milk  and  eggs  in  abundance. 

Huntington's  Chorea.— This  is  a  disorder  of  the  nervous  system, 
named  after  George  Huntington  (born  1850),  an  American  physician, 
who  gave  the  first  essential,  comprehensive  and  distinctive  descrip- 
tion of  the  disease.  Huntington's  grandfather  and  father  had  observed 
the  disorder  in  one  of  its  American  foci,  Easthampton,  Long  Island, 
and  chieflv  through  their  studies  of  several  generations  of  afflicted 
families   the   essentially   hereditary   nature   of   the   malady   became 

apparent.  o    i     i       >       u 

Huntington's  chorea  has  no  relationships  to  Sydenham  s  chorea, 
that  essentially  infantile  disturbance  of  cerebro cerebellar  tract  coordi- 
nations following  so  frequently  upon  infectious  disease  or  exhaustion. 
Huntington's  chorea  is  essentially  hereditary  and  chronic,  occurring, 
as  a  rule,  in  adults  from  thirty  to  fifty  years  of  age. 

The  condition  did  not  escape  earlier  observers.  Thilenms  gave  a 
report  of  a  case  apparently  as  early  as  1816.  Rufs,  another  in  1834. 
Waters  made  his  striking  comment  in  1841. ^  Dr.  Charles  G.  Gorman, 
of  Luzerne,  Pa.,  wrote  an  inaugural  thesis,  in  1848,  on  this  afl'ection, 
which  has  been  lost.  Dr.  Irving  W.  Lyon,  while  house  physician  at 
Bellevue  Hospital,  wrote  a  paper  on  "Chronic  Hereditary  Chorea," 
which  was  published  in  the  A  merican  Medical  Times  in  1863.  Hunting- 
ton's paper  appeared  in  the  Medical  and  Surgical  Reporter,  Phila- 
delphia, in  1872.  Since  this  time  a  rich  bibliography  has  accumulated, 
which  in  the  Huntington  number  of  Xenrographs,  edited  by  Dr. 
William  Browning,  in  1908,  mounted  to  200  titles. 

Etiology.— So  far  as  is  known  the  disorder  is  hereditary.  From 
eugenic  studies  of  Davenport,  Muncie,  and  JelHffe,^  the  chief  deter- 
mbiants  appear  to  behave  as  Mendelian  dominants.  Heilbronner  has 
said  that  the  disorder  appears  at  later  intervals  in  succee(ling  genera- 
tions, but  evidence  from  extensive  eugenic  studies,  which  includes  the 
studv  of  1000  cases  of  Huntington's  chorea,  limited  to  a  few  families, 
shows  that  it  appears  at  earlier  years  in  succeeding  generations.  The 
data  here  indicated  that  the  disease  behaves  as  a  complex  m  which 
age,  motor  disturbances,  and  mental  defect  behave  more  or  less 
independently  one  of  another.  When  all  three  factors  combme,  the 
result  is  Huntington's  chorea.    No  other  etiology  is  known. 

Symptoms.— As  it  is  not  possible  in  a  short  resume  to  discuss  the 
separate  factors,  the  older  lines  of  descri])ti()n  which  regard  the  dis- 
order as  a  unit  will  be  followed.    Thus  one  speaks  of  an  insidious  onset, 

>  Dunglison's  Practice  of  Medicine,  ii,  312.  »      *         t 

2  American  Neurological  Aaaqciation,  1913;  Muacje  and  Davenport:  Am.  Jour. 
Insanity,  1916. 


576     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

usually  coming  on  betAveen  the  years  of  thirty  and  forty.  The  earlier 
signs  are  either  slight  changes  in  character,  irritability,  moroseness, 
eccentricities,  or  the  choreic  movements  become  prominent  in  the 
picture.  The  facial,  neck,  and  upper  extremity  muscles  are  usually 
involved  first.  There  are  involuntary,  jerky  movements,  usually  of 
muscle  groups,  not  of  muscle  fibers.  The  excursions  brought  about  are 
massive,  i.  e.,  excessive,  loose,  and  h>T)otonic.  The  hand  is  thrown  to 
one  side,  the  whole  arm  sweeping  outward;  the  neck  is  jerked  back- 
ward, the  head  bowed  forw^ard  in  a  quick,  loose-jointed,  jerky  sort  of 
way.    The  motor  unrest  spreads  over  the  entire  body. 

The  patient,  after  several  years — for  the  motor  disturbance  advances 
slowly — becomes  jerky,  and  although  for  a  long  time  voluntary 
movement  is  able  to  check  the  excessive  motion,  finally  control  is  lost 
and  the  patient  is  confined  to  a  chair  or  his  bed,  making  his  peculiar 
broad,  jerky  movements.    During  sleep  the  motions  cease. 

Nearly  all  of  the  voluntary  muscles  may  be  involved.  The  eye 
movements  seem  to  resist  to  the  last.  The  speech  becomes  explosive, 
or  grunt-like,  very  incoherent  at  times  by  reason  of  the  involuntary 
movements  of  tongue,  lips,  diaphragm,  and  chest.  Even  swallowing 
is  involved  in  the  later  stages.  Writing  soon  becomes  impossible  by 
reason  of  the  jerkiness  of  the  hands  and  arms.  Walking  becomes 
successively  more  and  more  unsteady  until  the  patient  becomes 
bed-ridden. 

There  are  few  disturbances  of  sensibility  and  these  are  observed  only 
late  in  the  deteriorated  phases.  The  knee-jerks  are  active  or  unin- 
volved;  there  are  no  atrophies,  nor  paralyses,  nor  hypertrophies. 

The  mental  changes  may  develop  apart  from  the  motor  ones,  and 
in  choreic  families  mental  choreics  are  to  be  recognized  who  perhaps 
may  never  show  motor  signs  or  those  who  show  choreic  movements 
very  late.  This  is  the  basis  of  Diefendorf's  constitutionally  defec- 
tive group.  These  patients  even  in  childhood  may  be  excessively 
nervous,  irritable,  and  excitable.  There  are  often  diScult  children  to 
manage.  Their  eccentricities  become  more  and  more  marked  with 
advancing  years.  Some  show  marked  grades  of  feeble-mindedness  and 
occasionally  are  born  choreic  and  defective. 

In  the  great  majority  of  the  cases  the  insidious  and  slow  develop- 
ment of  great  instability  and  irascibility  shows  itself  coincident  with 
or  following  the  choreiform  movements.  Angry  outbursts  and  destruc- 
tive impulses  occur,  often  followed  or  preceded  by  periods  of  marked 
moroseness  and  despondency.  This  depression  or  gloom  may  be  a 
forerunner  of  suicide.  Diefendorf  remarks  that  this  despondency  is 
not  due  entirely  to  the  realization  of  having  the  disease.  With  some 
patients,  however,  the  suicide  is  to  be  traced  to  the  knowledge  of  the 
taint.  Suspiciousness,  paranoid  ideas  and  jealousies  are  not  infre- 
quent mental  signs.  Emotional  deterioration  follows.  The  patient 
loses  all  interest  in  his  work,  his  appearance,  his  home,  etc.  Some 
become  tramps.  Intemperance  and  free  erotic  activities  may  show 
themselves  with  this  gradual  deterioration.     Indifference  shades  off 


THE  CHOREAS  577^ 

into  absolute  incapacity  and  deterioration  becomes  profound,  always, 
however,  showing  itself  in  the  affective  sphere  more  prominently  than 
in  the  intellectual  capacities,  although  these  latter  are  not  free  from 
gross  defect;  the  patients  being  forgetful,  poor  in  ideas,  disorderly 
in  thought  and  weak  in  judgment. 

Diefendorf  speaks  of  a  group  in  which  the  mental  symptoms  develop 
somewhat  similarly  to  those  seen  in  the  hebephrenic  t^pes  of  dementia 
precox.  These  patients  complain  of  insomnia  and  general  malaise. 
They  often  then  develop  ideas  of  reference,  anxiety,  suspiciousness 
and  ideas  of  infidelity.  In  some  of  these  impulsive  activities  show 
themselves.  Homicide  has  taken  place.  Kraepelin  cites  an  illustra- 
tion of  a  choreic  father  who  killed  his  three  small  children  by  hanging, 
as  he  feared  he  could  not  support  them,  then  quietly  took  a  walk  and 
was  quite  unconcerned  about  the  affair  at  a  judicial  hearing.  The 
eating  is  often  impulsive  and  ravenous.  In  some  of  these  cases,  as 
with  the  inferior  group,  the  mental  symptoms  may  develop  long  before 
the  choreic  symptoms,  and  the  diagnosis  of  a  katatonic  schizophrenia 
may  be  made  as  the  motor  symptoms  become  manifest.  Here  the 
diagnostic  difficulties  are  very  definite. 

It  would  appear  from  Diefendorf  and  the  studies  cited  that  the 
mental  and  motor  traits  are  more  or  less  independent  one  of  the  other. 
In  inheritance  they  seem  to  show  as  such.  Some  patients  have  showii 
choreic  movements  for  twenty  to  thirty  years  without  mental  signs. 

Course. — This  is  subject  to  great  variation.  Often  the  patients 
suddenly  develop  great  motor  unrest;  the  mental  signs  augment 
rapidly,  and  the  patient  dies  in  exhaustion  five,  ten  or  fifteen  years  after 
the  onset  of  the  symptoms.  Usually  the  disorders,  /.  e.,  motor  and 
mental,  are  progressive;  rarely  they  may  diminish  after  reaching  a 
severe  grade.    Many  die  of  intercurrent  disease. 

Pathology. — Xo  unitars'  interpretation  seems  yet  possible.  The  motor 
and  the  mental  symptoms  are  best  considered  separately. 

A  number  of  autopsies  have  shown  a  variety  of  findings.  At  times 
there  are  chronic  meningeal  thickenings,  again  generalized  brain 
atrophy.  This  reduction  of  the  cortex  may  show  to  a  marked  degree 
in  the  loss  of  cells,  particularly  of  the  third  layer.  There  is  a  compen- 
satory increase  in  neuroglia.  In  some  patients  arterioslcerosis  has  been 
present,  in  others  not.  The  older  patients  showed  the  arteriosclerotic 
changes.  These  cortical  cellular  changes  are  apparently  more  frequent 
in  the  frontal  areas.    They  are  correlated  with  the  mental  defects. 

The  pathology  behind  the  motor  manifestations  is  more  obscure. 
Theoretical  considerations  as  well  as  pathological  findings  point  to  an 
implication  of  the  rubro-thalamo-cortical  extensions  of  the  cerebellar 
pathways  as  chiefly  responsible  for  the  perverted  movements.  Kleist, 
Zingerle,  Jelgersma,  and  Winkler  adduce  obser\-ations  from  various 
sides  which  tend  to  show  that  these  mechanisms  are  involved.^  Numer- 
ous autopsies  show  changes  in  the  lenticular  region  which  may  be 

'  Roussy  et  Lhermitte:    Archives  de  Mcdecin,  1915. 
37 


578     PARALYSIS  AGITANS,  CHOREA  AND  RELATED  SYNDROMES 

taken  to  support  these  contentions.  Thus  the  motor  signs  have  a 
pathology  closely  related  to  that  seen  in  paralysis  agitans  and  other 
midbrain  tremors.    The  putamen  region  shows  distinct  losses. 

Therapy. — There  is  no  efficient  therapy.  Many  patients  need  hos- 
pital care.  Prophylaxis  is  important,  Mendelian  dominance  arguing 
for  certain  factors  at  least,  that  these  patients  should  not  procreate. 
The  percentage  of  chance  of  escape  for  Huntington  choreics  is  about 
one  in  four  at  the  best.  Some  branches  escape,  and  in  accordance 
with  the  law  of  dominants,  a  branch  once  free,  is  always  free.  Only  a 
few  exceptions  to  this  are  found  in  the  Davenport-Muncie  series. 


CHAPTER  IX. 
CEREBELLAR  SYNDR0:MES. 

The  cerebellum  occupies  the  posterior  cerebral  fossa;  is  separated 
from  the  occipital  lobes  of  the  cerebrum  by  the  tentorium,  and  rests 
upon  the  pons  and  medulla,  forming  part  of  the  upper  boundary  of 
the  fourth  ventricle.  It  is  connected  with  the  rest  of  the  nervous 
system  by  the  anterior  meduHary  velum,  the  superior,  middle,  and 
inferior  cerebellar  peduncles  and  posteriorly  by  the  posterior  medullars- 
velum.  The  tela  choroidea  forms  the  posterior  continuation  of  this 
latter  structure,  and  serves  as  a  roof  to  the  posterior  part  of  the  fourth 
ventricle.^ 

Being  so^  intimately  connected  with  structures  in  the  midbrain,  the 
red  nucleus  and  the  optic  thalamus,  with  bulbar  and  pontine  centers 
and  with  the  cord;  lying  above  important  structures,  and  containing 
important  nuclei,  the  dentate  nucleus,  Deiters'  nucleus,  nucleus 
globosus,  nucleus  eraboliformis,  tectal  nuclei,  etc.;  with  a  multiplicity 
of  afferent  and  efferent  tracts,  the  possibilities  of  symptomatology 
are  very  numerous.    It  is  not  an  independent  organ. 

The  cerebellum  is  a  central  switchboard,  composed  of  complex  fiber 
and  synaptic  connections,  carrying  on  the  work  of  the  coordination 
of  the  reflexes  of  the  proprioceptive  system  (Sherrington) ;  that  is, 
that  system  of  receptors,  correctors  and  effectors  which  handle 
stimuli  which  originate  within  the  body  proper  (proprioceptors)  as 
contrasted  with  the  exteroceptive  system  of  receptors,  connectors  and 
eft'ectors  which  handle  energy  stimuli  from  the  external  world.  It 
thus  represents  the  entire  body.  This  extremely  intricate  and  much 
discussed  problem  of  cerebellar  localization,  particularly  of  different 
muscle  groups  cannot  be  entered  into  here  very  exhaustively.  The 
student  is  referred  to  the  studies  of  Folk,  Rijnberk,  Lewandowsky, 
Greggio,^  Thomas  and  Brouwer*  for  more  detailed  information.  We 
add  here  a  tabular  review  of  Folk's  localization  hypothesis  which,  in 
the  main,  has  seemed  to  be  supported  by  the  more  modern  investi- 
gations and  it  may  be  stated  that  there  exist  in  the  cerebellar  hemi- 
spheres centers  for  both  upper  and  lower  extremities.  These  centers 
are  decomposable  into  secondary  centers  representing  the  chief  articu- 
lations and  the  mode  of  action  of  the  muscles,  abduction,  adduction, 

'  Lowenstein:    Ztschr.  f.  N.  u.  P.,  ref.  vol.  v. 

'  Thomas:  The  Cerebellum,  Nervous  and  Mental  Disease  Monograph  Series.  Bolk: 
Das  Cerebellum  der  Sjiugethiere,  1906. 

'  Greggio:    Folia  Neurobiologica,  1914. 

*  Brouwer:  P.sychiatrische  u.  Neurologische  Bladen,  1915,  p.  104;  Archiv  f.  Psychia- 
trie,  1913,  li,  p.  539- 

(579) 


580 


CEREBELLAR  SYNDROMES 


flexion,  extension,  etc.,  are  all  represented.  In  the  vermis,  centers 
exist  for  the  muscles  of  the  head,  eyes,  tongue,  jaws,  face,  larynx, 
pharynyx  and  trunk.  Passive  as  well  as  active  motion  are  involved  in 
the  disorders  of  these  centers  and  the  asthenias  and  attitudes  of  the 
muscle  groups  offer  important  diagnostic  signs.  Meyers  galvanometric 
and  pedagraphic  methods  are  valuable  for  their  study.^  Many  of 
these  paths  are  definitely  known;  others,  particularly  those  connected 
with  the  viscera,  are  still  under  investigation .^  The  chief  functions 
known  are  related  to  the  localization  of  the  body  in  space,  and  static 
tonic  motor  innervation. 

ILobulus  1  f  Lingula;  lobulus  centralis  alae;  lobulus 
Lobulus  2  I  lunatus  anterior.  (Head  muscles:  eye, 
Lobulus  .3  I  chewing,  mimetic,  tongue,  larynx, 
Lobulus  4  [      pharynx.) 


Anterior  lobe 


Posterior  lobe 


Lobulus  media- 
nus  posterior 
(vermis  inferior 


Lobulus  C^ 


f  Lobulus  simplex;   neck  mus- 
cles. 
Lobulus  C^  (impaired  extrem- 
ity center). 
Lobulus  C,  pyramis  1  -r,      ^i.-  j         •       i 

Lobulus  b,  uvula  efel  [t)^  ''''     "^^  "'''" 

Lobulus  a,  nodulus    J  '' 


Lobulus  lateralis 
posterior 


Lobulus  ansiformis 


Lob.    paramedianus ; 

Paraflocculus   (floc- 
culus;   formatio 
vermicularis 
gluteal  muscles) 


Cms  primum  (Lob.  semilu- 
naris; extremity  muscles; 
paired  forefeet. 

Cms  secundum  (lob.  crucea- 
tus ;    extremity    muscles ; 
paired  hindfeet. 

tonsilla;  extremity  muscles; 
paired  hindfeet. 

Pars  tonsillaris;  tail  muscles. 
Pars  floccularis. 


From  the  cerebellar  cortex,  which  may  thus  be  looked  upon  as 
chiefly,  if  not  exclusively,  sensory,  these  impulses  pass  to  the  various 
nuclei  of  the  cerebellum,  and  are  there  redistributed.  It  would  appear 
that  these  intrinsic  cerebellar  nuclei  are  mainly  motor,^  the  move- 
ments of  the  head  being  chiefly  referable  to  the  intrinsic  nuclei,  those 
of  the  trunk  and  limbs  to  the  paracerebellar  nuclei. 

The  chief  afferent  or  receptor  paths  arrive  by  way  of  the  three 
cerebellar  peduncles. 

Bechterew  {loc.  cit.)  describes  seven  paths  as  passing  through  the 
inferior  cerebellar  peduncle,  or  restiform  body.    These  are  in  part: 

1.  The  dorsospinocerebellar  tract  of  Flechsig,  which  passes  up  the 
lateral  ventral  side  of  the  lateral  column,  originating  from  cells  in 
Clarke's  column,  from  the  upper  lumbar  to  the  upper  dorsal  segments. 
This  tract  passes  up  through  the  inferior  cerebellar  peduncle  (corpus 
restiforme),  and  is  thought  to  be  distributed  to  the  middle  lobe  of 
the  vermis  and  the  ventrolateral  lobe  of  the  lobus  centralis*  (Mott). 

1  Meyers:    Jcur.  Nerv.  and  Ment.  Dis.,  1919. 

^  Bechterew:  Die  Funktionen  der  Nervencentra,   1909,  ii. 

'  Horsley:  Functions  of  the  Cerebellum,  Brain,  1906,  xxix,  446, 

*  Monatsschrift,  1891,  i,  104. 


CEREBELLAR  SYNDROMES  581 

2.  The  posterior  columns  of  GoU  and  Burdach  send  fibers  from 
their  nuclei  in  the  medulla  by  way  of  the  restiform  body,  dorsally 
and  uncrossed  to  the  inferior  vermis,  ventrally  and  crossed  to  the 
superior  vermis.  (Many  authors  claim  that  these  bundles  have  no 
connection  with  the  cerebellum.) 

3.  The  olivocerebellar  tract,  which  originates  in  the  cord,  makes 
SA,Tiapses  in  the  inferior  olive,  from  which  it  passes  direct  (?)  to  Deiters' 
nucleus,  and  crosses  to  the  superior  vermis. 

4.  Vestibulocerebellar  path  from  the  vestibular  ganglion,  which 
sends  its  central  fibers  to  the  nucleus  vestibularis,  and  to  Deiters' 
nucleus  in  the  tegmentum,  and  from  thence  to  the  inferior  vermis. 
(The  details  of  these  pathways  are  given  in  the  chapter  on  the  vestib- 
ular functions,  also  in  Plates  X  and  XI.) 

These  pass  up  through  the  lateral  part  of  the  restiform  body.  In 
the  median  portion  there  are  two  groups  of  fibers:  One  contains 
sensory  fibers  from  the  cranial  nerves,  the  trigeminus  and  vestibular. 
They  form  the  direct  cerebellar  sensory  path  of  Edmger.  Other  fibers 
connect  the  nuclei  of  the  cranial  sensory  nerves  to  the  cerebellum. 
Both  of  these  bundles  end  for  the  most  part  in  the  tegmental  nuclei. 
This  tractus  nucleocerebellaris  is  an  indirect  path.^ 

In  the  middle  cerebellar  yeduncle  incoming  fibers  come  from  the 
nuclei  of  the  pontine  reticular  formation  and  the  ventrolateral  pontine 
nuclei.  Certain  of  these  fibers  are  in  relation  to  fibers  coming  from 
the  frontal  area  to  the  pontine  nuclei  and  form  part  of  a  frontocere- 
bellar  reflex  path  (Fig.  327). ^ 

The  fibers  passing  to  the  cerebellum  by  means  of  the  superior 
cerebellar  peduncle  are  four  in  number,  according  to  Bcchterew.  The 
best  kno^^^l  of  these  is  Gowers'  tract,  which  passes  into  the  cerebellum 
by  means  of  the  superior  cerebellar  peduncle.  According  to  Edinger 
and  Horsley  this  tract  is  distributed  to  the  entire  cerebellar  cortex. 
Fibers  from  the  thalamus,  from  the  red  nucleus  and  collaterals  from 
the  nuclei  of  the  eye  muscles  have  also  been  traced  through  the  superior 
cerebellar  peduncles. 

The  chief  efferent  tracts  are  (1)  The  superior  cerebellar  peduncle 
carrying  impulses  from  the  dentate  nuclei  to  the  red  nucleus  and  hypo- 
thalamus, (2)  the  tractus  fastigio  bulbaris  probably  from  the  nucleus 
fastigii  crossed  and  uncrossed  to  the  tegmentum  in  part,  and  in  part 
they  end  about  the  acoustic  nuclei,  (3)  a  fiber  system  passing  out 
through  the  midcerebellar  peduncle  to  ascend  in  the  raphe  of  the 
opposite  tegmentum,  (4)  arcuate  fibers  going  to  the  medullary  teg- 
mentum lying  dorsally  and  ventrally  to  the  pyramidal  fibers  and  (5) 
fibers  to  the  upper  segments  of  the  spinal  cord.^ 

The  cerebellum  therefore  forms  an  important  sensorimotor  station 

*  Bechterew,  ii,  961.  Bing:Die  Bedeutung  d.  spino-cerebellaren  Systeme,  1907. 
Rynberk:  Folia  Neurobiologica,   1907. 

^  Schaffer:  Der  Kleinhirnanteil  der  Pyramideiibahn,  Ztschr.  f.  N.  u.  P.,  vol.  xxvii, 
p.  435.  Bruns,  R. :  Zur  Kenntniss  der  B'ldungsfehler  des  Kleinhirns,  Arch,  suiss.  d. 
N.  ot  P.,  vols,  i,  ii,  iii. 

'  Hoestermanli :  Neur.  Ctbl.,  1911.     Sauer:  Folia  Neurobiologica,  1914. 


582 


CEREBELLAR  SYNDROMES 


in  a  complicated  series  of  reflexes  which  work  for  the  most  part  auto- 
matically. The  cerebellar  cortex  contains  the  chief  primary  reception 
synapses  for  relaying  the  impulses  coming  from  the  cord  and  brain- 
stem traversing  the  paths  just  enumerated,  convey  receptor  stimuli 
chiefly  concerning  the  states  of  tension  of  the  muscular  apparatus  of 


Fig.  327. — The  fiber  tracts  of  the  posterior  cerebellar  peduncle,  ra,  anterior  roots 
rp,  posterior  roots;  fc,  posterior;  fc',  ventral  cerebellar  tracts;  fi,  aberrant  .pyramidal 
fibers;  fG,  column  of  Goll;  fB,  column  of  Burdach;  fd,  descending  tract  from  Deiters' 
nucleus;  VIIIv,  vestibularis;  fi,  fibers  from  lateral  nuclei  of  medulla;  fgc,  fbc,  cerebellar 
fibers  from  posterior  column  nuclei;  pv,  vpi,  lateroventral  and  dorsomedian  pontine 
nuclei;  nD,  Deiters'  nucleus,  nv,  vestibularis  nucleus;  d,  descending  bundle  of  median 
portion  of  posterior  cerebellar  peduncle;  a,  ascending  fibers  of  vestibularis;  nd,  tectal 
nuclei;  co,  descending  path  from  tectal  nuclei  to  superior  olive;  cs,  superior  cerebellar 
peduncle;  rn,  nucleus  retrolenticularis ;  fl,  fibers  from  Deiters'  nucleus  to  posterior 
longitudinal  bundle;  os,  superior  olive;  fa,  fibers  from  flocculus  to  vermis;  cm,  median 
cerebellar  peduncle;  fv,  fasciculus  verticalis  pontis;  fct,  central  tegmental  tract;  oi, 
inferior  olive;  fo,  olivary  fasciculus.     (Bechterew.) 


the  trunk,  the  extremities,  and  the  head,  and  the  states  of  pressure  in 
the  joints  and  tendons.  The  reflexes  combine  to  regulate  the  con- 
stantly altering  positions  of  the  entire  body  in  space,  and  also  possibly 
of  the  viscera.  The  cerebellum  therefore,  in  this  sense,  acts  as  a  regu- 
lating, coordinating  organ  for  the  estimation  of  the  body  in  space.    To 


Fig.  328. — The  ascending  cerebellar  tracts  of  the  spinal  cord  and  their  central  path- 
ways; B,  Burdach's  column;  ca,  anterior  cerebellar  fasciculus  to  the  vermis  through  the 
anterior  medullary  velum  co,  cerebello-olivary  fillers;  cp,  common  path  from  posterior 
cohmin  nuclei  to  vermis;  d,  dentate  nucleus;  fi,  internal  arcuate  fibers;  fp,  posterior 
arcuate  fibers;  ft,  fibers  from  vermis  cortex  to  tegmental  nuclei;  G,  column  of  GoU; 
g,  corticovermis  association  fibers;  gl,  nucleus  globosus;  p,  nucleus  emboliformis;  rp, 
posterior  sensory  root  fibers;  sc,  subcortical  paths  of  the  brachium  conjunct ivum; 
80,  olivospinal  tract;  t,  tegmental  nuclei;  Ir,  fasciculus  spinocerebellaris;  Vi,  inferior 
vermis;  Vs,  superior  vermis.     (Bechterevv.) 


584 


CEREBELLAR  SYNDROMES 


the  spinal,  midbrain,  cerebral  reflex  arcs  there  are  also  added  fronto- 
pyramido-ponto-bulbo-cerebellar  arcs  which  contain  involuntary  as 
well  as  voluntary  regulatory  coordinating  impulses,  acting  to  orient 
the  body  in  practically  all  of  its  spatial  relations.  Cyon  adds  that 
therefore  the  cerebellum  is  intimately  concerned  with  the  mathematical 
sense  in  its  objective  space  relations.^ 


Fig.  329. — Descending  pontine  and  cerebellar  tracts;  VI,  root  fibers  abducens;  cp, 
corticopontine  fibers;  crp,  fibers  from  pons  to  cerebellum;  d,  dentate  nucleus;  fed,  cere- 
bello-Deiters'  fibers;  fo,  tegmento-olivary  fibers;  ft,  fibers  from  vermis  to  tegmental 
nuclei;  g,  cortical  association  fibers;  NVI,  abducens  nucleus;  nd,  Deiters'  nucleus. 
Nol,  inferior  olive;  nt,  tegmental  nuclei;  os,  superior  olive;  ra,  anterior  root  fibers; 
(Bechterew.) 

Symptoms. — The  general  symptoms  of  disturbances  of  the  proprio- 
ceptive pathways  and  the  cerebral  projicient  systems  as  they  converge 
in  the  cerebellum,  offer  a  multitudinous  complex,  the  details  of  which. 


»  Cyon:  Das  Ohrlabyrinth,  1908. 


CEREBELLAR  SYNDROMES 


585 


and  their  anatomicopathological  correlations,  are  still  being  rapidly 
added  to.  Among  these,  however,  there  are  certain  outstanding  trends 
which  are  capable  of  partial  difPerentiation  as  syndromes  at  the  present 
time,  and  to  which  attention  will  be  directed. 

Cerebellar  Ataxia. — This  complex  of  disturbances  is  one  of  the 
classical  signs  of  cerebellar  disorder.  It  consists  in  a  disturbance  of 
the  coordination  of  the  larger  muscular  group  actions,  principally  of 
the  trunk  and  lower  extremities,  although  the  head,  eyes,  and  upper 
extremities  are  not  uninvolved.  Thus,  standing,  walking,  and  the 
making  of  fine  coordinated  movements  are  interfered  with  in  the 
absence  of  signs  of  distinct  paralysis. 


Fig.  33U  Fig.  331 

Figs.  330  and   331. — The  peculiar  station  and  gait  of  cerebellar  ataxia.     (Thomas.) 

In  walking,  the  wobbling,  side-stepping  gait,  so  well  described  by 
early  French  authors  (Duchenne,  of  Boulogne)  as  the  drunken  gait, 
is  characteristic.  In  severe  grades  of  cerebellar  ataxia  standing, 
and  even  sitting,  becomes  impossible.  In  milder  grades  one  sees 
these  tj-pes  of  disturbance  in  many  choreas,  in  paralysis  agitans,  in 
general  paresis,  multiple  sclerosis,  etc. 

A  i)artial  study  of  the  cerebellar  gait  has  shown  two  characteristic 
trends  of  disturbance:^  first,  staggering  (lateropulsions)  toward  the 
affected  side,  at  times  forward  or  backward,  according  to  location  of 
lesion   in  vermis  (or  dentate  nucleus).     The  patient  feels  as  though 

'  Stewart  and  Holmes:  Brain,  1904. 


586 


'  } 


CEREBELLAR  SYNDROMES 


shoved  to  one  side  and  in  the  attempt  at  restitution  overcorrects 
(asynergia  of  Babinski)  and  thus  sways;  second,  the  entire  orienta- 
tion in  space  is  influenced  and  the  patient's  movements,  as  a  whole, 
swerve  in  the  direction  of  the  affected  side  (menagery  movements,  as 
seen  in  whirling  white  mice;  tumbling  movements,  as  seen  in  the 
tumbler  pigeon). 

Conscious  attempts  at  correction  (frontocerebellar  paths)  produce  the 
larger  zig-zags  in  the  general  course  of  the  progression.  Forw^ard  and 
backward  movements  have  their  special  localizing  signs  to  be  spoken 
of.  From  the  anatomical  standpoint  these  ataxias  may  result  from 
involvement  of  the  spinocerebellar  paths  (Flechsig,  Gowers — vestib- 
ular systems),  as  in  the  Friedreich 
and  Marie  ataxia  group ;  from  involve- 
ments  in  the  cerebellum  itself  (tumors, 
cysts,  ageneses,  scleroses) ;  in  affections 
involving  the  superior  cerebellar  pe- 
duncles— from  bulbar  and  pontine  in- 
volvements of  these  paths,  and  also 
from  implication  of  the  cerebellorubral, 
cerebellothalamic,  and  frontocerebellar 
paths.  Typical  cerebellar  ataxias  are 
thus  seen  in  some  frontal  tumors,  with 
classical  intoxication  gait. 

Dysmetria. — Chiefly  associated  with 
ataxias  of  the  trunk  and  the  lower 
extremities  in  standing,  walking,  etc., 
are  the  bilateral  or  unilateral  (usually 
homolateral)  dysmetrias  of  the  upper 
extremities.  The  individual  muscular 
activities  are  illy  coordinated,  both  as 
to  space  and  time,  the  desired  act  only 
being  arrived  at  after  several  efforts, 
with  over-  and  undercorrection  in 
point  of  the  measure  of  the  movement 
and  as  to  its  timing.  This  dysmetria 
may  be  brought  out  by  grasping  a  glass, 
when  the  brusqueness  of  the  attack 
and  the  over-extension  of  the  fingers  is  manifest,  by  the  brusque  pro- 
nation and  supination  of  the  hand,  by  the  finger-nose  test,  finger- 
finger  test,  finger-ear  test  and  by  writing.  Visual  aid,  or  its  lack,  has 
little  or  no  influence  on  the  dysmetria  as  tested  by  the  finger-nose 
test  and  finger-finger  test,  and  the  dysmetria  is  a  constant  one,  non- 
increasing  as  in  an  intention  tremor  (with  varying  gradations,  for 
certainly  the  intention  tremors  of  multiple  sclerosis  are  often  due  to 
interference  with  cerebellar  mechanisms).  Guillain  has  reported  an 
interesting  war  picture  of  multiple-sclerosis-like  tremor  from  cerebellar 
involvement. 1 

1  Reunion  du  vi  Armee,  17  May,  1915. 


Fig.  332. 


-Cerebellar  gait  attitude. 
(Thomas.) 


CER  E  BELL  A  R  S  YN  DROMES 


587 


The  Barany  finger  tests  almost  invaiiably  show  important  modi- 
fications in  cerebellar  disease  and  are  always  to  be  utilized.  (See 
Examination  chapter.) 


Fig.  333. — Test  for  dysmetria  in  cerebellar  disturbances. 


Fig.  334. — ShowinR  cerebellar  dysmetria. 

AdiadokoHnesis } — Originally  described  by  Babinksi^  as  character- 
istic of  cerebellar  disease,  this  symptom,  consisting  of  a  disability  in 

'  Macfie  Campbell  et  Crouzon.  Rev.  Neur.,  1902,  p.  1186. 
*  Soe.  de  Neurol.,  Nov.  6,  1902. 


588  CEREBELLAR  SYNDROMES 

the  performance  of  rapid  movements  involving  the  alternating  actions 
of  agonist  and  antagonist  muscles  (see  Examination),  is  not  invariably 
found  nor  always  clearly  indicative  of  cerebellar  disorder;  yet  it  is 
so  frequently  found  as  to  merit  special  attention.  It  is  in  part  a  variant 
of  dysmetria  brought  out  by  a  special  test.  Here  the  timing  sense  that 
is  regulated  by  the  cerebellum  is  at  fault.  In  the  absence  of  paralysis 
it  usually  is  indicative  of  cerebellar  path  involvement.  It  is  frequently 
absent  in  extracerebellar  tumors  in  which  there  are  other  well-marked 
cerebe  lar  signs,  as  in  frontal  tumors. 

Vertigo.^As  the  chief  organ  of  orientation  in  space,  severe  disturb- 
ance of  certain  of  the  cerebellar  reflex  paths  causes  vertigo,  which 
is  apt  to  be  a  prominent  and  a  fairly  constant  sign.  The  vertigo  is 
of  a  rotatory  character.  The  patient  may  not  only  feel  himself  revolv- 
ing in  space,  but  objects  may  go  around  from  right  to  left  or  from  left 
to  right ;  more  rarely  the  vertigo  has  an  up  or  down  character.  Each 
of  these  two  characters  is  to  be  closely  inquired  into.  Here  the  chief 
lesions  are  connected  with  the  vestibular  paths,  as  the  labyrinth  is 
the  chief  cephalic  ganglion  in  the  whole  proprioceptive  system,  of 
which  the  cerebellum  constitutes  the  coordinating  switchboard.  Thus 
labyrinthine  disease  itself,  as  well  as  disease  of  the  extracerebellar  or 
intracerebellar  vestibular  paths  may  give  rise  to  the  symptom.  By 
means  of  the  specific  tests  devised  by  Barany  (see  chapter  on  Exami- 
nation) a  separation  of  labyrinthine  diseases  of  extracerebellar  origin 
is  usually  possible. 

Further,  enough  experience  has  accumulated  (Stewart  and  Holmes, 
loc.  cit.)  to  show  that,  in  general,  objects  rotate  from  the  diseased  to 
the  well  side  for  mtracerebellar  as  well  as  extracerebellar  affections, 
whereas  the  subjective  sense  of  rotation  is  usually  from  the  diseased 
to  the  well  side  in  intracerebellar  involvement,  and  the  reverse  in 
extracerebellar  involvement  of  the  paths.  This  generalization,  a 
little  too  broad,  is  in  need  of  further  study,  and  of  more  accurate 
localization. 

Nystagmus. — A  fourth  sign,  rarely  absent  in  cerebellar  path  dis- 
turbance, is  nystagmus.  It  is  also  closely  related  to  the  vestibular 
reflex  system,  and  may  result  from  extracerebellar  involvement  as 
well  as  from  intracerebellar  implication  of  the  paths. 

True  vestibular  nystagmus  is  almost  invariably  accompanied  by 
vestibular  vertigo  and  ataxia.  Vestibular  nystagmus  itself  is  usually 
modified  by  the  position  of  the  head,  hence  every  person  with  nys- 
tagmus must  be  examined  with  the  head  in  three  planes;  a  patient 
with  vestibular  nystagmus  tends  to  rotate  within  the  plane  of  the 
nystagmus,  and  in  the  direction  opposite  to  that  of  the  quick  nystagmus 
movement. 

A  patient  with  vestibular  nystagmus  then,  who  bends  his  head  for- 
ward at  90  degrees  will  rotate  in  a  direction  directly  opposite  if  he  bend 
his  head  backward  90  degrees.  The  laws  of  intracerebellar  nystagmus, 
apart  from  actual  vestibular  disease,  remain  to  be  investigated  (con- 
jugate deviations,  skew  deviations,  and  other  eye  displacements  are 


CEREBELLAR  SYNDROMES 


589 


to  be  interpreted  in  the  light  of  forced  movements  having  their  analo- 
gies to  nystagmus,  and  are  considered  in  the  chapter  on  ]\Iidbrain 
Disease.  See  illustrations  of  conjugate  palsies;  also  in  chapter  on 
the  Eye  Paths.  See  plate  of  oculorotary  and  cephalorotarv  mechanisms 
(Plate  VIII). 

Closely  associated  with  disorder  of  the  \-estibular  system  are  pain 
in  the  muscles  of  the  neck,  nausea,  vomiting,  amblyopias,  and  loss  of 
consciousness. 


Fig.  335. — Dysmetria  of  Babinski  devel- 
oped on  attempting  to  take  hold  of  a  glass. 
The  fingers  are  held  very  far  open.  (Thomas.) 


Fig.  336. 


-Asynergia  of  Babinski. 
(SchaUer.) 


Cerebellar  Ilyjjotonvs. — Palpation  of  the  muscles,  testing  of  resis- 
tance movements,  and  looseness  in  the  performance  of  passive  move- 
ments, reveal  a  type  of  muscular  hypotonus  in  cerebellar  affections 
which  is  usually  homolateral.  This  hypotonia,  or  atonia,  is  usually 
accompanied  b>'  normal  or  even  exaggerated  tendon  reflexes  in  con- 
trast to  that  of  peripheral  neuritis  or  tabes.  One  feature  of  this 
hypotonus  noted  by  Stewart  and  Holmes  is  striking.  If  a  resistance  to 
a  definite  movement  be  suddenly  relaxed,  in  the  normal  flexing  of  the 
arm,  for  instance,  there  is  a  sudden  flexor-jerk,  followed  by  an  extensor 
recoil.  In  a  cerebellar  hypotonic  reaction  the  flexor-jerk  is  excessive, 
and  is  rarely  followed  by  a  recoil. 


590  CEREBELLAR  SYNDROMES 

Asthenia. — A  paresis  or  asthenia,  usually  homolateral,  is  closely 
related  to  cerebellar  h>T)otonus.  Its  presence  in  cerebellar  disorder 
has  usually  been  interpreted  as  due  to  a  lesion  of  the  pyramidal  tracts 
by  contiguity;  unquestionably,  however,  it  is  a  true  cerebellar  symp- 
tom. It  is  chiefly  present  when  the  cerebello-vestibulo-spinal  and 
rubrospinal  tracts  are  affected.  Since  the  rubrospinal  tract  is  to  be 
interpreted  as  an  auxiliary  to  the  pyramidal  tract,  the  different  opinions 
of  various  authors  may  find  a  common  adjustment. 

Cerebellar  Asynergia.— Described  by  Babinski^  as  a  special  symptom 
of  cerebellar  disease,  this  sjTnptom  is  in  reality  one  of  the  components 
of  cerebellar  ataxia,  but  in  the  anteroposterior  plane,  rather  than  in 
the  lateral  planes.  It  consists  in  the  patient's  inability  to  balance 
himself,  whereby  his  legs  either  walk  away  from  under  him,  or  he 
pitches  forward  without  their  following.  It  is  a  severe  grade  of  retro- 
pulsion  and  propulsion,  as  seen  in  paralysis  agitans,  and  due  in  both 
instances  to  similar  pathological  foundations. 

Cerebellar  Fits. — Originally  described  by  Jackson^  as  tetanus-like 
convulsive  seizures,  with  characteristic  holding  of  the  body  in  extended 
rigid  position. 

Forced  Movements. — ^These  are  present  in  the  neck  muscles,  muscles 
of  the  eyes,  and  appear  as  irritative  or  as  defect  symptoms,  due  to 
disease  of  the  hemispheres  or  of  the  middle  cerebellar  peduncle.  (See 
Diseases  of  Midbrain.) 

Speech  Disturbances. — Dysarthrias  usually  indicate  the  same  type  of 
ataxia  as  found  in  other  muscles  of  the  body,  adiadokokinesis.  They 
are  usually  present  with  defects  of  the  cerebellum,  and  may  indicate 
general  defect  of  the  entire  apparatus,  disease  of  the  bulbocerebellar 
tracts  or  pressure  upon  the  bulbar  nuclei  from  contiguous  new 
growths. 

Chief  Syndromes. — In  discussing  disorders  of  the  cerebellum  it  is 
convenient  to  take  up  first  affections  of  the  peduncles,  although  very 
rare,  then  of  the  cerebellum  itself,  and  finally  diseases  of  extracere- 
bellar  location,  where  pressure-producing  growths  occupying  the  pos- 
terior fossa  implicate  the  cerebellar  mechanisms,  and  those  of  its  con- 
tiguous structures,  the  pons,  medulla,  and  fourth  ventricle. 

Inferior  Cerebellar  Peduncle. — Corpus  Restiforme. — Isolated  disease  of 
this  structure  is  rare.  Pressure  due  to  bulbar  and  pontine  disease  is 
not  uncommon.  The  most  characteristic  symptoms  are  modifications 
of  the  eye  movements,  convergent  and  divergent  strabismus,  even 
skew  deviation,  forced  positions,  turning  of  body  toward  the  site  of 
lesion,  and  vertigo,  with  tendency  to  fall  in  the  direction  of  the  side 
of  the  lesion.  The  chief  mechanism  involved  is  the  cerebello-vestibulo- 
spinal  tract.^    (See  Midbrain  Lesions.) 

1  Soc.  de  Neurol.,  Nov.  9,  1899;  Rev.  Mensuelle  des  Med.  interne  et  de  Thcrapeu- 
tique,  May,  1909. 

2  British  Med.  Jour.,  November  4,  1917;  Reprint,  Brain,  190G,  p.  425. 

'  Adler:  Die  Symptomatologie  der  Kleinhirnerkrankungen,  Wiesbaden,  1899,  has 
collected  the  cases. 


CHIEF  SYNDROMES  591 

Lesions  in  the  Middle  Cerebellar  Peduncle. — ^These  cause  rolling  move- 
ments of  the  body  on  its  vertical  axis,  skew  deviation  of  the  eyes, 
^lagendie-Hertwig  syndrome  consisting  in  one  eye  being  higher  than 
its  mate.  The  patients  behave  as  though  they  had  bilateral  vestibular 
disease,  causing  the  rolling  motions;  the  eye  symptoms  depend  on 
lesions  of  the  fasciculus  anteromarginalis,  fibers  to  the  posterior 
longitudinal  bundle,  and  interference  with  the  fibers  to  the  abducens 
nucleus. 

There  are  few  uncomplicated  cases  on  record.  Pontine  lesions 
often  give  rise  to  s^inptoms  from  implication  of  the  middle  peduncle. 
(See  Midbrain  Syndromes.) 

Lesions  in  the  Superior  Cerebellar  Peduncles. — Isolated  lesions  of  these 
peduncles  are  rare.  The  symptoms  are  usually  choreic,  or  paralysis- 
agitans-like  tremors  on  the  same  side  of  the  lesion — possibly  due  to 
implication  of  the  cerebello-rubro-spinal  bundle  in  the  tractus  cerebello- 
tegmenti.  Forced  positions  of  the  head  to  the  side  of  the  lesion  have 
been  described.  Ocular  implications  rarely  occur,  although  nystagmus 
has  been  observed.     (See  ^Midbrain  Lesions.) 

Lesions  within  Cerebellum  Itself. — ^The  most  important  of  these  are 
ageneses  or  aplasias,  scleroses  or  atrophies,  hemorrhage,  softening,  in- 
flammation, abscesses,  and  tumors. 

Aplasias  of  Cerebellum. — ^These  are  congenital,  and  represent  a 
vast  array  of  different  conditions;  total  lack  of  cerebellum,  absence 
of  the  lateral  (in  old  sense)  lobes,  absence  of  vermis,  unilateral  loss, 
irregular  defects,  and  general  congenital  smallness  of  the  cerebellum 
and  cerebrum. ,  A  consistent  symptomatological  grouping  is  not  yet 
possible.  ]\Iingazzini^  has  attempted  it.  His  grouping  of  the  con- 
ditions is  as  follows: 

1.  Pure  unilateral  ageneses  and  atrophies. 

2.  Pure  bilateral  ageneses  and  atrophies. 

3.  Cerebellar  atrophies  associated  with 

(a)  Disease  of  the  cerebrum. 

(b)  Disease  of  the  spinal  cord. 

1.  Unilateral  loss  of  a  lateral  (in  old  sense)  lobe  may  be  present 
without  any  symptoms  according  to  present  developed  modes  of 
testing.  Few  of  these  cases  have  been  tested  by  more  recent  methods. 
In  certain  instances  unilateral  atrophy  has  been  associated  with 
epileptiform  convulsions,  or  retropulsion,  when  the  cortex  of  the 
aft'ected  hemisphere  is  atrophic;  if  the  atrophy  involves  a  part  of  the 
vermis,  slight  motor  signs,  such  as  slowing  of  the  gait,  have  been 
observed. 

2.  Pure  Bilateral  Agenesis. — In  some  of  the  cases  reported  no  symp- 
toms have  been  observed  (old  cases).  The  commonest  symptoms  are 
difficulty  in  standing  and  walking.  The  patient,  in  high  grades  of 
atrophy  or  aplasia,  is  unable  to  stand,  or  sometimes  even  to  sit;  in 
the  milder  grades  the  station  is  wobbly,  the  feet  placed  far  apart,  and 

'  Monatschr.  f.  Neur.  u.  Psych.,  1906,  xviii,  76.  See  Bruns,  R. :  Archives  suisses  de 
Neur.  et  de  Psych.,  vols,  i,  ii,  iii,  for  complete  modern  discussion. 


592 


CEREBELLAR  SYNDROMES 


walking  is  possible  only  with  assistance.  The  gait  is  then  the  classical 
drunken  stagger.  There  is  marked  asynergia  of  the  trunk  and  lower 
extremities.     Tremors,   ataxias,   incoordination    (asynergias)    of   the 


Fig.  337. — Posterolateral  pontine  syndrome.  Lesion  of  the  lateral  part  of  the  left 
pontine  tegmentum,  involving  the  nuclei  of  the  trigeminus,  the  crossed  secondary  sensory 
paths  of  the  tegmentum,  partially  involving  the  superior  cerebellar  peduncle  (Pes) 
and  the  median  lemniscus  (Rni),  and  not  involving  the  anterior  portion  of  the  pons. 

On  the  right  there  is  hemianesthesia  of  the  extremities  of  the  syringomyelitic  type, 
above  all  for  pain  and  temperature  sense. 

On  the  left  there  is  paralj^sis  of  the  muscles  of  mastication  (pterygoid,  masseter, 
temporal)  by  lesion  of  the  motor  nucleus  of  the  trigeminus.  There  is  slight  anesthesia 
in  the  trigeminus  region  (sensory  nucleus  V)  and  choreo-athetoid  movements  of  the 
extremities  from  involvement  of  the  superior  cerebellar  peduncle.     (Dejerine.) 


CEREBELLAR  AGENESES  593 

upper  extremities  are  also  present.  Hypotonus,  muscular  weakness, 
slow,  irregular,  hesitating  or  explosive  speech  are  also  present.  Nys- 
tagmus may  or  may  not  be  present,  there  is  usually  adiadokokinesis, 
the  knee-jerks  are  usually  normal,  or  even  slightly  exaggerated  at 
times,  even  in  the  presence  of  hypotonus.  Bilateral  atrophies  show 
similar  symptoms. 

Comhined  Aplasias  of  the  Cerebellum  and  Brain. — Combettes'  (old 
period)  patient,  with  absolute  absence  of  the  cerebellum  had  from  birth 
epileptiform  attacks,  was  able  to  walk  but  fell  often.  Many  of  these 
patients  are  idiotic  and  imbecile,  and  show  symptoms  similar  to  those 
enumerated  in  the  previous  paragraph. 

jMingazzini  includes  the  olivo-ponto-cerebellar  atrophies  here,  but 
these  are  discussed  later. 

Holmes^  calls  these  cases  "  congenital  smallness  of  the  central  nervous 
system,  with  cerebellar  symptoms." 

A  number  of  conditions  may  be  grouped  here.  Some  of  Marie's 
so-called  hereditary  cerebellar  ataxias  are  best  referred  here.  Irreg- 
ular staggering  gait,  Romberg,  disorder  of  speech,  nystagmus,  and 
ataxias  of  limbs  are  the  chief  symptoms.  These  patients  have  shown 
small  cerebellums  with  apparently  intact  tracts  in  cerebellum  and  cord. 

Olivo-pontQ-cerehellar  Atrophy. — This  tA'pe  was  described  by  Thomas 
and  shows  a  fairly  definite  syndrome.  Anatomically  there  is  atrophy 
of  the  cerebellar  cortex,  of  the  bulbar  olive,  and  of  the  gray  matter 
of  the  pons.  There  is  total  degeneration  of  the  middle  cerebellar 
peduncles,  partial  degeneration  of  the  inferior  cerebellar  peduncles, 
and  a  relative  integrity  of  the  cerebellar  nuclei.  It  is  not  necessarily 
hereditary,  familial  nor  congenital.  It  comes  on  at  an  advanced  age 
and  progresses  slowly.  Clinically  there  is  great  defect  in  equilibration 
in  standing  and  walking — drunken  gait.  Romberg  is  absent.  Some 
irregular  intention  tremor,  usually  nystagmus,  and  also  scanning 
speech  is  present. 

Cerebellum  and  Cord  Atrophies  or  Aplasias.- — Here  also  a  motley 
group  is  on  record.  These  cases  will  vary  greatly  in  proportion  to 
the  varying  degree  of  the  lesion  in  the  cerebellum  and  in  the  cord. 
Marie's  hereditary  cerebellar  atrophies  belong  here.  Some  authors 
are  disposed  to  refer  Friedreich's  disease  to  this  group  also,  and  to 
claim  that  between  these  two  disorders  one  finds  every  gradation  from 
the  Friedreich  type,  with  mostly  cord  and  little  cerebellar  change,  to 
Marie's  type,  with  more  cerebellar  and  little  cord  change. 

The  chief  signs  here^  are  ataxias  of  the  leg,  arm,  facial,  ocular,  head, 
laryngeal,  and  pharyngeal  muscles.  Cerebellar  reeling,  no  Romberg. 
Later  palsies  and  contractions.  Exaggerated  tendon  phenomena. 
In  most  of  these  cases  the  disorder  is  predominantly  in  the  cord. 

In  other  cases  with  degeneration  of  the  spinocerebellar  tracts  with 
normal  or  only  small  cerebellum  one  finds  patients  with  staggering 

'  Brain.  1907,  p.  546. 

2  Holmes:  Brain,  1907,  loc.  cit.,  for  literature. 

3  Brown:  Brain.  1892,  xv,  250. 
38 


594  CEREBELLAR  SYNDROMES 

gait,  scanning,  explosive,  slow  speech,  nystagmoid  jerkings  of   the 
eyes,  muscular  cramps,  fatigability  of  muscles,  normal  or  exaggerated 


I 


Fig.  338. — Scoliosis  of  Friedreich's  ataxia. 


Fig.  339. — Friedreich's  ataxia,  showing  the  permanent  extension  of  the  great  toe. 


CEREBELLAR   TUMORS  595 

knee-jerks.    As  mentioned,  Friedreich's  disease  properly  belongs  to  this 
rubric. 

Primary  Parenchymatous  Degeneration. — Holmes^  has  described 
this  condition.  It  usually  sets  in  about  middle  age  and  progresses 
slowly.  Staggering  or  reeling  gait  is  an  early  s\Tiiptom,  then  asynergia 
of  the  upper  extremities,  and  later  hesitating,  scanning,  or  explosive 
articulation,  nystagmus,  tremor  of  the  head  and  limbs.  Tendon 
reflexes  are  normal  or  slightly  exaggerated.  Xo  clonus,  no  Babinski. 
Sphincters  intact  and  normal  psyche.  ]Most  of  the  cases  have  shown  a 
familial  character. 

Cysts  of  Cerebellum.- — Cysts  and  cystic  timiors  are  not  always 
distinguishable.  Together  they  form  a  small  part  of  tumor  formations 
in  the  cerebelliun  (5  to  10  per  cent.).  The  symptoms  of  cerebellar 
cysts  are  practically  identical  with  those  of  cerebellar  tumor,  but  the 
operative  outlook  is  much  better. 

Cerebellar  Tumors.^ — Before  discussing  the  subject  of  tumors  of  the 
cerebellum  proper  and  their  sxTnptomatology  a  word  may  be  said 
regarding  the  question  of  cerebellar  localization.  The  researches  of 
Bolk,  and  others*  in  comparative  anatomy,  and  of  Horsley^  have 
served  to  make  fairly  certain  that  both  in  the  cortex,  and  in  the 
intrinsic  nuclei  there  are  definite  localizations,  the  former  with  refer- 
ence to  sensory  representations  from  different  portions  of  the  body, 
the  latter  with  reference  to  motor  representations. 

With  reference  to  sensory  representation,  studying  practically  only 
the  terminations  of  Gowers'  tract,  Horsley^  concluded  that  there 
was  no  evidence  of  differentiation  of  the  cerebellar  cortex  into  localized 
receiving  stations  for  the  impressions  (muscular,  arthritic)  which 
ascend  from  the  arm,  trunk,  or  leg  muscles,  joints,  etc.,  respectively. 
This  author  holds  that  the  results  of  the  work  of  Bolk  and  others 
did  not  guard  sufficiently  against  lesions  of  the  adjacent  nuclei. 

While  this  may  be  true  for  the  distribution  of  Gowers'  tract,  it  is 
not  true  for  the  distribution  of  the  olivocerebellar  tract.  Stewart  and 
Holmes^  have  shoMii  that  fibers  from  certain  portions  of  the  inferior 
olive  pass  to  definite  regions  in  the  contralateral  cerebellar  cortex. 
The  function  of  these  olivocerebellar  paths  Is  still  in  question.  As 
to  definite  localization  for  other  receptor  paths  (chemical,  etc.),  exact 
knowledge  is  absolutely  wanting  at  the  present  time. 

So  far  as  localization  of  motor  f mictions  in  the  nuclei  is  concerned, 
this  seems  to  have  received  definite  confirmation  by  the  work  of 
Horsley  Hoc.  cit.). 

Cerebellar  tumors  are  relatively  frequent.  Almost  one-third  of 
the  brain  tumors  occurring  in  childhood  and  adolescence  are  of 
cerebellar  origin.  In  order  of  frequency  one  finds  gUoma,  tubercle, 
sarcoma,  and  fibronia, 

1  Brain,  1907,  p.  466. 

2  Williamson:  Revnew  of  Neurology  and  Psychiatry,  March.  1910,  for  literature. 
'  Stewart  and  Holmes:  Brain,  1904,  xxv-ii,  522. 

*  See  Van  Rynberk,  Ergebnisse  d.  Physiol.,  1907.  *  Brain,  1908. 

«  Ibid,  1900.  '  Ibid..  1908. 


596 


CEREBELLAR  SYNDROMES 


The  chief  general  symptoms  of  cerebellar  tumors  are  headache, 
usually  severe,  occipital  or  frontal,  and  apt  to  be  confined  to  a  sagittal 
plane;  papilledema  and  later  optic  atrophy,  which  is  rapidly  progres- 
sive; vomiting,  vertigo,  and  tenderness  to  percussion  over  the  occiput. 


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Fig.   340. — Bilateral  softening  of  the  cerebellum.     (Schaller.) 

Tumors  in  the  hemispheres,  not  involving  the  central  tracts  or  the 
intrinsic  nuclei,  may  give  rise  to  no  localizing  symptoms.  But  there 
is  usually  an  implication  of  these  contiguous  structures  with  added 
symptoms. 


Fig.  341. — Tumor  of  cerebellum.     (Goodhart.) 

These  are  the  classical  cerebellar  syndromes  of  gait,  and  attitude, 
asynergia,  ataxia  and  adiadokokinesis  of  the  same  side,  with  hypo- 
tonia of  special  character  already  described,  and  motor  paresis  of  the 
same  side.    To  these  are  usually  added  nystagmus  and  eye  deviations 


CEREBELLAR   TUMORS 


597 


already  noted.  The  nystagmus  is  apt  to  be  pronounced  only  on 
looking  to  the  affected  side,  and  is  usually  slower  and  coarser  than  the 
nystagmus  of  labyrinthine  origin,  or  of  involvement  of  the  vestibular 
tracts.  As  these  latter  are  frequently  impinged  upon,  it  is  useless  to 
insist  upon  too  fine  distinctions  in  the  character  of  nystagmus.  Unless 
the  pyramidal  tracts  are  influenced  by  pressure,  the  arm  and  leg  tendon 
reflexes  are  not  markedly  exaggerated,  nor  are  they  lost,  and  the 
Babinski,  Oppenheim,  Schaefer  and  Remak  signs  of  pyramidal  tract 
involvement  are  not  present.  The  abdominal  reflexes  are  usually 
unmodified. 

Finger-pointing   tests  are  of    special  value  in  localization  in  the 
initial  stages. ^ 


Fig.  342. — "Skew  deviation"  after  removal  of  a  tumor  from  the  left  lateral  lobe 
of  the  cerebellum;  the  left  eye  is  directed  downward  and  inward,  the  right  eye  upward 
and  outward.     (Holmes  and  Stewart.) 

As  the  tumors  increase  in  size  there  are  added  symptoms  due  to 
encroachments  or  pressure  upon  contiguous  structures.  These  are 
usually  the  signs  of  involvement  of  the  pyramidal  tracts,  eye  palsies, 
and  of  the  cranial  nerves — from  the  fifth  to  the  twelfth.  These  all 
show  on  the  oi)posite  side  of  the  body.  The  two  lower  branches  of 
the  facial  are  involved,  the  tongue  protrudes  to  the  yjaralyzed  side 
and  is  without  atrophy  or  R.  D.  Occasionally  the  medulla  is  pressed 
upon  and  one  finds  all  branches  of  the  facial  involved  with  atrophy  of 
the  tongue.  Homolateral  anesthesia  of  the  cornea  may  be  present, 
due  to  trigeminus  involvement.  Homolateral  aft'ections  of  the  ears, 
deafness,  buzzing  and  homolateral  pain  to  pressure  on  the  mastoid 
may  aid  in  diagnosis.  Percussion  should  never  be  neglected.  Oppen- 
heim has  called  attention  to  the  cracked-pot  sound  often  present  in 
cerebellar  tumors. 


'Rothmann:  Neur.  Centralljlatt,  1914,  No.  1, 


598  CEREBELLAR  SYNDROMES 


f 


Unilateral  signs  are  apt  to  pass  over  into  bilateral  signs  as  the  pres- 
sure increases,  with  dysarthria,  dysphagia,  continued  vomiting,  and 
finally  cardiac  and  respiratory  signs. 

Lumbar  puncture  may  give  important  information  in  clearing  up 
a  diagnosis  of  meningitis-serosa  or  hydrocephalus. 

Dyssjmergia  Cerebellaris  Progressiva — Chronic  Progressive  Cerebellar 
Tremor.^ — As  dyssynergia  cerebellaris  progressiva,  Ramsay  Hunt  has 
described  a  chronic  progressive  tremor  disturbance  of  cerebellar  origin. 

This  affection  is  characterized  by  generalized  intention  tremors, 
which  begin  as  a  local  manifestation  and  then  gradually  involve  in 
varying  degree  the  entire  voluntary  muscular  system.  The  tremor 
which  is  extreme  when  the  muscles  are  in  action,  ceases  entirely  during 
relaxation  and  rest.  This  disorder  of  motility  is  associated  with  dis- 
turbances of  muscle  tone  and  of  the  ability  to  measure,  direct  and 
associate  muscular  movements;  the  clinical  manifestations  of  which 
are  dyssynergia,  dysmetria,  hypotonia,  adiadokokinesis  and  asthenia. 
All  of  these  symptoms,  including  the  volitional  tremor,  which  is  an 
extreme  expression  of  the  underlying  disturbance  of  muscle  tone  and 
synergy,  indicate  a  disorder  of  midbrain  and  cerebellar  mechanisms. 

These  cases  are  further  distinguished  by  the  absence  of  true  nystag- 
mus, objective  vertigo  cerebellar  fits,  vestibular  seizures  and  disturb- 
ances of  equilibrium,  symptoms  which  are  so  frequently  associated 
with  gross  lesions  of  the  cerebellum. 

The  clinical  picture  is  strictly  limited  to  a  progressive  disturbance 
of  synergic  control,  the  most  striking  characteristic  of  which  is  the 
ataxic  intention  tremor,  which  accompanied  any  movement  of  the 
affected  part,  whether  volitional,  reflex  or  automatic. 

Pathology. — The  pathology  is  as  yet  unknown,  but  the  progressive 
tendency,  chronic  course  and  well-defined  cerebellar  symptomatology, 
would  suggest  an  organic  disease  caused  by  degeneration  of  certain 
special  structures. of  the  cerebellar  mechanism  which  are  concerned  in 
the  regulation  of  the  tonus  and  synergies  of  muscles. 

Differential  Diagnosis. — In  general  appearance,  the  motor  disturbance 
which  characterizes  the  progressive  cerebellar  dyssynergia  is  similar 
to  the  intention  tremor  of  multiple  sclerosis.  It  differs,  however,  in  the 
slow  and  gradual  manner  of  progression  and  the  strict  limitation  of  the 
symptomatology  to  tremor  and  its  associated  dysmetria,  dyssynergia, 
hypotonia,  and  intermittent  asthenia. 

All  other  symptoms  of  multiple  sclerosis,  such  as  nystagmus, 
objective  vertigo,  pyramidal  and  sensory  tract  symptoms,  temporal 
pallor,  hemiplegic  attacks,  forced  laughter,  alterations  of  the  reflexes 
are  absent;  so  that  a  cerebellar  type  of  this  affection  may  be  excluded. 

The  pseudosclerosis  of  Westphal  may  also  be  eliminated  by  reason 
of  the  strict  limitation  of  the  symptomatology  to  the  volitional  tremor, 
together  with  the  absence  of  mental  deterioration,  pigmentary  deposits 
and  the  other  symptoms  which  characterize  the  recorded  cases  of  this 
obscure  affection. 

'  Brain,  1914,  xxxvi. 


CEREBELLAR  INJURY  509 

The  theory  of  a  functional  disturbance,  in  the  nature  of  hysteria 
or  the  traumatic  neurosis,  is  not  tenable  in  the  absence  of  an  adequate 
etiological  factor,  and  the  mental  and  somatic  symptoms  which  char- 
acterize these  affections. 

The  rare  tremor  t^■pe  of  Parkinson's  disease  may  likewise  be  excluded 
by  reason  of  the  nature  of  the  tremor  and  the  existing  hypotonia  and 
dyssynergia,  which  differ  fundamentally  from  the  muscle  manifestations 
that  characterize  this  affection. 

Huntmgton's  chorea,  athetosis  and  myoclonus  multiplex  are  readily 
differentiated  by  the  character  of  the  motor  disorder  and  persistence 
during  muscular  relaxation. 

Of  especial  importance  from  the  diagnostic  standpomt  is  the  group 
of  the  so-called  essential,  hereditars'  or  family  tremors.  Progressive 
dyss\iiergia  is  not  a  tnie  tremor,  but  a  synergic  disturbance  which  is 
evident  only  when  the  extremity  is  in  action,  and  consists  of  coarse 
irregular  movements  in  which  the  constant,  vibratory  characteristics 
of  true  tremor  is  almost  entirely  lacking. 

Prognosis. — The  prognosis  is  unfavorable  but  like  other  system 
diseases  the  affection  is  compatible  with  many  years  of  life.  The 
whole  course  of  the  disease  is  chronic  and  slowly  progressive,  and  the 
motor  life  becomes  more  and  more  restricted,  so  that  the  patients  in 
time  are  almost  entirely  dependent  upon  the  care  of  the  nurses  or 
relatives.  Once  established,  the  tremor  never  disappears,  except  during 
rest. 

There  are  dt^ys,  and  sometimes  longer  periods  of  even  weeks,  during 
which  the  tremor  suffers  temporary  exacerbations.  After  the  sub- 
sidence of  these  crises  of  tremor  there  is  a  return  to  the  usual  chronic 
condition. 

Wounds  of  the  Cerebellum. — ^The  World  War  has  increased  greatly 
the  possibilities  of  study  of  cerebellar  function.  These  studies  are  too 
numerous  to  be  here  reviewed.  A  syndrome  of  irritation  due  to 
cerebellar  substance  loss  has  been  described  by  Goldstein  and  Reich- 
mann^  and  several  important  contributions  have  been  made  by 
Thomas,^  Levi^  and  others.  Goldstein  and  Reichmann's  irritation 
syndrome  is  characterized  by  overvaluation  of  weights,  of  spatial  and 
of  time  relations,  by  increased  sense  of  heaviness  and  of  pseudospon- 
taneous  movements.  Deviation  of  finger  test  with  conservation  of 
cerebellar  center  stimulation  and  increase  of  labyrinthine  deviation. 
There  is  adiadokokinesis,  excess  of  Holmes-Stewart  contraction  test 
and  increased  pressure  and  muscle  sensation. 

Hemorrhage  of  Cerebellinn,* — Spontaneous  cerebellar  hemorrhage  is 
extremely  rare.  Hemorrhage  from  wounds  of  war  have  been  com- 
paratively frequent  in  the  world  war.  Its  symptomatology  will  depend 
largely  on  its  size  and  the  location  of  the  effusion.    The  superior  cere- 

»  .\rch.  f.  Psychiatric,  1916,  lii,  2:  Ibid.,  1917,  Ivii. 
^  Etudes  sur  les  blessures  du  cervelet,  1918. 

3  Bull.  Acad,  de  m6d.,  May  8,  1917,  No.  19.  Claude  et  Lhermitte:  Soc.  med.  d.  Hop., 
May  26,  1916. 

<  Starr:  Medical  Record,  May  2,  1906. 


600 


CEREBELLAR  SYNDROMES 


cellar  artery  is  oftenest  involved,  thus  implicating  certain  portions  of 
the  dentate  nuclei.  Extension  into  the  fourth  ventricle  is  to  be  borne 
in  mind. 

The  onset  of  the  symptoms  is  usually  sudden,  or  preceded  by  pain 
in  the  back  of  the  head,  with  slight  giddiness,  or  forced  position  of 
the  head.  There  is  usually  marked  vertigo,  recurring  on  attempts 
to  move,  and  usually  persisting  in  non-fatal  cases.  Cerebellar  gait, 
forced  position  of  head,  depending  on  location,  nystagmus,  especially 
on  lateral  movements.  Operative  interference  is  generally  useless  in 
the  spontaneous  tj^es.  War  wounds  may  be  localized  according  to 
the  principles  laid  down  by  Bolk  and  Barany  (loc.  cit.)  especially  when 
made  by  smaller  missiles  and  after  the  acute  symptoms  have  subsided. 
The  finger-pointing  tests  are  especially  valuable  in  localization.^ 


Fig.   343. — Hemorrhage  of  cerebellum.      (Larkin.) 


Cerebellar  Abscess. — These  are  relatively  frequent,  and  originate 
mainly  from  middle-ear  infections,  either  by  way  of  the  temporal 
lobes  or  the  mastoid,  and  wounds,  from  trauma,  which  latter  may 
have  occurred  a  long  time  previously.  Occasionally  abscess  may 
result  from  thrombi  due  to  abscess  of  the  lungs,  ulcerative  endocar- 
ditis, etc. 

These  abscesses  vary  considerably  in  size  from  that  of  a  pea  to  an 
apple,  and  their  development  is  either  acute  or  chronic. 

The  characteristic  symptoms  are  headache,  usually  occipital,  and 
radiating  into  the  neck  region,  producing  marked  stiffness  of  the 
neck,  at  times  resembling  the  pain  of  a  cerebrospinal  meningitis. 
General  unrest,  nausea,  vomiting,  and  stupor  are  present.  Hyper- 
thermia may  be  added,  but  a  cerebellar  abscess  may  run  a  course  of 

^  See  Jones:  Equilibrium  and  Vertigo,  loc.  cit.  Stiefler:  Ztschr.  f.  d.  ges.  Neur.  u. 
Psych.,  1915,  24.  Friedliinder:  Neur.  Centralblatt,  November  21,  1915.  Bauer: 
Wien.  kl.  Wchnschr.,  1916,  29. 


POSTERIOR  FOSSA   SYNDROMES  601 

several  months  without  temperature.  Of  special  cerebellar  symptoms 
ataxia,  nystagmus  and  rotatory  vertigo  are  characteristic.  This 
vertigo  is  usually  rendered  worse  and  vomiting  is  induced  by  move- 
ment of  the  body;  hemiparesis  and  hemiasynergia  are  usually  present 
on  the  side  of  the  lesion,  but  these  are  not  constant  signs. 

By  an  increase  in  the  size  of  the  abscess,  sympton  s  of  pressure, 
precisely  similar  to  those  mentioned  under  Tumor,  may  develop. 
Papilledema,  with  optic  atrophy,  is  not  infrequent  in  large  abscesses. 

Brain  puncture  by  special  aspiration  needles  is  advisable  to  clear 
up  the  diagnosis  in  complicated  cases.  Lumbar  puncture  is  useful  in 
excluding  meningitis. 

Associated  Posterior  Fossa  Complications. — Occipital  Lobes. — These 
may  be  pressed  upon  anterosuperiorly  by  a  foreign  body  growing  upon 
or  within  the  superior  lobe  of  the  cerebellum.  Hemianopsia  and  mind- 
blindness  may  then  occur. 

Corpora  quadrigemina. — Ocular  palsies  of  a  nuclear  character,  occur, 
first  on  one  side,  then  upon  the  other.  The  oculomotor  and  obducens 
are  oftener  involved  than  the  trochlear.  There  is  less  apt  to  be  a 
paralysis  of  accommodation  or  changes  in  the  pupils,  and  the  loss  of 
conjugate  motion  of  the  eye  is  rarely  found.  Implication  of  the  pos- 
terior corpus,  and  of  the  middle  geniculate  causes  deafness,  usually 
bilateral,  and  if  the  lateral  geniculate  be  pressed  upon,  amblyopia, 
without  papilledema.    Pineal  tumors  may  cause  the  same  picture. 

Cerebral  Peduncles. — If  these  structures  are  markedly  impinged 
upon,  the  Weber-Gubler  s\iidrome  of  alternate  hemiplegia  and  oculo- 
motor palsy  may  be  found.  From  milder  irritative  pressures  one 
obtains  the  Benedict  syndrome,  oculomotor  palsy,  with  tremor  of  the 
opposite  side.  If  the  lemniscus  is  impaired  there  is  crossed  anesthesia 
and  ataxia.    (See  Midbrain  S^'ndromes.) 

Pons. — Here  one  finds  a  number  of  sjTidromes  added  to  the  initial 
cerebellar  symptoms.  Crossed  hemiplegia  with  facial  palsy  (Millard- 
Gubler)  and  R.  D.  Occasionally  from  a  more  anterior  pressure  there 
may  be  homolateral  facial  palsy  without  R.  D. 

Crossed  Hemiplegia  and  Abducens  Palsy. — Both  are  usually  asso- 
ciated with  hypoglossal  involvements.  Conjugate  deviations  to  the 
opposite  side  of  the  lesion  are  observed  in  these  cases  when  the  abducens 
nucleus  is  involved.  Such  conjugate  palsies  speak  more  for  tumors 
within  the  pons  {q.  v.).    (See  Midbrain  S>Tidromes.) 

Crossed  hemiplegia  and  trigeminus  involvement  are  occasionally 
found,  and  also  alternate  hemiplegias  with  cochlearis  symptoms. 
Here  the  hearing  defect  is  due  to  destruction  of  the  intrapontine 
fibers  of  the  cochlearis;  or  to  pressure  on  the  tuberculum  acousticinn. 

Tumors  of  the  Fourth  Ventricle. — These  may  be  considered  here 
because  of  the  cerebellar  sjTnptoms  induced.  These  tumors  are  for 
the  most  part  glioma,  sarcoma,  psammoma,  carcinoma.  They  give 
rise  to  symptoms  due  to  pressure  on  the  medulla  and  pons  and  almost 
always  cause  a  marked  hydrocephahis  with  advancing  stupor  and 
confusion.     Cvsticercus  mav  also  be  found  here.     Bruns  has  caUed 


602 


CEREBELLAR  SYNDROMES 


attention  to  the  following  features:    Alternating  periodicity  of  head- 
ache,  nausea,   vomiting,   vertigo,   changes   in  pulse  and  breathing, 


I 


^' 

■■  '^"'' " 

1 

i 

;jr.l 

.   <? 

«.»> 

K 

=  %^^ 

W^- 

v><-.  ■ 

■4ifllMmu 

w 

^ 

W 

Fig.  344. — Tumor  oi  fourth  ventricle  bringing  pressure  on  vestibular  nuclei,  causing 

nystagmus.     (Sehaller.) 


Fig.  345. — ^Tunior  (neurofibroma)  of  cerebellopontine  angle.     (Larkin.) 


CEREBELLOPONTINE  ANGLE  SYNDROMES  603 

with  sudden  let-up  of  all  these  symptoms.  The  vertigo  and  vomiting 
are  set  up  by  changes  in  position  of  the  head;  sudden  movement  of 
the  head  may  cause  immediate  unconsciousness.  Cerebellar  ataxia, 
mild  nystagmus  and  occasionally  diplopia  are  other  signs. 

One  more  word  may  be  said  about  cerebellar  tumors  and  their 
diagnosis.  They  may,  in  the  presence  of  few  signs  only,  be  mistaken 
for  affections  of  the  frontal  lobes  (fronto cerebellar  paths),  parietal 
lobes  (implications  of  central  sensory  components)  and  of  the  optic 

thalamus. 

In  frontal  lobe  tumors  special  intelligence  defects  are  usually  found, 
if  carefully  examined  for  by  the  methods  of  Ziehen,  Sommer,  and 
Kraepelin!  The  tremor  is  apt  to  be  ver^-  fine  and  rapid,  hemiparesis, 
if.  present,  is  crossed,  and  shows  spastic  phenomena;  the  speech  dis- 
turbance is  aphemic;  the  conjugate  deviations  are  irritative  and  not 
paralytic.  Then  anosmia,  apraxia,  witzelsucht  and  aphasia  are  often 
added.    Skew  deviations,  and  hji^otonus  are  not  known  for  frontal 

tumors. 

Parietal  lobe  disease  only  occasionally  offers  difficulties,  while  the 
characteristic  senson.^  disturbances  and  central  pains  of  optic  thalamus 
involvement  should  exclude  this  structure. 

Cerebellopontine  Angle  Tumors  .'—These  should  be  discussed  here 
because  of  the  symptoms  of  cerebellar  pressure  and  of  vestibular 
involvement.  Two  main  types  of  tumor  come  under  review.  Those 
from  the  pia  of  the  cerebellum,  and  those  growing  on  or  about  the 
acoustic  nerve.    Fibromata,  myomata,  and  sarcomata  are  the  most 

frequent.  i    n  j       i 

These  tumors  press  upon  the  pons  and  middle  cerebellar  peduncle 
and  the  svmptoms  vary  slightly,  according  to  the  variations  in  pressure 
on  these \wo  structures.  The  eighth  nerve  is  usually  involved  early; 
buzzing  and  deafness  are  observed.  Facial  palsy  is  usual  with 
corneal  anesthesia  from  pressure  on  the  fifth;  trigeminal  pains  are 
frequent.  Ptosis  may  appear.  Pressure  on  the  cerebellum  causes 
the  typical  gait  and  the  ataxia,  homolateral  paresis,  and  hypotonus. 
A  contralateral  paresis  from  pressure  on  the  pyramidal  tracts  usually 
develops.  This  gives  the  usual  signs  of  a  pyramidal  tract  involvement. 
Homolateral  static  tremor  is  frequent,  also  a  sense  of  subjective  rotation 
toward  the  side  of  the  lesion. 

Treatment  of  Cerebellar  Conditions.— Cummata  must  be  attacked  by 
the  usual  antisyphilitic  treatment,  otherwise  surgery  offers  the  only 
opportunit>-,  and  this  is  limited  to  the  attack  upon  c>sts  which 
may  give  fortunate  results.  The  results  of  operations  for  abscess  are 
improving,  as  are  also  those  for  tumors.  But  as  each  case  is  a  law 
unto  itself,  it  is  futile  to  generalize. 

>  Henschen  F. :  Ueber  Geschwulste  der  hinteren  Schiidelgrube  irn  besonderen  des  Klein- 
hirnbriickenwinkels,  1911,  for  full  literature  to  1911;  also  English  literature,  Stewart  and 
Holmes:  Brain,  1904;  Weisenberg:  Jour.  Am.  Med.  Assn.,  1908;  Starr:  Jour.  Nerv  and 
Mert.  Dis.,  1910;  Lewandowsky:  Handbuch  der  Neurologie.  Gushing:  Tumors  of  the 
Acoustic,  1918. 


CHAPTER   X. 

DISEASES  OF  THE  MENINGES. 

Here  diseases  of  the  dura,  the  arachnoid,  and  the  pia  are  to  be 
recognized.  Under  the  first  various  types  of  meningeal  hemorrhage 
and  inflammation — pachymeningitis — are  found.  Under  the  latter — 
various  forms  of  leptomeningitis  and  cerebrospinal  meningitis. 

DURAL  DISEASE. 

1.  Meningeal  Apoplexy  {Traumatic  Meningeal  Hemorrhage.  Frac- 
ture of  the  Skull). — Etiology. — Trauma  from  blows,  instruments, 
violence  of  various  kinds,  causes  either  a  rupture  of  the  branches  of  the 
middle  meningeal  artery,  the  veins,  or  of  the  dural  sinuses.  vSuch 
hemorrhages  may  be  found  at  the  site  of  the  injury,  or  at  the  point 
opposite.^ 

The  hemorrhage  may  be  extradural,  between  the  pia  and  dura, 
epidural,  or  between  the  pia  and  cortex,  arachnoidal  hemorrhage 
The  bleeding  may  be  diffuse  or  circumscribed.  At  childbirth  such 
hemorrhages  with  partial  destruction  of  the  brain  substance  itself  are 
extremely  frequent.  Here  the  bleeding  is  almost  always  exclusively 
venous. 

Pathology. — Macroscopically  such  hemorrhages  resemble  hemor- 
rhagic pachymeningitis,  but  microscopically  they  differ,  especially  in 
the  absence  of  new  elements — vessels,  plasma  cells,  etc. 

Not  infrequently  in  severe  fractures  the  brain  substance  is  also 
involved. 

Symptoms. — These  will  vary  according  to  the  mechanism  of  the 
producing  lesion,  the  presence  or  absence  of  a  communicating  wound, 
the  form  of  the  fragments  and  the  location.  Bursting,  bending,  simple, 
compound,  fragmented  or  perforating  fractures  are  the  types  met 
with  in  war  neurosurgery.  Basal  fractures  are  apt  to  be  linear, 
cortex  fractures,  comminuted  with  displaced  fragments  and  much 
laceration.  In  severe  injuries  there  are  signs  of  shock  and  concussion 
in  addition  to  the  symptoms  of  pressure.  Unconsciousness  becomes 
increasingly  deeper,  the  pulse  is  slow  in  the  beginning,  then  becomes 
rapid,  vomiting  takes  place,  urination  and  defecation  are  involuntary, 
irregular  respiration,  with  increasing  temperature,  and  death  often 
results  unless  operation  relieves  the  pressure. 

1  A.  Mayer:  Zentbl.  f.  Gyn.,  1915,  No.  46.    Gushing:  Keen's  Surgery,  1917.     British 
Jour.  Surg.,   1918.     Guiilain:  Presse  m?d.,  September  5,   1918,  for  meningeal  hemor- 
rhages in  war. 
( 604 ) 


MENINGEAL   HEMORRHAGE 


605 


In  less  severe  lesions  the  initial  symptoms  of  concussion  with  vary- 
ing grades  of  stupor  partially  clear  up  for  an  hour  or  more,  even 
twenty-four,  or  a  few  days.^  Then  compression  symptoms  develop 
which  Kocher  has  divided  into  four  phases:  (1)  Stage  of  compensation 
compression  through  the  cerebrospinal  fluid  shifting.  Headache, 
mental  dulness  and  slight  focal  signs  characterize  this  stage.  Then  (2) 
increased  headache,  vertigo,  irritability,  restlessness,  blanching  cf  cere- 
bral veins,  it  may  be,  with  delirium.  The  focal  symptoms,  depending 
on  location,  are  apt  to  be  increased.  There  may  be  spasms,  epileptiform 
con\Tjlsions,  often  of  Jacksonian  type,  monoplegias,  hemiplegias,  the 
arm  often  suffering  the  worst.  The  hemiplegia  is  usually  on  the  side 
opposite  to  the  site  of  injury,  but  in  about  5  per  cent,  of  the  cases 


Fig.  346. — Pia-arachnoid  hemorrhage  from  contra  coup.     (Larkin.) 

is  found  on  the  side  of  the  lesion  (uncrossed  pyramids  or  contra  coup). 
Aphasias  are  not  infrequent  in  left-sided  injuries.  The  tendon  reflexes 
are  usually  increased  on  the  paretic  side,  while  the  skin  reflexes  are 
usually  diminished.  The  Babinski  phenomenon  is  frequent  on  the 
paralyzed  side,  and  occasionally  present  on  the  side  of  the  hematoma. 
Occasionally  hemianesthesia  and  hemianopsia  can  be  made  out.  Guil- 
lain  calls  attention  to  the  slight  biliary-  discoloration  of  the  skin  as  a 
.special  sign  in  severe  meningeal  hemorrhages  from  war  shocks,  with 
or  without  cranial  trauma. 

Basal  fractures  may  simply  show  the  signs  of  concussion,  prolonged 
coma,  with  gradual  recovers-  or  death.     Not  infrequently  there  is 


•  Connell:  Free  Interval  in  Meningeal  Hemorrhages,  Surg.,  Gjn.  and  Obstet.,  March, 
1906. 


606 


DISEASES  OF   THE  MENINGES 


bleeding  from  the  ears  or  nose,  or  there  may  be  subconjunctival 
eccliATnoses.  Cranial  nerve  injury  is  not  an  infrequent  accompanying 
symptom.  It  may  be  the  auditory,  or  the  sixth,  or  the  facial.  At  times 
all  three  may  be  involved.  Stage  (3)  shows  increasing  compression 
when  the.  pulse  commences  to  slow  down,  the  splanchnic  vessels  are 
constricted  and  there  is  a  rise  in  blood-pressure.  The  respiration  is 
stertorous  and  coma  is  present  with  dilated  pupils,  it  may  be,  and  loss 
of  all  reflexes.  With  increasing  compression  (4)  and  death  there  is 
circulatory  failure,  rapid  pulse,  deep  stupor  and  decreasing  blood- 
pressure.  In  most  head  injuries  there  is  an  initial  lowering  of  the  tem- 
perature followed  by  a  rise,  the  height  of  which  is  more  or  less  in  pro- 
portion to  the  severity  of  the  head  injury.  High  temperatures  seem 
more  associated  with  contusions  than  with  simple  hemorrhage.  Tem- 
peratures of  104°  F.  and  over  are  ominous. 


Fig.  347. — Traumatic  supradural  hemorrhage.     (Larkin.) 


In  severe  meningeal  hemorrhages  from  brain  injuries  or  compression 
the  pupils  vary  greatly.  Wiesmann  has  shown  in  70  cases,  that  in 
39  both  pupils  were  dilated  and  immobile,  in  20  there  was  dilatation 
on  the  side  of  the  hemorrhage,  in  7  both  pupils  were  small,  and  in  4 
there  was  dilatation  on  the  side  opposite  the  lesion.  The  recent  war 
studies  show  great  variability  in  the  pupillary  symptoms.  They  are 
so  variable  as  to  be  of  small  diagnostic  value,  in  gross  lesions  parti- 
cularly.   In  about  one-half  the  cases  it  may  be  said  that  the  larger 


BIRTH  PALSIES  607 

pupil  corresponds  to  the  greater  intracerebral  pressure  Widely 
dilated  and  non-reacting  pupils  offer  a  sinister  prognosis.  The  fundus 
findings  are  of  great  value  in  determining  intracerebral  pressure 
especially  in  old  cases,  less  so  in  acute  ones,  as  they  rarely  develop 
within  twenty-four  hours. 

Birth  Palsies. — In  birth  hemorrhages,  Seitz  has  shown  that  subten- 
torial  hemorrhages  behave  differently  from  convexity  hemorrhages.  In 
the  former  the  children  frequently  show  no  sign  of  asphyxia,  then  after 
a  few  hours  respirator^'  disturbances  set  in,  the  breathing  becomes 
irregularly  quickened,  with  spasms  and  cyanosis.  Then  spasms  of  the 
eyeballs  occur,  less  often  facial  spasm.  If  the  pressure  is  directed 
do^Tiward  toward  the  medulla,  opisthotonos  and  muscular  rigidity 
develop,  and  not  infrequently  priapism. 

In  convexity  hemorrhages  the  child  shows  considerable  restlessness, 
refuses  to  suckle,  and  then  develops  signs  of  brain-pressure,  pressure 
in  the  fontanelles,  respiratory  disturbances,  drowsiness  to  unconscious- 
ness, with  some  slowing  of  the  pulse.  Localizing  symptoms  may  then 
show  themselves,  spasms  of  the  opposite  arm  and  leg,  increased  tendon 
reflexes,  and  slight  hypertonus.  In  children  the  late  results  bring  about 
various  syndromes.  When  the  hemorrhage  and  destruction  occupies 
the  motor  areas  alone,  the  type  of  Little's  disease  is  present.  Slight 
variations  in  localization  of  the  hemorrhage  and  destruction  bring 
about  variant  forms  of  Little's  disease  with  sensory  involvement. 
Cerebellar  localizations  bring  about  anomalous  cerebellar  diplegic 
tA^DCS.^  According  to  the  localization  of  the  hemorrhage  the  following 
cerebral  types  of  birth  palsy  may  be  distinguished: 

1.  Atonic  types  with  mental  defect — frontal  lobe. 

2.  Spastic  t\-pes — Little's  disease,  motor  area. 

3.  Sensory  t^-pes — speech  defects,  posterior  central. 

4.  Pseudobulbar  palsies. 

5.  Cerebellar  diplegic  tj'pes  (Batten,  Clark,  L.  P.). 
Diagnosis. — In  birth  hemorrhages  it  may  be  extremely  difficult  to 

determine  whether  one  has  to  deal  solely  with  a  pure  meningeal 
apoplexy,  or  whether  there  is  also  intracerebral  disturbance.  If  there 
is  a  definite  free  interval  after  the  initial  signs  of  concussion,  with  the 
gradual  or  sudden  onset  of  compression  signs,  the  probabilities  are  for 
hematoma  (80  per  cent.).  The  length  of  free  interval  oft'ers  no  certain 
criterion  as  to  site  of  hemorrhage.  Choked  disk,  often  transitory, 
also  speaks  for  hematoma.  Bloody  spinal  fluid,  which  does  not  clot, 
speaks  for  intradural  hemorrhage,  occasionally  extradural.  Neisser's 
brain  puncture  and  lumbar  procedures  often  helj)  in  clearing  up  a 
diagnosis.    Long  intervals  speak  for  abscess. 

The  diagnosis  of  war  fractures  is  usually  obvious.  X-ray  for  type 
of  fracture  is  imperative.  In  a  certain  percentage  of  cases  sugar  appears 
in  the  urine.  Lumbar  puncture  oft'ers  many  important  diagnostic  signs 
in  hidden  fractures  of  the  base. 

'  Clark,  L.  P.:  Jour.  Nerv.  and  Ment.  DL«.,  1915.  Tr.  Am.  Xeur.  Soc,  1916.  Hunt: 
J.   R.:  Loc.   cit.     Batten:  Brain.    1913. 


608 


DTSEASES  OF   THE  MENINGES 


Therapy. — This  is  surgical  for  vault  and  comminuted  fractures  and 
should  be  immediate.  The  exact  procedure  must  be  determ  ined  largely 
by  the  SAinptoms.  The  prognosis  is  apt  to  be  good  if  the  patient  lives 
forty-eight  hours,  those  dying  after  this  usually  succumbing  to  abscess 
or  meningitis.  The  general  prognosis  is  bad,  about  |  fatal.  Even  the 
intracranial  hemorrhages  of  childbirth  may  be  controlled  by  skilled 
surgical  measures.  The  results  of  surgical  interference  are  three  times 
as  good  as  leaving  the  patients  aione. 

In  the  late  cases  surgical  section  of  the  proper  posterior  roots  may  be 
of  great  service  in  overcoming  the  spastic  states.     (Figs.  348  and  349). 


Fig.  348. — Before  operation.     Fig.  349. — After  operation. 

Figs.  348  and  349. — Little's  disease.    Spastic  diplegic  type  from  motor  area  destruction. 

Posterior  root  operation.     (Kamamura.^) 

Traumatic  late  apoplexy  is  a  special  variety  in  which  degenerative 
processes  complicate  the  picture.  The  patients  may  develop  the 
signs  of  hemorrhage,  usually  intracranial,  several  hours  or  days  after 
the  injury  (larvated  apoplexy.)  Such  cases  are  to  be  diagnosed  with 
much  caution.  Senility,  pronounced  arteriosclerosis,  and  syphilis 
should  be  excluded  .^ 

2.  Inflammation  of  Dura  (Pachymeningitis) .^(A)  Pachymeningitis 
Externa. — Following  severe  trauma,  purulent  processes  of  the  frontal 
sinuses,  the  middle  ear,  mastoid,  erysipelas,  caries,  or  osteomyelitis, 
one  occasionally  observes  an  inflammation  of  the  external  surface  of 
the  dura  of  the  cerebrum.  It  is  usually  localized.  A  similar  process 
may  be  present  in  the  spinal  dura. 

Symptoms. — These  are  usually  hidden  in  those  of  the  causative 
lesion  and  vary  with  the  acuteness  and  extent  of  the  process.   Localized 

J  Surg.,  Gynec.  and  Obst.,  August,  1918,  vol.  xxvii.  No.  2,  p.  131. 

2  Stadelmann:  Deut.  med.  Wchnschr.,  1903;  Allen:  Jour.  Nerv.  and  Ment.  Dis.,  1909. 


PACHYMENINGITIS  609 

cerebral  or  spinal  pains,  muscular  twitching,  spasms,  marked  scalp 
tenderness  to  percussion,  and  slight  rise  in  temperature  are  the  chief 
signs.  At  times  symptoms  of  localized  pressure  of  the  cerebrum  or  of 
the  cord,  resembling  tumor,  are  observed. 

Treatment. — The  treatment  is  that  of  the  causative  factors. 

{B)  Pachymeningitis  Interna  Simplex.— This  may  consist  of  a 
localized  or  circumscribed  purulent  exudate,  giving  in  the  main  the 
symptoms  of  a  brain  abscess  or  a  brain  tumor.  In  rare  instances 
(pneumonia,  etc.),  a  secondary  pseudomembranous  productive  inflam- 
mation occurs. 

(C)  Pachymeningitis  Interna  Hemorrhagica. — This  consists  of  a 
chronic  congestion  in  the  internal  layers  of  the  dura,  associated  with 
repeated  small  hemorrhages  and  the  organization  of  a  new  membrane 
which  tends  to  extend  and  thicken.  It  is  by  no  means  infrequent^  and 
gives  rise  to  chronic  psychoses  of  an  illy  defined  t;v'pe. 

History. — IMorgagni  noted  the  affection  in  the  eighteenth  century, 
Baillarger^  followed  the  older  authors  in  assuming  it  to  be  a  primary^ 
hemorrhage,  with  new  membrane  tissue  formation.  Heschl  and  Vir- 
chow^  first  pointed  out  the  inflammatory'  nature  of  the  productive 
inflammation,  and  the  consequent  hemorrhage  due  to  the  rich  forma- 
tion of  new  bloodvessels.  Jores  and  modern  authors  support  these 
views. 

Occurrence,  Pathology. — Usually  a  disorder  of  advanced  years, 
hemorrhagic  pachymeningitis  may  be  found  in  children.* 

It  is  extremely  frequent  in  general  paresis,  and  Blackburn  has 
found  it  accompanying  chronic  excited  and  chronic  depressed  states,  in 
patients  running  a  course  resembling  senile  dementia.  It  is  fre- 
quently an  alcoholic  complication.  It  is  seen  in  some  chronic  choreic 
affections.  Tuberculosis,  nephritis,  leukemia,  scorbutus,  Barlow's 
disease,  and  hemophilia  have  seemed  to  stand  in  causal  relations  in 
some  instances. 

In  the  initial  stages  a  productive  inflammation  causes  the  formation 
of  a  thin,  delicate,  yellowish-brown  membrane  on  the  inner  surface 
of  the  dura.  The  base  is  rarely  afl'ected,  the  temporal  regions  more 
often.  New  bloodvessels  form,  then  their  walls  give  way,  givmg  rise 
to  extravasations  of  blood.  The  process  of  new  membrane  formation 
and  bleeding  goes  on  hand  in  hand  until  the  whole  membrane  may  be 
one  or  more  centimeters  thick,  compressing  the  brain,  with  which  it  is 
usually  closely  united  by  new  connective-tissue  formation,  and  new 
bloodvessels,  and  causing  atrophies,  degenerations,  softening,  or  scle- 
roses of  the  near-lying  i)ortions  of  the  cortex.'^  The  process  may  extend 
to  the  spinal  cord. 

•  Blackburn:  Government  Hospital  Autopsy  Reports. 
^  Maladies  du  Cerveau,  1854. 

'  Wiirzburger  Verhandlungen,  1856,  p.  134. 

*  Goppert:  Jahr.  f.  Kinderheilk.,  1905,  Ixi,  51;  Freund:  Monat.  f.  Kinderheilk., 
1909,  vii. 

^  Jores  u.  Laurent,  Ziegler's  Beitrage,  xxix. 
39 


610  DISEASES  OF  THE  MENINGES 

Symptoms. — The  disorder  may  be  present  for  years  without  symp- 
toms. In  paresis  it  may  not  add  any  definite  symptoms  to  the  under- 
lying disorder,  or  it  may  cause  a  number  of  compHcating  pressure 
pictures.  When  the  process  has  advanced  to  a  definite  extent  both 
general  and  local  symptoms  show  but  are  so  indefinite  as  to  defy  diag- 
nosis. Gradually  increasing  head  discomfort,  headache,  often  severe, 
some  nausea  or  vomiting,  irregular  periods  of  confusion  or  distress 
in  the  head,  with  at  times  mild  delirious  excitement,  are  among  the 
more  characteristic  earlier  signs. 

With  rapid  extravasations,  acute  pressure  symptoms  may  develop, 
with  epileptiform  convulsions,  hemiparesis,  comatose  states  with  slow 
pulse,  aphasias,  irregularities  in  gait  of  ataxic,  diplegic  nature.  It  is 
very  characteristic  that  these  may  be  transitory.  Persistent  mono- 
plegias, with  Jacksonian  attacks  involving  irregularly  distributed 
muscle  groups  may  be  early  signs.  Often  persisting  stereotyped 
muscle  movements,  chewing,  automatic  arm,  hand,  or  leg  movements 
may  be  the  signs  of  a  local  irritative  lesion.  Constantly  putting  the 
hands  to  the  head  was  considered  suggestive  of  pachymeningitis  by 
Fiirstner.  It  is  a  frequent  sign  in  paresis.  The  eye  muscles  are  rarely 
involved  (conjugate  deviations,  nystagmus),  and  still  more  rarely  one 
finds  involvement  of  the  cranial  nerves  at  the  base. 

Papillitis,  or  choked  disk,  is  often  present.  The  pupils  are  not 
infrequently  irregular,  occasionally  myotic  in  the  early  stage,  dilated 
in  coma,  and  at  times  immobile  to  light  and  accommodation.  Argyll- 
Robertson  may  be  present  with  or  without  positive  Wassermann. 
Increased  tendon  reflexes  show  the  presence  of  irritation  of  the  motor 
cortex,  clonus  and  the  Babinski  reflex  being  occasionally  present,  and 
at  times  coming  and  going.  Irregular  but  inconstant  temperature 
changes  are  often  present. 

Course. — This  varies  considerably,  is  usually  chronic,  shows  remis- 
sions and  exacerbations.  At  times  the  patient  recovers  completely; 
again  the  disorder  is  progressive  and  may  bring  about  death  after  a 
long  psychotic  period  of  irregular  excitement  or  depression. 

Diagnosis. — It  must  be  separated  as  a  primary  and  as  a  secondary 
process  in  alcoholism,  paresis,  cerebrospinal  syphilis,  etc.  Brain  tumor, 
abscess,  hydrocephalus,  sinus  thrombosis,  leptomeningitis,  apoplexy 
are  to  be  borne  in  mind.  Traumatic  meningitis  must  be  excluded 
if  an  antecedent  trauma,  even  of  mild  grade,  such  as  falling  from  the 
bed,  in  bath  tub,  hard  crack  on  the  head  from  running  into  door,  etc., 
should  have  occurred. 

Neisser's  procedure  of  brain  puncture  is  often  desirable  in  those 
patients  that  give  signs  of  brain  tumor,  brain  abscess,  etc. 

Therapy. — In  acute  progressions,  local  bleeding  is  advisable.  Hydro- 
therapy with  stimulation  of  elimination  is  useful.  Brain  puncture  has 
been  of  service,  also  lumbar  puncture  in  children.  Abstinence  from 
alcohol  is  imperative.  Mercury  in  the  positive  Wassermann  cases 
is  indicated.  The  pains  are  often  relieved  by  analgesics,  and  by 
warm  baths, 


DISEASES  OF  THE  ARACHNOID  AND  PIA  611 


DISEASES  OF  THE  ARACHNOID  AND  PIA.      LEPTOMENINGITIS. 

1.  Acute  Leptomeningitides  (Cerebrospinal  Meningitides). — The 
studies  of  recent  years  particularly  those  which  have  come  from  the 
camps  during  the  mobilization  of  troops  have  shown  a  host  of  causes 
for  acute  inflammation  of  the  cerebral  and  spinal  arachnoid  and  pia. 
These  vary  very  widely  as  to  severity ^ — simple  meningism  to  the 
gravest  forms  of  general  meningeal  involvement — epidemic  cerebro- 
spinal meningitis,  epidemic  polio-myelo-encephalitis,  general  SA-philitic 
meningomyelitis,  etc.  It  becomes  impossible  to  present  a  logical  classi- 
fication of  these  disorders,  either  from  the  etiological,  pathological,  or 
clinical  standpoint.  In  general  only  the  more  circumscribed  types  of 
meningitis  will  be  considered  here.^ 

Causes. — These  are  many.  Trauma  is  one  of  the  most  important 
and  the  menmgitides  from  skull  and  brain  wounds  were  common 
during  the  war  1914-1918.  Such  trauma  may  act  either  through  direct 
infection,  as  by  compound  fracture,  or  secondarily  bring  about  a  septic 
meningitis,  through  hemorrhage,  thrombosis,  and  subsequent  infection. 

Traumatic  early  and  late  meningitides  appear.  Many  tuberculous 
leptomeningitides  arise  from  trauma.  Occasionally  one  meets  with 
late  pundent  meningitides,  due  to  old  encapsulated  abscesses,  old 
projectiles  (bullets,  splinters,  etc.),  old  fractures  of  lamina  cribrosa. 

Infection  from  suppurative  processes  in  the  neighborhood  is  one 
of  the  most  frequent  causes  of  the  type  of  meningitides  under  con- 
sideration, and  chief  of  these  is  otitic  meningitis.  Suppurative  otitis 
may  lead  to  intra-  or  extracranial  abscess,  sinus  phlebitis,  thrombosis, 
direct  infection,  infection  through  labyrinth,  through  mastoid,  etc. 
Serous  meningitis  may  also  have  an  otitic  origin. 

Nasal  and  frontal  sinus  infection  gives  rise  to  a  small  number  of 
these  meningitides;  they  may  be  purulent  or  serous.  Operations  upon 
the  nose  may  be  complicated  by  meningitis. 

Facial  erysipelas  occasionally  is  a  cause;  rarely  facial  furunculosis. 

Specific  organisms  give  rise  to  specific  types  of  meningitis.  Among 
these  are  measles,  mumps,  scarlet  fever,  varicella,  typhoid,  gonorrhea, 
syphilis,  pneumonia,  diphtheria.  The  influenza  bacillus  is  an  extremely 
important  organism  in  this  respect,  often  giving  rise  to  severe  epidemics 
of  meningitis.  Malaria,  yellow  fever,  anthrax,  leprosy,  actinomycosis, 
pneumonia,  whooping-cough  each  may  be  the  exciting  agents.  Septic 
extension  from  acute  articular  rheumatism  is  a  factor.  Tuberculosis 
is  a  large  item.  The  ej)idemic  type  due  to  the  Diplococcus  intracel- 
lularu  is  one  of  the  most  characteristic  of  the  types.  Old  abscess  for- 
mations in  the  lung,  liver,  bladder,  prostate,  teeth  pockets,  etc.,  all 
may  give  rise  to  a  meningitis. 

Occasionally  one  ascribes  certain  meningitides  to  chemical  poisons, 
diabetes,  lead,  gout,  are  among  these. 

>  Recent  monographs  are  by  Schultze  (Nothnagel)  and  Finkelnburg,  Handbuch  d. 
J»Ieurologie,  ii,  1090,  in  both  of  which  complete  bibliographies  are  to  be  found, 


612 


DISEASES  OF   THE  MENINGES 


Symptoms. — These  show  certam  variations  according  to  the  mode 
of  infection  and  the  type  of  infecting  agent.  Since  simple  purulent 
meningitis,  tuberculous  meningitis  (usually  a  mixed  infection)  and 
epidemic  cerebrospinal  meningitis  are  the  chief  infections,  the  following 
description  will  apply  to  these  and  no  attempt  will  be  made  to  cover 
the  symptomatology  of  the  entire  group. 

Headache. — This  is  one  of  the  earliest  signs,  and  is  frequent,  usually 
increasing  in  intensity  as  the  disease  progresses.  It  is  mostly  diffuse, 
but  may  first  appear  in  the  occipital  region,  or  in  the  forehead.  The 
slightest  movement,  percussion,  etc.,  increases  it. 

Stiffness  of  Neck. — This  is  a  striking  symptom  and  develops  early, 
sometimes  preceding  the  headache.  Such  early  stiffness  may  or  may 
not  be  apparent  to  the  patient,  but  comes  out  on  careful  examination; 
and  is  not  infrequently  accompanied  by  painful  pressure  points  over 
the  cervical  vertebrae.  When  well  developed  the  least  movement  of 
the  neck  is  painful  and  the  attitude  of  the  patient  on  movement  is 
striking.  In  young  children  it  is  not  infrequently  absent,  and  in  adults 
in  chronic  cases.  It  is  a  further  characteristic  that  this  stiffness  and 
painfulness  shows  considerable  variation  even  during  the  twenty-four 
hours. 


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Hyperesthesia. — The  skin,  the  muscles,  the  joints,  can  hardly  bear 
the  slightest  touch  or  pressure,  and  sensitiveness  to  light  and  to  sound 
are  early  signs  of  meningeal  irritation.  Occasionally  the  sense  of  smell 
is  extremely  acute. 

Nausea  and  Vomiting. — These  are  common  initial  symptoms  (80  per 
cent.).    Occasionally  the  vomiting  persists.    Early  vertigo  is  frequent. 

Mental  Sy7nj)toms. — These  occur  early,  particularly  in  children,  and 
more  especially  in  tuberculous  meningitis.  The  patients  become 
peevish,  throw  their  toys  away,  are  capricious,  their  attention  varies. 
They  not  infrequently  have  mild  delirium  early,  especially  at  night, 
or  are  confused.  Very  young  children,  one  to  three  years,  often  show 
less  mental  iuA^olvement.  Older  patients  are  excited,  sleep  badly,  are 
restless,  mutter  in  their  sleep,  or  have  well-marked  delirium.  Later  a 
semicoma  may  gradually  develop,  with  variations  in  intensity  from 
slight  confusion  to  a  confused  delirium.     Marked  ups  and  downs 


DISEASES  OF  THE  ARACHNOID  AND  PI  A  613 

characterize  the  epidemic  forms,  but  some  degree  of  confusion  or  coma 
is  more  or  less  constant. 

Temperature. — The  patients  practically  always  show  a  rise  in  tem- 
perature. High  temperatures  (104°  to  106°  F.)  usually  characterize 
the  purulent  meningitides ;  often  preceded  by  chill.  Such  tempera- 
tures may  remain  high,  or  not  infrequently  show  remissions.  The 
tuberculous  meningitides  usually  show  a  lower  curve. 

Motor  Irritation,  or  Paralytic  Phenomena. — Cramps  and  epileptiform 
con\Tilsions  occur  more  often  in  the  early  stages  with  children  than 
with  adults.  They  sometimes  are  very  persistent.  Circumscribed 
spasms,  Jacksonian  in  type,  are  not  infrequent.  Occasionally  there 
are  choreic,  athetoid,  or  tremor  movements,  which  come  and  go,  or 
are  continuous. 

IVIuscular  rigidity  is  an  early  and  persistent  sign,  showing  early  in 
the  neck,  later  in  the  back,  with  opisthotonos,  or  stiff  lordosis  postures. 
These  muscular  rigidities  are  often  subject  to  considerable  fluctuation 
with  the  degree  of  internal  or  external  hydrocephalus.  Lumbar  punc- 
ture often  influences  them  greatly. 

Kernig's  sign  is  frequent,  and  is  based  upon  this  generalized  muscular 
rigidity  (reflex,  spasmodic  contraction  of  rectus  femons). 

Paretic  signs,  monoplegia,  or  hemiplegia,  are  less  frequently  met 
with,  and  then  more  in  the  late  stages.  Such  indicate  localized  puru- 
lent processes,  with  abscess  formation  in  the  motor  area,  and  are 
usually  accompanied  by  con^^Jlsions.  Spinal  pareses  (paraplegias)  are 
rare. 

Speech  disturbances,  usually  cortical,  and  aphasias  are  occasionally 
observed,  more  particularly  in  tuberculous  meningitides. 

Cranial  Nerve  Sign^. — Disturbances  of  the  cranial  nerves  are  among 
the  most  characteristic  signs  in  the  meningitides.  The  pupils  are 
often  unequal;  frequently  markedly  myotic  in  the  beginning,  they 
show  irregular  widening  later.  With  increasing  coma  they  usually 
widen,  and  react  sluggishly  or  not  at  all.  Convergence  reactions  are 
less  easily  tested,  but  also  show  slowness. 

Eye  palsies  are  frequent.  Mild  ptosis,  unilateral  or  bilateral,  diver- 
gent and  convergent  strabismus — the  abducens  is  particularly  prone 
to  disturbance — and  double  vision  is  often  present.  These  eye  palsies 
vary  from  hour  to  hour  in  extent,  and  in  intensity,  becoming  perma- 
nent in  the  long  protracted  cases  particularly.  Protrusion  of  the  eye- 
ball is  a  rare  sign,  nystagmus  frequent. 

Papillitis  is  very  frequent,  and  early;  optic  atrophy  is  common  (18 
to  25  per  cent.).  Permanent  blindness,  however,  is  rare.  The  trigem- 
inus is  rarely  involved. 

Facial  palsies  are  very  frequent,  but  are  usually  incomplete,  vary 
from  day  to  day,  and  are  rarely  permanent. 

Hearing  is  frequently  affected.  Complicating  otitis,  and  laby- 
rinthitis often  leave  these  patients  deaf.  It  is  a  frequent  complication 
hi  the  second  and  third  weeks  of  the  disease. 

The  vagus  involvement  causes  pulse  and   respiratory  anomalies 


614  DISEASES  OF  THE  MENINGES 

which  are  also  influenced  by  central  processes.  The  pulse  is  initially 
hastened  in  practically  all  forms,  and  usually  remains  rapid  in  the 
later  stages,  save  in  those  forms  of  less  acute  progress,  notably  in 
tuberculous  meningitis,  where  it  is  often  slow,  especially  after  the 
second  week  of  the  disease  (75  per  cent. — Heubner),  Marked  irregu- 
larity of  the  cardiac  rhythm  is  conspicuous. 

The  respiration  is  also  irregular  and  Cheyne-Stokes  type  is  frequent 
in  the  severe  purulent  and  tuberculous  types  toward  the  end. 

Reflexes. — The  tendon  reflexes  are  usually  somewhat  exaggerated, 
particularly  in  the  early  stages,  but  may  be  missing  from  the  begin- 
ning (medullary  and  ventricular  pressure).  With  the  advance  of  the 
disease  they  may  be  missing.  Marked  variations  and  irregularities  are 
to  be  expected,  thus  lost  knee-jerks  may  be  associated  with  ankle- 
clonus  and  Babinski.  This  latter  is  a  very  frequent  early  sign  and  later 
disappears.  It  should  be  remembered  that  it  is  normally  present  in 
young  children  up  to  six  to  eight  months  of  age,  and  hence  is  to  be 
neglected  in  diagnosis  in  young  children.^ 

Luvibar  Puncture. — This  is  of  primary  importance.  The  pressure 
is  initially  increased.  Later  this  is  not  marked  as  the  fluid  is  purulent. 
In  the  ordinary  purulent  meningitides  the  fluid  is  usually  cloudy, 
sometimes  only  microscopically  so,  again  markedly  purulent,  in  which 
the  specific  organisms  may  be  found  by  proper  methods.  In  tuber- 
culous meningitis  the  fluid  is  less  apt  to  be  cloudy,  especially  early, 
but  by  proper  technic  the  bacillus  is  found  (33  per  cent,  early  stages, 
50  per  cent,  with  pressure  signs,  100  per  cent,  in  paralytic  stages 
— Pf  aundler) . 

Cytologically^  one  finds  that  in  purulent  meningitis  there  is  a  pre- 
ponderance of  pol>Tiuclear  leukocytes,  while  in  tuberculous  meningitis 
the  l^Tnphocytes  are  increased.  This  is  not  an  absolute  rule  and  there 
are  variations  during  the  course  of  the  disease.  The  cytological  find- 
ings should  be  checked  up  with  the  clinical  ones.^ 

Irregular  Symptoms. — Herpes  is  not  infrequent  (75  per  cent.,  save 
in  young  children)  in  the  epidemic  form,  and  usually  appears  within 
the  first  week  (two  to  five  days).  It  is  most  frequent  about  the  nose, 
lips,  and  forehead.  It  does  not  persist  long  as  a  rule.  Other  skin 
eruptions  are  not  infrequent,  especially  the  rose-colored  spots  of  the 
diplococcus  types.  Eruptions  resembling  measles,  scarlet  fever,  urti- 
caria are  occasional,  while  erythemas  and  hemorrhagic  spots  are  rare. 

Gastric  disturbances  are  frequent,  especially  constipation.  Diarrhea 
may  be  an  initial  symptom  in  young  children. 

Tonsillar  and  pharyngeal  redness  and  soreness  are  not  infrequent 
in  the  epidemic  t^pes,  and  bronchitis  is  often  present  in  the  later  stages. 

Course  and  Prognosis.— Whereas  most  of  the  tj^pes  of  leptomeningitis 
show  much  the  same  general  symptomatology,  it  is  more  particularly 

1  See  Goppert  for  careful  study  of  reflexes;  Klin.  Jahr.,  xv,  423;  Berl.  klin.  Wchnschr., 
1905,  21,  22. 

2  Schonbroii:  Klin.  Vort.,  384;  Med.  Klin.,  1906,  p.  593. 

3  Consult  Rehm.  u.  SchottmuUer's  valuable  atlas  of  cerebrospinal  fluid  findings,  1913. 


EPIDEMIC  CEREBROSPINAL  MENINGITIS  615 

in  their  developmental  course  that  the  differences  appear.  These 
variations  may  be  viewed  as  fundamentally  due  to  specific  differences 
in  the  microorganisms  in  question,  although  it  may  be  said  that  in  a 
number  of  instances  the  only  differences  observed  by  the  best  clinical 
observers  have  been  those  of  the  microscopic  slide,  or  the  test-tube. 
Hence  it  may  be  stated  that  under  certain  circumstances  the  patholog- 
ical fact  that  the  patient  has  a  meningitis  governs  the  entire  picture 
and  all  forms  are  precisely  alike,  but  in  the  main  it  is  true  that  specific 
variations  exist,  and  should  be  sought  for  when  facing  any  particular 
case  of  leptomeningitis. 

In  general,  fairly  sharp  distinctions  can  be  made  between  (1)  the 
infectious,  (2)  the  epidemic,  (3)  the  tuberculous,  and  (4)  the  serous 
forms. 

1.  Infectious  Meningitis. — Here  the  initial  disturbance  in  the  ear, 
nose,  mastoid,  frontal  sinus,  fracture,  etc.,  precede,  and  its  symptoms 
often  hide  those  of  the  suppurative  meningitis  that  follows.  The 
onset  is  usually  acute.  Headache,  local  or  diffuse,  is  early.  The 
temperature  usually  mounts  rapidly  to  104°  F.  or  105°  F.,  with  initial 
chill;  the  pulse  and  respiration  are  rapid.  There  is  photophobia  and 
hyperacusis  within  a  few  days.  The  mental  signs  come  on  soon.  Con- 
fusion, somnolence,  or  coma  are  frequent,  often  punctuated  by  active 
delirious  intervals.  Lumbar  puncture  usually  shows  a  purulent  fluid. 
The  tendon  reflexes  are  usually  increased  and  the  cranial  nerve  signs 
are  marked  within  the  first  week.  No  special  skin  eruptions  are  noted. 
Neither  tache  cerebrale  nor  dermographism  are  marked. 

With  irregular  septic  temperature,  increasing  coma  or  delirium, 
increasing  signs  of  local  pressure  or  destruction,  con^'ulsions,  and 
paralyses  the  more  serious  cases  end  fatally  within  two  to  five  days  in 
children,  or  one  to  two  weeks  in  adults. 

Other  patients  show  less  grave  symptoms,  run  a  subactive  course 
and  recover  in  three  to  four  weeks,  but  in  general  the  prognosis  is 
unfavorable.  Those  that  get  well  probably  have  a  circumscribed 
process. 

2.  Epidemic  Cerebrospinal  Meningitis. — Known  for  centuries,  first 
recognized  as  epidemic  in  1805  in  Switzerland,  in  1806  in  IVIassachu- 
setts,  this  form  has  been  extensively  observed  the  world  over.^  Its 
epidemic  onset  is  usually  verv  insidious,  and  spring  and  winter  are 
the  months  of  predilection  in  the  north  temperate  zones.  It  is  now 
endemic  in  large  cities.  Children  under  ten  are  more  prone  to  the 
infection.  The  exciting  agent  is  the  Meningococcus  intracelhdaris 
of  Weichselbaum^  of  which  a  series  of  types  have  been  isolated  varsing 
in  their  virulence.  The  disease  is  contagious,  the  contagion  being 
possibly  carried    in    the  nasopharynx.''     Abortive,   subacute,   acute, 

1  See  Report  of  State  Board  of  Health,  Massachusetts,  1898,  for  extensive  review  with 
literature  to  that  date.  Articles  of  Finklenburg,  loc.  cit.,  for  later  literature  and  current 
literature  in  Jour.  Nerv.  and  Ment.  Dis.,  1918,  1919. 

2  Fortschritte  d.  Mod.,   1877,  p.  622. 

'  See  Fildes  and  Baker:  British  Med.  Jour.,  October  5,  1918. 


616 


DISEASES  OF   THE  MENINGES 


chronic,  and  fulminating  cases  are  recognized,  with  every  possible 
intermediary  type.  Recent  studies  among  the  armies  in  Europe  and 
in  the  United  States  have  contributed  very  widely  to  the  knowledge 
of  the  possibilities  of  this  disorder  which  has  been  prevalent  in  many  of 
the  large  camps.  See  Index  Medicus,  War  Supplement,  1914-1917, 
p.  60. 

The  malignant  or  fulminating  cases  may  end  fatally  in  twenty-four 
hours,  with  headache,  nausea,  vomiting,  delirium,  coma,  convulsions, 
stiff  neck,  high  temperature,  rapid  pulse,  Cheyne-Stokes  respiration 
and  death  from  acute  toxemia. 

Abortive  cases,  which  are  more  often  found  in  adults  than  in  children, 
show  rudimentary  meningeal  signs.  Headache,  backache,  nausea  and 
some  vomiting  with  marked  stiffness  of  the  neck  occur,  or  the  patients 
may  only  have  slight  vertigo,  malaise,  and  nausea,  and  keep  on  with 
their  work.  Fever  is  usual  and  occasionally  deafness  develops  in 
these  mild  attacks. 


Fig.  351. 


-Trend  of  blood  curve  in  cerebrospinal  meningitis  in  favorable  and  unfavorable 
;ases.    A'',  neutrophiles ;  L,  lymphocytes;  E,  eosinophiles.     (Rusca.) 


The  usual  subacute  or  acute  types  show  a  latent  period  averaging 
three  to  five  days,  consisting  of  irregular  backache,  headache,  slight 
vertigo,  and  sweating,  with  signs  of  a  nasopharyngitis.  Then  a  chill 
with  slight  rise  in  temperature  accompanied  by  vomiting  indicates  the 
onset.  Children  become  restless,  cry,  and  are  very  irritable.  Usually 
within  twenty-four  hours  meningeal  signs  appear.  Stiff  neck,  headache, 
mental  confusion,  are  present;  not  infrequently  convulsions  appear  in 
children.  Photophobia,  hyperacusis,  excessive  sensitiveness  to  pres- 
sure, and  hj'pertonicity  then  show  themselves  with  Kernig's  sign 
and  increased  tendon  reflexes.  The  cranial  nerve  signs  then  develop. 
Herpes  labialis  is  frequent  from  the  second  to  the  fifth  day,  and  a  few, 
macular  eruptions  (spotted  fever)  may  appear.  Other  skin  eruptions 
develop  in  the  first  week.  The  cerebrospinal  fluid  shows  a  characteris- 
tic picture,    (See  Rehm  and  Schottmiiller.)    The  blood  findings  afford 


EPIDEMIC  CEREBROSPINAL   MENINGITIS  617 

some  clue  as  to  the  prognosis.  The  leukocyto  is  is  characterized  more 
or  less  definitely  by  an  increase  in  the  polymorphoneutrophile  cells 
which  in  the  patieats  with  a  good  prognosis  tend  to  diminish  and  are 
accompanied  by  an  increase  in  the  lymphocytes,  with  a  slight  increase 
in  eosinophile  cells.  In  the  unfavorable  cases  this  crossed  curve  does 
not  take  place  and  the  eosinophile  cells  disappear  entirely.^ 

The  coma  contmues,  shows  considerable  variation  in  intensity,  and 
the  patient  gradually  improves,  or  shows  increasing  signs  of  pressure, 
convulsions,  palsies,  and  dies  within  seven  to  fourteen  days,  after 
ineffectual  attempts  at  maintaining  nourishment. 

Other  patients  show  a  much  more  chronic  course,  lasting  weeks  or 
months,  with  considerable  variation  in  the  intensity  of  the  headache, 
coma,  or  delirium,  with  eye  palsies,  mono-  or  hemiplegia,  marked 
muscular  rigidities,  which  later  may  leave  obstinate  rigidities  with  or 
without  arthritis,  constant  emaciation,  and  variable  temperature 
until  death  takes  place,  often  with  increasing  size  of  the  head — hydro- 
cephalus and  signs  of  increased  cerebral  pressure. 

In  other  more  favorable  cases,  periods  of  clearness  or  of  betterment 
become  longer  and  more  pronounced,  intermingled  with  the  severer 
symptoms  already  outlmed.  The  variability  in  clinical  course  is 
enormous,  but  the  prognosis,  up  to  the  time  of  the  introduction  of 
the  Flexner  serum,  was  distinctly  unfavorable,  the  mortality  ranging 
from  50  to  80  per  cent.  Apparently  mild  cases  often  die  and  some 
extremely  ill  children  recover.  Recovery  with  defect,  either  deafness, 
blindness,  cranial  nerve  palsies,  monoplegias,  hemiplegias,  or  mental 
defects,  are  not  infrequent. 

Treatment. — Since  a  specific  antimeningitic  serum  has  been  elabo- 
rated by  Flexner^  the  prognosis  has  been  much  improved.  The  mor- 
tality has  fallen  to  25  per  cent,  in  some  of  the  recent  epidemics.^  The 
best  results  are  obtained  in  children  of  from  five  to  ten  years. 

The  effects  of  serum  treatment  are  often  seen  very  soon — twenty- 
four  hours  after  injection.  The  Kernig  sign  and  stiffness  of  the  neck 
persist,  however.  The  attenuation  of  the  symptoms  is  very  marked 
in  many  of  the  cases,  as  well  as  shortening  of  the  disease.  Lysis  is 
the  usual  mode  of  recovery  of  non-serum-treated  cases.  Crises  occur 
much  more  often  in  serum- treated  cases  (25  per  cent.).  Furthermore, 
the  permanent  sequelae  of  the  disease  are  markedly  reduced  by  the 
serum  treatment.  In  the  meningitides  of  the  camps  1917-191S, 
polyvalent  sera  were  widely  employed  to  advantage  as  at  least  four 
meningococcus  types  may  be  present  in  differing  proportions. 

The  general  treatment  will  be  considered  with  that  of  the  other 
forms  of  meningitis. 

3.  Tuberculous  Meningitis.— This  form  was  first  isolated  about  1830 
(Rilliez  et  Barthez  and  Robert  Whytte),  although  one  can  see  its 
chief  features  in  the  Nosologie  of  Sauvages,  written  in  1763.    In  this 

'  Rusca:  Deutsch.  Arch.  f.  klin.  Med.,  1911,  103. 

2. Jour.  Exp.  Med.,  1907. 

'Flexner:  International  Clinics,  1909;  Jour.  Am.  Med.  Assn.,  October  30,  1900. 


618  DISEASES  OF   THE  MENINGES 

form  the  onset  is  characteristically  subacute  or  chronic,  one  or  two 
weeks,  exceptionally  months,  and  is  almost  invariable  secondary  to 
tuberculosis  in  other  organs.  General  symptoms,  such  as  loss  of 
appetite,  irritability,  loss  of  flesh,  general  malaise,  with  loss  of  desire 
to  play,  fretfulness  and  ready  fatigability  are  the  precursors.  Head- 
ache and  dizziness,  with  irregular  fever  movement  is  then  observed, 
and  occasional  dreamy  states,  during  which  the  patient's  manner  is 
peculiar. 

Then  gradually,  or  suddenly,  the  patient's  manner  becomes  much 
changed;  mild  delirium  or  coma  develops,  and  convulsions  appear. 
The  patients  lie  in  bed,  are  restless,  rolling  from  side  to  side  with  sharp 
cries  or  whimpers,  and  frequent  placing  of  the  hand  to  the  head. 
Passive  motions  of  the  head  forward  invariably  bring  out  resistance 
and  pain.  The  sensitiveness  of  the  skin  to  pressure  or  touch  is  marked, 
and  hypertonus  with  muscular  twitchings  and  Kernig's  sign  are  present. 

The  temperature  ranges  from  102°  F.  to  104°  F.,  and  is  usually  less 
marked  than  in  the  suppurative  or  epidemic  types.  Lumbar  puncture 
in  the  first  week  usually  gives  a  clear  fluid  under  pressure,  with  excep- 
tionally the  tubercle  bacillus  or  pus  elements. 

The  cranial  nerve  signs  may  then  develop  in  the  second  to  third 
week,  and  show  more  ups  and  downs  than  is  usually  present  in  the 
other  types.  Localized  pressure  signs  with  hemiplegias  or  monoplegias 
then  develop  and,  not  infrequently,  the  previously  increased  tendon 
reflexes  diminish  or  are  lost. 

The  patients  gradually  get  worse,  convulsions  are  frequent,  and 
rigidity  is  followed  by  flaccidity,  and  the  patient  dies  with  signs  of 
marked  exhaustion,  sometimes  with  agonal  rise  of  temperature  imme- 
diately preceding.  The  whole  attack  may  terminate  within  a  compara- 
tively short  time,  two  or  three  weeks,  or  may  run  for  months. 

Atypical  forms  are  especially  frequent  in  adults  such  as  apoplecti- 
form onsets,  with  aphasia,  monoplegia,  or  hemiplegia.  The  picture 
may  be  that  of  a  toxic  delirium.    The  prognosis  is  bad. 

4.  Serous  Meningitis.- — Dietl,  in  1846,  first  isolated  this  type,  which 
is  of  uncertain  origin,  and  characterized  by  varying  grades  of  edematous 
exudate  with  infiltration  of  round  cells.  The  symptoms  are  usually 
those  of  a  mild  meningitis.  Headache,  stiff  neck,  marked  sensitive- 
ness are  constant,  whereas  convulsions  and  signs  of  pressure  are  less 
frequent.  Again  the  disorder  may  be  acute  and  very  severe,  with  high 
temperature  and  signs  of  cranial  nerve  involvement.  The  usual 
course  is  less  stormy.  Papillitis  is  a  frequent  symptom,  and  should 
be  borne  in  mind  in  those  patients  in  whom  the  serous  exudate  is 
more  or  less  circumscribed,  giving  the  general  impression  of  a  brain 
tumor.  Lumbar  puncture  shows  increase  in  pressure  with  some 
lymphocytes. 

In  the  serous  meningitis  of  alcoholic  origin  (wet  brain,  meningo- 
encephalitis), there  is  usually  a  busy  delirium,  excessive  hyperesthesia, 
with  marked  twitching  of  the  muscles.  This  type  is  comparatively 
infrequent  but  is  seen  often  in  the  large  cities — Bellevue  Hospital 


SEROUS  MENINGITIS  619 

service  particularly — in  all  drinkers  of  long  standing  in  tHe  thirties.  It 
is  seen  following  a  very  hard  drinking  spell  and  often  is  accompanied 
by  delirium  tremens  (q.  v.).  The  patients  become  semiconscious,  in  a 
muttering,  fairly  busy  delirium  with  hallucinations  of  sight  and  hearing 
which  come  and  go.  There  is  marked  hyperesthesia  and  the  pupils  are 
contracted.  The  coma  is  apt  to  deepen,  the  temperature  rises,  the 
tongue  is  very  heavily  brown-coated.  Involuntary  evacuations  may 
occur.  Increasing  temperature,  104°  F.,  rapid,  irregular  and  feeble 
pulse,  loss  of  tone  of  the  skin  and  muscles  all  point  to  a  lethal  termina- 
tion. Others  slowly  improve  and  recover  without  or  with  (Korsakov) 
defect. 

5.  SyphilUic  Menitigitis. — See  S^'philis  of  the  Brain.  . 

Differential  Diagnosis. — The  chief  disorders  that  may  be  confounded 
with  the  meningitides,  especially  in  the  beginning  of  the  disorder,  or 
in  mild  cases,  are  as  follows:  Internal  pachj-meningitis,  which  rarely 
gives  temperature  or  lumbar  puncture  findings.  Encephalitis,  and 
encephalomyelitis;  in  the  former  localizing  symptoms  develop  early, 
and  the  spinal  fluid  is  clear;  in  the  latter  flaccid  palsies  rapidly  develop, 
and  the  fluid  is  not  purulent.  Brain  abscess  may  be  complicated  by 
meningitis,  or  localized,  in  which  latter  case  the  localizing  symptoms, 
septic  course,  and  clear  spinal  fluid  are  of  aid. 

Infectious  sinus  thrombosis  may  resemble  meningitis  very  closely. 
There  is  a  greater  tendency  for  the  lower  cranial  nerves  to  be  involved, 
particularly  the  vagus,  hypoglossal  and  spinal  accessory.  The  spinal 
fluid  is  usuallyclear. 

Delirium  tremens  shows  an  active  occupation  delirium,  optic  hallu- 
cinations, marked  fine  tremors  and  little  temperature.  Hysteria  rarely 
shows  temperature,  and  many  of  the  organic  signs  described  by  earlier 
authors  as  found  in  hysteria  are  better  referred  to  as  organic  brain 
disease  with  hysterical  epiphenomena. 

Treatment. — This  must  first  be  prophylactic,  and  mvolves  general 
hygienic  precautions  in  tuberculosis;  prompt  aural  treatment  in 
otitis,  mastoiditis;  surgical  asepsis  of  wounds  in  all  head  operations. 
The  isolation  of  the  epidemic  tj-pes  is  advisable,  and  nasal  antisepsis 
imperative.  Quarantine  measures  are  important  and  are  best  con- 
trolled through  cultures  of  the  nasal  and  pharyngeal  mucus.  The 
active  treatment  is  surgical  for  most  of  these  forms,  especially  if  the 
symptoms  show  early  signs  of  being  circumscribed,  or  when  it  seems 
possible  to  get  at  an  initial  focus  of  infection,  as  in  the  various  septic 
infectious  forms — ear  disease,  etc. 

In  diffuse  general  meningitis  the  responsibilities  are  great,  and  it 
is  difficult  to  decide  in  the  individual  cases. 

Epidemic  types  are  best  treated  by  serum.  Surgical  treatment  of 
tuberculous  meningitis  has  not  yet  met  with  sufficient  success  to 
warrant  its  advocacy. 

Lumbar  puncture  has  given  ver\'  brilliant  results  in  some  patients, 
in  others  it  has  been  of  little  service.  With  the  development  of  acute 
hydrocephalus  it  is  indicated,  and  it  is  in  general  a  harmless  procedure. 


620  DISEASES  OF  THE  MENINGES 

The  punctures  may  be  made  frequently,  and  25  to  40  c.c.  of  fluid 
removed.  The  amount  remo\ed  should  be  controlled  by  the  pressure. 
Hess  (/.  c.)  speaks  of  the  Rusca  leukocyte  curve  as  affording  a  criterion 
for  lumbar  puncture.  The  drop  in  eosinophile  cells  and  the  failure  of 
the  lymphocytes  to  mount,  with  increased  fever,  these  are  indications 
for  puncture. 

Of  the  sera,  the  Kolle-Wassermann  in  septic  cases  has  given  good 
results.  Flexner's  serum  for  the  epidemic  tApes  has  been  mentioned. 
In  using  this  serum  a  lumbar  puncture  is  first  made,  and  from  10  to 
50  c.c.  of  fluid  allowed  to  drain  off,  the  amount  being  determined 
largely  by  the  pressure.  One  drop  in  from  three  to  five  seconds  is  a 
general  rule  for  determining  this.  Then  from  10  to  50  c.c.  of  the 
antimeningitic  serum  is  slowly  injected  by  gravity.  The  serum  may 
be  used  every  three  or  four  days.  Control  smears  of  the  organisms  in 
the  fluid  afford  some  clue  to  the  frequency  of  use  of  the  serum.  With 
numerous  bacteria,  active  cellular  exudates  indicate  more  frequent 
use  of  serum.  In  the  alcoholic  types,  prompt  and  thorough  catharsis 
should  first  be  used.  Diaphoresis  by  hot  packs  is  of  service.  Support- 
ing and  tonic  treatment  is  then  required. 

General  treatment  consists  in  keeping  the  patient  quiet.  Bromides, 
chloral,  veronal  and  other  mild  h^-pnotics  are  of  service.  H^'podermic 
use  of  morphine  or  hyoscine  may  be  necessary,  but  other  things  being 
equal,  is  undesirable.  The  continuous  hot  bath  is  especially  valuable 
in  delirious  patients. 

In  the  begmning  a  prompt  use  of  calomel  is  called  for.  The  room 
should  be  darkened  and  as  quiet  as  possible.  Pain  may  be  controlled 
as  far  as  possible  by  analgesics,  and  local  counter-irritants. 

Particular  attention  should  be  given  to  the  nourishment  and  rest 
of  the  patient.  Continuous  fussing  and  overnursing  are  to  be  avoided 
in  these  cases.  There  are  no  specific  remedies.  Uro tropin  in  large 
doses  may  be  tried,  as  it  is  partly  broken  do\sTi  into  formaldehyde  in 
the  cerebrospinal  fluid.    The  colloidal  silver  salts  are  disappointing. 

In  the  long,  tedious  convalescence  of  many  of  these  little  patients 
one's  ingenuity  is  taxed  to  stimulate  the  appetite,  provide  the  proper 
amount  of  outdoor  play,  and  to  strengthen  the  paretic  or  paralyzed 
muscles. 

2.  Chronic  Leptomeningitis. — This  is  usually  a  secondary  affection 
in  paresis,  senile  dementia,  chronic  lead  poisonmg,  and  chronic  pachy- 
meningitis; it  is  rare  as  a  primary  affection  save  as  s^'philitic  or  tuber- 
culous. 

Chronic  tuberculous  leptomeningitis  is  usually  of  the  convexity, 
generally  anterior,  involving  the  frontal  and  motor  areas.  It  behaves 
as  a  low,  mild-grade  meningitis,  often  with  intermittent  circumscribed 
symptoms,  simulatmg  those  of  a  brain  tumor. 

Traumatic  cases  often  show  chronic  vertigo,  pain'*,  epileptiform 
con\'ulsions,  aphasias,  nausea  and  vomiting,  and  gradual  mental 
involvement.  In  order  to  establish  a  diagnosis  of  trauma  these  signs 
should  stand  in  direct  relationship  to  the  injury,  and  should  not  be 


HYDROCEPHALUS 


621 


complicated  by  signs  originating  at  a  distance  from  the  site  of  the  injury 
or  from  other  causes  (Argyll-Robertson  from  syphilis,  for  instance). 

Chronic  meningeal  inflammations  are  occasionally  found  in  children, 
giving  the  signs  of  a  posterior  basilar  meningitis.  They  are  often 
accompanied  by  hydrocephalus.  Opisthotonos  is  frequent.  Many 
are  due  to  syphilis,  as  the  Wassermann  tests  show. 

Hydrocephalus. — An  accumulation  of  fluid  within  the  cranial 
cavities  takes  place  in  a  variety  of  aft'ections.  It  is  invariable  in  the 
acute  meningitides,  in  greater  or  lesser  degree;  in  tuberculous  menin- 
gitis it  is  often  extreme  and  comes  on  with  great  rapidity — acute 
hydrocephalus  being  practically  sjTionymous  with  a  tuberculous 
basilar  meningitis. 


Fig.  352. — Showing  marked  hydrocephalus.    Clinically  the  patient  showed  epileptic 
attacks  and  was  markedly  feeble-minded.     (Munson.) 

External  hydrocephalus  is  often  synonjTnous  with  serous  meningitis; 
as  an  entity  it  is  infrequent. 

Hydrocephalus  is  a  ver\'  frequent  complication  in  tumors  of  the 
brain,  particularly  in  tumors  of  the  third  ventricle,  the  pineal,  the 
corpora  quadrigemina,  and  those  causing  pressure  upon  the  aqueduct. 


622  DISEASES  OF  THE  MENINGES 

These  conditions  are  discussed  under  Brain  Tumor.  Ventricular  hydro- 
cephalus often  results  from  such  structural  anomalies  as  cephalocele, 
and  spina  bifida. 

In  children  one  may  find  a  primary  chronic  hydrocephalus  of  as  yet 
unsettled  pathology;  but  there  is  usually  a  chronic  ependymitis  present 
of  possible  hereditary  luetic  origin. 

One  to  five  pints,  even  quarts,  of  fluid  may  accumulate.  The  fluid 
is  clear,  slightly  alkaline  with  sp.  gr.  0.1005,  containing  the  earthy 
chlorides,  albumin,  phosphates,  and  occasionally  a  sugar-reducing  sub- 
stance. The  presence  of  high  percentages  of  albumin  and  many 
cellular  elements  indicates  a  more  active  inflammation.  As  a  result 
of  the  pressure  the  ventricles  widen,  the  septum  lucidum  is  displaced, 
the  cortex  thins,  the  infundibulum  dilates,  the  optic  chiasm  is  pressed 
upon.  Extreme  distention  may  convert  the  pallium  into  an  enormous 
cyst,  with  the  smallest  vestige  of  a  cortex.  The  thalamus  may  be 
flattened,  even  the  pons. 

The  cranial  bones  may  become  separated.  The  head  enlarges, 
usually  symmetrically,  at  the  vault  and  at  the  base.  In  older  children, 
however,  hydrocephalus  may  exist  without  these  changes  in  the 
position  of  the  cranial  bones  taking  place.  The  average  cranial  cir- 
cumference at  birth  is  fourteen  inches;  at  one  year  eighteen  to  nine- 
teen inches.  In  hydrocephalic  heads  this  is  increased.  As  a  rule  the 
congenital  types  show  the  largest  heads.  These  patients  are  rarely 
born  alive,  or  they  live  a  short  time  only. 

Symptoms. — Two  or  three  or  more  months  after  birth  it  is  noted 
that  the  child's  head  is  increasing  in  size  with  more  than  the  usual 
rapidity.  Somnolence  and  lethargy  are  frequent;  the  child  may  not 
be  able  to  hold  the  head  up.  A  whiny,  peevish,  irritability,  with 
frequent  sharp  cries  is  the  rule.  With  fairly  rapidly  increasing  internal 
pressure  the  coma  is  marked,  showing  great  variability,  however; 
vomiting  is  frequent,  the  hearing  is  affected,  also  the  sight;  choked 
disks,  spasticities,  with  usually  symmetrically  increased  reflexes. 
The  pupils  are  usuafly  contracted  and  sluggish  to  light  early.  With 
severe  grades  of  pressure,  there  may  be  extreme  dilatation.  Con- 
vulsions appear  and  death  results  in  from  three  to  six  months,  with 
signs  of  emaciation. 

In  the  cases  of  more  gradual  increase  of  intracranial  pressure  a 
marked  degree  of  accommodation  takes  place.  The  symptoms  are 
those  of  dulness  or  stupidity,  the  children  are  usually  less  bright,  the 
choked  disk  may  be  very  little  marked,  or  may  be  excessive  if  the 
bones  have  not  permitted  distention,  and  may  be  followed  by  atrophies; 
but  many  cases  recover  with  only  a  slight  degree  of  mental  reduction 
— weak-mindedness  or  only  stupidity.  There  is  a  proverbial  cheeri- 
ness  in  these  patients.  Some  few  riiake  total  recoveries,  and  may 
show  brilliant  mental  capacities. 

In  the  hydrocephalus  of  brain  tumor  in  adults,  with  its  up-and-down 
course,  somnolence  and  headache,  choked  disk,  dilated  pupils,  some- 


SUNSTROKE  623 

what  inactive  to  light,  are  characteristic.  A  cracked-pot  percussion 
note  is  often  very  characteristic  in  the  young  and  also  in  many  adults. 

Therapy. — Many  cases  are  hopeless  from  the  onset.  A  Wassermann 
test  should  always  be  made,  both  of  the  blood  and  cerebrospinal 
fluid,  for  the  double  purpose  of  determining  s^^hilis  or  other  inflam- 
matory disorder.  Many  cases  of  ependymitis  are  sj-philitic  in  origin. 
For  these  prompt  mercurial  treatment  is  indicated;  salvarsan  or  enesol 
are  useful.  Inunctions  are  also  valuable  in  the  less  rapidly  advancing 
cases.     lodin  therapy  is  advantageously  combined. 

Cerebral  irritative  phenomena  need  hot  baths  and  bromides. 

Tapping  is  frequently  of  service,  but  not  always.  Puncture  of  the 
corpus  callosum  may  prove  valuable.  Lumbar  puncture  is  not  prac- 
ticable to  relieve  pressure,  as  frequently  the  aqueduct  of  Sj'lvius  is 
blocked;  a  dry  lumbar  tap  is  of  diagnostic  service. 

SUNSTROKE. 

This  is  a  condition  brought  about  by  exposure  to  the  direct  rays  of 
the  sun  or  to  great  heat  from  any  source. 

The  se^'ere  cases  are  marked  by  profound  unconsciousness,  very  high 
temperature — 108°  to  110°  F.,  perhaps  by  convulsions,  delirium  and 
death.  The  milder  cases  are  so-called  cases  of  heat  exhaustion,  with 
general  prostration,  little  or  no  rise  in  temperature,  and  they  recover 
spontaneously.  These  patients  not  infrequently  show  marked  cardiac, 
renal,  or  other  organic  disease  which  undoubtedly  conditioned  the  onset 
of  the  symptoms  by  rendering  the  individual  especially  susceptible  to 
this  particular  type  of  trauma.  In  the  severe  cases  coma  is  profound, 
there  may  develop  paralyses,  hemiplegia,  or  other  symptoms  of  focal 
brain  injury — meningitis  or  encephalitis  may  follow,  and  if  the  patient 
lives,  convulsive  phenomena,  definite  psychoses,  neurastheniform 
conditions,  focal  disturbances,  ocular  disturbances  and  deafness, 
and  general  conditions  of  weakness,  may  ensue. 

Therapy. — The  patients  with  hyperpyrexia  should  be  placed  imme- 
diately in  cold  water,  the  temperature  of  which  is  kept  down  by  ice. 
The  drop  in  the  bodily  temperature  will  determine  the  length  of 
time  of  immersion.  In  the  heat  exhaustion  cases  a  supportive  line 
of  treatment  is  indicated. 


CHAPTER   XL 
DISEASES  OF  THE  BRAIN. 

ENCEPHALITIS— ABSCESS  OF  THE  BRAIN. 

Encephalitis  is  an  inflammation  of  the  brain  substance.  There 
are  a  number  of  forms,  but  the  term  is  here  restricted  to  the  more 
acute  processes,  due  to  bacterial  or  toxic  action.  Primary  idiopathic 
encephahtis  does  not  exist.  General  paresis  is  a  diffuse  encephalitis, 
with  exudations  and  proliferation  of  new  glia  and  bloodvessels ;  multiple 
sclerosis  is  a  t}-pe  of  disseminated  encephalitis,  etc.,  but  the  discussion 
is  here  limited  more  particularly  to  acute  exudative  inflammations  of 
the  brain  substance  proper.    Abscess  is  a  frequent  secondary  result. 

Historical. — The  encephalitides  were  for  years  included  with  the 
meningitides,  possibly  under  the  term  phrenitis,  which  to  the  ancients 
meant  any  excitement,  accompanied  by  fever.  In  Hippocrates  the 
typical  description  of  phrenitis,  however,  is  a  typhoid  delirium.  Trau- 
matic encephalitides  were  known  to  this  author. 

Just  how  long  this  conglomeration  of  cerebral  affections  remained 
an  entity  in  nosology  cannot  be  determined.  Separation  of  types  is 
going  on  at  the  present  time,  and  it  must  be  recognized  at  best  that  a 
heretogeneous  group  must  be  included  for  the  present  under  the  symbol 
encephalitis,  a  term  used  as  early  as  1554  a.d.  by  Actuarius. 

Leaving  aside  the  older  works,  the  history  of  encephalitis  practically 
begins  with  the  works  of  Rostan  (1820),  Bouillard  (1826),  Lalleman 
(1830),  and  Fuchs  (1835),  in  which  processes  of  softening,  of  an 
inflammatory  or  non-inflammatory  character  were  commencing  to  be 
recognized.  Cruveilhier,  in  1829,  distinguished  an  apoplectic  soften- 
ing, suppurative  softening,  and  softening  with  disorganization  without 
pus  or  inflammation.  Virchow's  (1846)  work  on  thi-ombosis  and 
embolism  threw  much  light  on  the  subject,  while  the  studies  of  Durand 
Fardel  (1849),  Haye  (1868),  and  Huguenin  (1876)  commenced  to 
give  a  modern  touch.  Then  Wernicke  (1881)  described  a  toxic  form, 
chiefly  alcoholic,  and  Striimpell  (1884)  opened  up  the  large  study 
of  the  infective  types.  Finally,  the  modern  study  of  epidemic  polio- 
myelitis, by  Medin  and  Wickmann,  the  recognition  of  syphilitic  types 
by  Freud  and  Plant,  and  the  work  of  Councilmann  and  his  students 
have  served  to  widen  out  the  conception  of  the  infectious  types  of 
Striimpell.  Recent  monographs  of  value  are  by  Oppenheim  and 
Cassirer  (1907),  ^^ogt,  in  Lewandowsky's  Handbuch  der  Neurologic 
(1912),  and  Southard  in  Osier's  Modern  Medicine,  1915. 

Etiology.^ — Infections  and  intoxications  are  many  and  various. 
Among  the  former  are  found  the  organisms  of  influenza,  streptococcus, 
typhoid,  Diplococcus  meningitides,  pneumonia,  scarlet  fever,  measles, 
(624) 


ENCEPHALITIS  625 

tuberculosis,  s^'philis,  tn-panosomiasis,  malaria,  rabies,  poliomyelitis, 
etc.  The  commonest  acute  forms  are  due  to  the  influenza  bacillus, 
pyogenic  organisms  (external  and  internal  infections),  including  mas- 
toid, etc.,  poliomyelitis;  among  the  more  common  chronic  types 
are  tuberculosis  and  syphilis,  which  latter,  as  Plaut^  has  well  shown, 
is  responsible  for  many  of  the  infantile  cerebral  palsies.  An  acute 
epidemic  encephalitis  of  multiform  etiology,  which  under  the  title 
lethargic  encephalitis,  attracted  considerable  attention  in  1918-1919 
as  an  epidemic  extension  throughout  many  countries  of  Europe, 
Australia  and  South  America  and  found  in  the  United  States.  The 
mesencephalon  is  chiefly  involved  in  this  type.^  As  for  the  intoxi- 
cations, alcohol  plays  the  chief  role. 

Acute  Encephalitis. — Symptoms. — As  so  much  depends  upon  the  seat 
of  the  inflammatory  process,  it  is  natural  that  great  variations  in  the 
clinical  picture  should  be  expected.  The  type  of  infecting  agent  also 
introduces  a  variant.  The  infecting  agent  of  a  polioencephalomyelitis 
is  different  from  that  of  a  syphilitic  encephalitis.  Although  similar 
structures  may  be  involved  and  in  a  manner  very  closely  related  patho- 
logically, yet  nevertheless  the  mode  of  development  will  be  different. 
For  this  reason  some  of  the  more  pronounced  types  will  receive  separate 
consideration.  Any  attempt  to  generalize  on  the  whole  group  results 
in  a  descriptive  monstrosity  untrue  to  any  clinical  type. 

1.  Influenza  Encephalitis. — ^The  work  of  Wickmann  and  others  has 
shown  that  great  care  must  be  exercised  in  not  confusing  this  with 
poliomyelitis.  In  the  true  influenzal  type,  the  disorder  is  apt  to 
develop  in  young  adults  some  day,  or  even  weeks,  after  an  acute 
influenza.  Oppenheim  includes  other  infectious  types  here.  The 
"  cold  in  the  head"  seems  to  be  clearing  up,  when  most  intense  headache 
— often  in  the  occiput — nausea,  vomiting,  apathy,  or  drowsiness  com- 
mences to  indicate  something  more  than  the  usual  depression  of  an 
influenzal  attack.  Sometimes  there  is  a  rigor.  Confusion  and  mild 
stupor  are  frequent,  the  patient  being  aroused  with  some  difficulty 
after  a  few  days.  Symptoms  of  meningeal  irritation  are  not  prominent, 
and  a  clear  cerebrospinal  fluid  will  separate  this  condition  from  the 
closely  related  picture  of  cerebrospinal  meningitis.  There  is  usually 
much  febrile  irregularity.  The  pulse  is  very  variable,  being  not  infre- 
fjuently  slow.' 

P'ocal  symptoms  develop  irregularly.  If  the  pyramidal  region  is 
in\olved  there  is  premonitory  weakness  of  the  limbs,  then  paresis, 
then  paralysis.  Convulsive  seizures  may  be  present.  The  extent  may 
be  that  of  a  monoplegia  only,  or  a  hemiplega,  which  may  not  show 
in  the  coma.  Various  aphasias  may  occur,  pseudobulbar  palsies,  or 
occulomotor  palsies.  Sensory  anomalies  are  frequent,  but  are  difficult 
to  detect  because  of  the  mental  state. 

'  Nervous  and  Mental  Disease  Monograph  Series,  1911. 

*  See  Strauss,  Abrahamson:  New  York  Med.  .lour.,  1919,  i.  Wilson,  S.  A.  K.:  Lancet, 
July  6,  1918. 

'  Jelliffe:  Influenza  and  Encephalitia,  New  York  Med.  Jour.,  1918. 
40 


626 


DISEASES  OF   THE  BRAIN 


Many  patients  recover  absolutely,  others  are  left  with  slight  motor 
defects,  monoplegias,  or  severe  hemiplegias.  In  a  few  abscess  may 
develop.  In  others  there  is  a  complicating  ependymitis  with  hydro- 
cephalus, and  frequently  there  are  psychotic  complications. 

2.  Polioeneephalomyelitis  (Striimpell's  acute  encephalitis  of  chil- 
dren in  part). — This  has  been  discussed  under  the  cerebral  forms  of 
polioencephalomyelitis  (Heine-Medins'  disease).  This  disorder  shows 
the  usual  acute  onset  of  this  disease.  The  cerebrospinal  fluid  shows 
the  characteristic  bodies  described  by  Hough  and  Lafora;  the  Wasser- 

mann  reaction  of  the  cerebrospinal  fluid 
should  be  negative.  The  residuals  here 
may  be  purely  in  the  cerebrum,  the  mid- 
brain, pons,  medulla,  and  spinal  cord 
escaping  entirely.  Various  forms  of  infan- 
tile palsy  (so-called  Little's  disease), 
idiocy,  imbecility,  monoplegia,  etc.,  are 
to  be  encountered.  Epileptiform  con- 
vulsions are  an  infrequent  residual  of  a 
motor  zone  focus.  The  prognosis  is  usually 
good.  Many  patients  recover  entirely,  or 
with  mild  residuals.  As  a  rule  there  are 
pontine,  bulbar,  or  spinal  complications. 
3.  Polioencephalitis  Hemorrhagica 
Superior. — Wernicke  called  attention  to  a 
special  form  of  diffuse  encephalitis,  with 
pronounced  involvement  of  the  midbrain 
(ophthalmoplegias).  In  one  sense  this  is 
not  a  true  inflammatory  reaction.  In 
the  alcoholic  and  syphilitic  patients  the 
course  of  the  affection  resembles  that  of 
the  Korsakov  s^^ldrome,  with  pronounced 
eye  palsies.  There  is  usually  an  initial 
delirium,  sometimes  appearing  later. 
Headache,  nausea,  and  vomiting  precede 
the  development  of  an  irregular  type  of 
ophthalmoplegia,  apparently  nuclear, 
possibly  neuritic.  Ptosis,  nystagmus, 
irregular  palsies,  optic  neuritis  are  fre- 
quent. The  gait  is  cerebellar;  the  speech  is  slurring;  the  mental 
disorientation  for  time  and  place  and  confabulation  are  marked. 
Somnolence  and  loss  of  sensory  functions  are  also  frequent,  and 
point  to  the  involvement  of  the  thalamus.  There  is  no  fever,  no 
leukocytosis,  and  a  doubtful  cerebrospinal  fluid.  The  pulse  is  usually 
rapid — SO  to  120  often  found.  The  nerve  trunks  and  muscles  are 
usually  tender  in  the  alcoholic  types.  Many  of  these  patients  clear  up 
to  a  marked  degree,  but  there  is  usually  a  residual  mental  defect,  show- 
ing in  a  lack  of  initiative  and  mild  deteriorated  states. 
The  lesions  are  those  of  a  diffuse  neuritis,  with  hemorrhagic  foci. 


Fig.  353. — Infantile     cerebral 
palsy.     (Frauenthal.) 


ENCEPHALITIS  627 

A  non-alcoholic  variety  has  been  described  in  which  somnolence 
is  more  marked  than  delirium.  There  is  ptosis,  complete  ophthalmo- 
plegia, with  vertigo  and  ataxia,  disorientation  and  frequently  con- 
fabulation. 

Occasionally  poisoning  by  mussels,  by  fish,  bromides  or  from  rabies 
shows  similar  clinical  pictures. 

4.  Pyogenic  Types. — Here  a  multiplicity  of  affections  is  to  be  reck- 
oned with.  Age  is  no  bar.  Traumatism  bulks  high  in  the  percentage; 
also  ear  disease.  The  onset  is  usually  acute,  particularly  m  the  younger 
patients.  Trauma,  or  middle-ear,  labyrinthine,  sinus  or  other  pyogenic 
infection  is  followed  by  malaise,  headache,  nausea,  and  vomiting  with 
advancing  stupor,  or  couM^ilsions  and  increasing  coma.  Great  restless- 
ness, with  increasing  delirium,  usually  develops  within  from  three 
days  to  two  weeks.  Leukocytosis  is  usually  present.  Lumbar  puncture 
is  usually  negative,  except  when  meningeal  symptoms  are  also  present. 


¥^ 

•JU 

'  ■^' 

Fig.  .354. — Midbrain  lesions  in  paresi.s  with  Korsakov  syndrome;  Wernicke's 
polioencephalitis  superior. 

Topical  signs  are  very  frequent.  These  are  monoplegia,  hemiplegia, 
epileptiform  convulsions,  cranial  nerve  palsies.  If  the  delirium  clears 
up,  one  may  find  aphasias,  pseudobulbar  palsies,  various  midbrain 
syndromes,  hemianopsias,  and  bulbar  palsies. 

Many  patients  die  in  coma.  Others  recover  with  marked  mental 
defect  (cerebral  atrophies  of  childhood,  idiocy,  imbecility,  debility). 
In  others  the  general  signs  of  brain  abscess  {q.  v.)  become  apparent. 
Again  others  clear  up  with  hemiplegias,  monoplegia,  diplegias  (so 
called  Little's  disease).  Finally,  others  recover  entirely,  or  show  small 
focal  residual  lesions. 

Lethargic  Encephalitis. — Under  this  caption  is  included  a  series  of 
cases,  which  have  been  studied  in  Italy,  France,  Fngland  Australia, 
South  America  and  the  United  States,  characterized  by  acute  involve- 
ment by  a  general  infectious  process  chiefly  localized  in  the  mesen- 
cephalon. The  chief  symptoms  have  been  those  of  a  general  infection 
with  marked  languor  and  drowsiness  passing  into  coma  with  associated 
ocular  palsies  and  at  times  involvement  of  other  (facial  particularly) 


628  DISEASES  OF  THE  BRAIN 

cranial  nerves.  Ptosis,  strabismus,  nystagmus  of  ocular  origin,  are  the 
chief  mesencephalic  s\Tiiptoms;  the  marked  lethargy  also  indicating 
probable  thalamic  implication.  MacNalty  has  attempted  a  differ- 
entiation of  three  t^'pes.  (1)  General  infection  with  lethargy  and  no 
localizing  signs,  (2)  mesencephalic  t\ipes  with  pyramidal  or  extra- 
pyramidal signs,  ocular  palsies,  rubrospinal  ataxic  t^'pes,  with  lethargy, 
(3)  abortive  and  spinal,  cortical  and  pohTieuritic  t^-pes.  The  pro- 
dromal period  is  from  three  to  twenty-one  days,  with  lethargy, 
asthenia,  speech  slurring  or  chattering,  meningeal  irritation  signs, 
temperature  100°  to  102°  F.,  incontinence  of  urine  and  feces. 

Pathologically  the  changes  are  those  of  an  acute  infectious  process, 
somewhat  like  poliomyelitis  and  influenza,  but  apparently  differing  in 
the  character  of  the  plasma  cell  infiltration  in  some  particulars.  Many 
observers  are  disposed  to  believe  in  an  independent  infection,^  but 
separated  clinically  with  difficulty  from  influenza  and  poliomyelitis. 

Diagnosis. — It  is  impossible  with  present  methods  to  clearly  dis- 
tinguish all  of  the  various  encephalitides.  Many  are  not  diagnosed 
during  life,  the  course  being  so  rapid,  and  facilities  for  laboratory 
research  limited.  At  the  present  time  (1919)  any  attempt  at  present- 
ing the  problem  is  only  patchwork. 

Wernicke's  type  is  characteristic.  It  is,  however,  frequentlj^  reserved 
for  the  psychiatrist  to  make  the  diagnosis,  as  the  mental  symptoms 
so  mask  the  physical  signs.  Ophthalmoplegia,  neuritis,  disorientation 
for  time  and  place,  with  confabulation,  progressing  to  delirium  or 
coma,  the  almost  typical  facies,  closely  resembling  the  Korsakov  facies, 
tachycardia,  and  no  hyperthermia  are  the  leading  features.  Here  the 
entire  series  of  the  alcoholic  s\Tidromes  must  be  reviewed.  (See 
Alcoholism.) 

Polioencephalomyelitis  is  to  be  suspected  during  an  epidemic.  The 
acute  onset,  frequently  with  gastro-intestinal  or  nasal  respiratory 
predecessors;  the  frequency  of  spinal  and  bulbar  involvements;  usual 
absence  of  signs  of  neuritis;  lost  knee-jerks,  spinal  fluid  findings  are 
suggestive. 

Mesencephalic  syndromes  in  influenza  are  infrequent  but  they  do 
occur.2  There  are  undoubtedly  many  etiological  types  ef  lethargic 
encephalitis.  The  symptoms  are  solely  the  result  of  the  location  of 
the  process. 

Meningitis  often  calls  for  diagnosis.  Here  lumbar  puncture  is  of 
great  service,  since  many  of  the  initial  clinical  signs  are  identical. 
Increased  pressure,  presence  of  cellular  elements  and  pus  are  present  in 
purulent  meningitis.  In  epidemic  cerebrospinal  meningitis  there  is  the 
characteristic  organism. 

Herpes  is  usually  absent  in  encephalitis;  the  neck  and  muscle 
hyperesthesia  usually  less.    Eye-muscle  palsies  are  often  absent  in  the 

1  See  Economo,  Vienna:  Discussion,  British  Med.  Jour.,  November  2,  191S.  Strauss, 
Abrahamson:  New  York  Med.  Jour.,  1919.  Cleland  and  Campbell:  British  Med.  Jour., 
May  31,  1919.  Vaughan:  Jour.  Lab.  and  Clin.  Med.,  April,  1919,  and  numerous  cita- 
tions of  literature  in  Jour.  Nerv.  and  Ment.  Dis.,  1919. 

2  Jelliffe:  Influenza  and  the  Nervous  System,  loc.  cit. 


ABSCESS  OF  THE  BRAIN 


6^9 


pyogenic  types  of  encephalitis;  they  are  not  infrequent  in  meningitis. 
In  s\^hilitic  tj'pes  the  Wassermann  test  is  of  great  service. 

Therapy. — Practically  only  in  the  malarial  and  s^-philitic  encephali- 
tides  is  there  any  specific  therapy.  In  the  pyogenic  forms,  with  abscess, 
surgery  is  demanded.  The  poisonings  require  withdrawal.  Bromide 
poisoning  is  not  to  be  overlooked. 

All  these  patients  require  bed  treatment  in  a  darkened  room.  The 
toxic  cases  should  have  hot  sheets  and  elimination  encouraged.  Ice- 
bags  to  the  head  give  comfort  and  counter-irritation ;  they  do  not  affect 
internal  temperatures.  Active  catharsis  is  de- 
sirable, calomel  and  salines  being  advisable. 

In  the  influenzal  t\'pes,  salicylates  are  indi- 
cated. Otherwise  the  treatment  is  largely 
symptomatic.  The  residual  symptoms  call  for 
their  special  therapy. 

Abscess  of  the  Brain. — History. — Brain  ab- 
scesses have  been  recorded  for  many  years: 
Cruveilhier's  and  Carswell's  illustrations  are 
classic.  The  steps  that  led  up  to  the  present- 
day  conception  of  brain  abscess  are  partly  out- 
lined in  the  section  on  Encephalitis.  The  history 
of  these  two  groups  is  almost  identical  up  to 
the  appearance  of  Wernicke's  Lehrhiich  d. 
Gehirnkrankheiten ,  1883,  when  a  division  into 
infectious  and  non-infectious  abscesses  was 
foreshadowed.  Macewen's  (1893)  studies  gave 
a  great  impetus  to  the  understanding  of  the 
ports  of  entrance,  while  Korner's  (1902-1908) 
studies  have  set  in  clear  relief  the  importance 
of  aural  disease.  Detailed  monographic  or 
critical  studies  are  those  of  Oppenheim  and 
Cassirer  (1909),  Brissaud  and  Soques,  in 
Bouchard  and  Brissaud's  Traite  de  Medicine 
(1904),  IX,  Lewandowsky,  in  Lewandowsky's 
Handbuch  der  Xeurolgie  (1912).  Southard,  in 
Osier's  Modern  Medicine  (1915),  and  Warring- 
ton: Quarterly  Journal  of  Medicine,  January, 
1918. 

Etiology. — Cerebral  abscess  is  almost  exclusively  a  secondary  phe- 
nomenon due  to  infection.  Many  organisms  have  been  found  in 
cerebral  abscess.  They  are  derived  from  many  sources;  external 
trauma  with  or  without  fracture,  the  venous  sinuses,  and  otitis  media 
being  among  the  commonest.  Otitic  infection  supplies  about  a  third, 
the  usual  communication  being  through  the  tegmen  of  the  antrum. 
Nose  infections  and  pyogenic  processes  in  the  accessory  sinuses  play 
important  roles.  Metastatic  abscesses,  from  pyemia,  tuberculosis, 
osteomyelitis,  abscess  of  lung,  liver,  female  pelvic  disease,  etc.,  are 
among  the  curiosities.    Streptococcus  pyogenes  is  the  most  frequently 


Fig.  355.  —  Infantile 
cerebral  palsy.  (Frau- 
enthal.) 


630  DISEASES  OF   THE  BRAIN 


found  bacterium.  S.  aureus  citreus,  B.  pyocyaneus  and  pyogenes 
fetidus  are  frequently  found.  Multiple  infection  is  the  rule  and  a 
great  many  different  organisms  have  been  isolated. 

Symptoms. — There  is  always  some  antecedent  disorder,  although 
such  may  be  overlooked  or  be  silent,  the  acute  miliary  tuberculosis  of 
cardiac  disease  being  an  example.  The  symptoms  will  vary  greatly 
with  the  exciting  cause,  the  site,  and  the  size  of  the  abscess  or  abscesses. 
Certain  traumatic  abscesses  run  their  fatal  course  in  three  to  five  days, 
whereas  some  recorded  cases  have  persisted  for  years.  Intermediary 
types  of  every  variety  are  the  rule. 

There  are  enough  cases  described  to  show  that,  speaking  in  general 
terms,  one  may  recognize  (1)  a  stage  of  irritation,  (2)  a  stage  of  remis- 
sion or  latency,  and  (3)  a  stage  of  paralysis  or  of  acute  progression. 

1.  Primary  Stage. — In  the  initial  stages,  general  rather  than  localiz- 
ing signs  predominate.  The  patient  usually  has  an  acute  rise  in 
temperature;  it  is  frequently  slight,  insidious,  and  variable,  and  often 
absent  after  a  few  days.  The  yulse  is  often  slow.  Headache  is  rarely 
absent  but  is  very  variable  in  intensity.  It  is  most  frequently 
dull,  but  gives  a  sense  of  tension  in  the  head.  There  may  be  some 
vomiting. 

Such  a  tense  headache,  with  rise  in  temperature  and  slow  pulse 
following  an  otitis,  after  the  discharge  may  have  ceased,  is  of  grave 
import.  The  pain  is  situated  usually  over  the  side  affected ;  but  is  not 
infrequently  slight  or  absent  in  cerebellar  cases  (g.  i\)  or  here  may  be 
frontal  in  character.  Movements  are  apt  to  increase  the  headache. 
Anxiety,  insomnia,  restlessness  are  the  rule,  and  in  younger  patients 
the  sleep  is  often  broken  by  loud  cries.  There  may  be  some  delirium 
or  stupor  in  these  initial  stages,  which  occupy  the  first  three  or  four 
days,  occasionally  a  week.  There  is  frequently  some  leukocytosis  in 
this  stage. 

2.  Stage  of  Remission  or  Latency. — ^A  period  of  depression  often  sets 
in  about  this  time.  The  headache  diminishes,  the  fever  diminishes 
the  patients  are  fatigued,  somnolent,  indifferent.  The  pulse  is  apt  to 
remain  slow.  Percussion  at  this  time  may  reveal  tenderness.  The 
blood  count  is  variable,  with  a  tendency  to  poljiiuclear  increase. 
This  stage  shows  extraordinary  variation  in  different  cases,  sometimes 
persisting  weeks  or  months. 

In  this  stage  beginning  choked  disk  is  not  infrequent,  especially  if 
the  disorder  has  lasted  over  a  week  or  ten  days.  It  is  apt  to  first  appear 
homolateral  with  the  abscess.  There  are  indefinite  signs  of  a  cerebral 
affection;  variable  headache,  occasional  nausea,  vomiting,  faulting 
attacks,  or  convulsive  seizures. 

The  pupils  are  frequently  unequal,  if  there  is  pus,  and  the  more 
dilated  pupil  is  apt  to  be  on  the  side  of  the  abscess.  With  maximum 
equal  dilatation,  the  localization  is  difficult  from  the  pupils  alone, 
and  such  dilatation  argues  for  a  large  abscess.  With  increasing  size, 
immobility  appears.  Contracted  pupils  are  found,  in  a  few  instances, 
toward  the  end  in  severe  cases. 


\ 


ABSCESS  OF   THE  BRAIN  631 

3.  Stage  of  Advance. — After  a  period  averaging  from  one  to  three 
months  the  symptoms  of  an  advancing  cerebral  pressure  manifest 
themselves.  There  are  delirium,  often  epileptiform  convulsions,  all 
of  the  symptoms  of  a  generalized  meningitis,  with  death. 

Occasionally  encapsulation  occurs  with  no  symptoms  for  from  ten 
to  twenty-eight  years^  (Xauwerck). 

Course. — The  course  just  described  is,  statistically  speaking,  the 
most  frequent,  but  it  is  by  no  means  universal.  In  many  cases  there 
is  an  acute  progressive  course,  without  any  intermission  or  remission. 
This  may  also  follow  a  latent  period,  or  may  develop  acutely  after  a 
trauma,  or  following  a  suppurative  process  in  the  ear  or  nose.  In  others 
there  occurs  an  acute  progressive  course,  with  a  remission  which  is 
incomplete.    The  chronic  course  is  as  described. 

Forms. — The  most  marked  are  the  traumatic,  the  otitic,  the  rhino- 
genic,  and  metastatic.  In  the  traumatic  cases  the  abscess  is  usually 
near  the  site  of  the  trauma,  and  the  symptoms  of  the  abscess  formation 
are  apt  to  be  complicated  by  the  traumatic  incidents.  The  symptoms 
of  a  leptomeningitis  develop  early,  whereas  those  of  a  purulent  destruc- 
tive nature  usually  require  from  eight  to  fourteen  days  (Bergmann), 
The  whole  development  is  usually  gradual,  and  the  stages  far  from 
being  sharply  set  off  one  from  another.  Headache,  fever,  vomiting, 
vertigo,  confused  delirium,  these  are  the  prodromal  signs.  The  focal 
lesions  occur  several  days  later,  either  as  an  epilepsy,  a  cortical  mono- 
plegia, a  speech  or  other  motor  or  sensory  defect. 

Traumatic  late  abscesses  are  also  known,  weeks,  months,  even  years 
go  by,  before  complete  evolution. 

Otitic  brain  abscesses  are  frequent,  yet  in  proportion  to  otitis  are 
rare  (Jansen,  7  in  5000  cases).  Here  the  original  disorder,  the  local- 
ization, and  the  complications  are  of  moment.  The  development  has 
already  been  sketched.  It  is  very  insidious  in  many  cases.  The 
localization  is  usually  either  temporosphenoidal  or  cerebellar.  Hein- 
man's^  study  of  818  cases  showed  that  there  were  539  cerebral  and 
279  cerebellar  involvements.  Of  645  carefully  studied  cases  the  local- 
izations were  as  follows:  Temporal  395,  temporal  and  occipital  19, 
temporal  and  frontal  4,  temporal  and  cerebellar  10,  occipital  9,  frontal 
3,  cerebellar  186,  others  scattering.  The  symptomatology  of  right- 
and  left-sided  temporosphenoidal  abscesses  or  tumors  offer  considerable 
variations.  A  right-sided  abscess  may  pursue  its  course  with  little  or 
no  localizing  signs.  Left-sided  ones  often  give  rise  to  aphasic  complexes. 
Pressure  upon  the  pyramidal  tracts  will  give  characteristic  hemiplegic 
phenomena;  there  are  apt  to  be  disturbances  of  smell,  direct  or  sub- 
jective, olfactory,  or  uncinate  fits  (Knap]),  loc.  cit.). 

lihinogenic  abscesses  are  usually  located  frontally.  In  additicm  to 
the  headache,  there  is  frequently  a  certain  degree  of  t()ii)or,  often 
associate<l  with  a  tendency  to  joke  (Witzelsucht),  isolated  choked  disk, 
a  cerebellar  type  of  ataxia,  when  the  cortico-rubro-cerel)ellar  i)athways 

'  See  Oppenheim,  loc.  cit.,  for  literature  of  these  rare  cases. 
*Arch.  f.  Ohrenhk.,  Ixxiii,  258. 


632  DISEASES  OF  THE  BRAIN 

are  implicated,  isolated  tremor  of  the  hand,  and  olfactory  disturbances. 
When  the  abscess  reaches  farther  back,  motor  symptoms  occur. 

Diagnosis. — Many  differential  points  arise.  The  etiology  is  the  most 
important  factor  to  bear  in  mind.  Traumatic  purulent  meningitis, 
apoplexy,  late  apoplexy  (Bollinger),  pachymeningitis,  brain  tumor, 
tuberculous  meningitis,  syphilitic  meningitis,  hemorrhagic  encephalitis, 
meningitis  serosa,  psychogenic  headache,  sinusitis  and  sinus  thrombosis 
are  the  chief  disorders  to  be  reviewed.  The  last  often  runs  a  very  simi- 
lar course,  and  is  often  associated  with  a  purulent  otitis,  as  a  funda- 
mental disorder.  Here  the  fever  is  apt  to  be  high  and  remittent,  even 
on  the  same  day,  rigors,  chills,  and  profuse  perspiration,  pulse  usually 
rapid  and  irregular,  convulsions  rare,  save  perhaps  in  children,  head- 
ache constant,  eye-grounds,  as  in  abscess,  usually  bilateral  neuritis, 
more  frequent  than  choked  disk.  Focal  symptoms  rare.  Physical  signs 
of  swollen  spleen,  and  metastatic  invasions  confirm  the  diagnosis  of 
thrombosis. 

A  purulent  meningitis  may  be  more  difficult  to  differentiate.  It  may 
be  caused  by  similar  factors;  it  is  usually  more  acute  and  stormy  in 
onset;  it  usually  runs  a  shorter  course,  shows  higher  and  more  con- 
tinuous fever,  herpes,  the  pulse  usually  rapid,  irregular,  often  slow  in 
beginning  as  in  abscess,  initial  unrest,  irritability,  confusion,  delirium 
in  contrast  to  a  heaviness  or  coma  in  abscess,  headache  constant, 
vomiting  frequent,  convulsions  common,  general  clonic  and  often 
muscular  twitches,  the  eye-grounds  are  less  often  positive,  meningeal 
irritation  phenomena  (Kernig,  spasticity,  clonus,  etc.),  common,  local 
symptoms  more  confined  to  cranial  nerves  and  basal  signs  rather  than 
intracerebral  focal  signs,  spinal  symptoms  frequent,  whereas  in  abscess 
they  are  rare.  Furthermore,  the  cerebrospinal  fluid  is  more  apt  to 
contain  globulin  and  show  a  lymphocytosis  or  even  pus. 

Prognosis. — When  left  alone,  cerebral  abscess  almost  invariably 
causes  death.    Calcification  or  external  discharge  occasionally  occurs. 

Treatment. — This  is  exclusively  prophylactic  in  nose,  throat,  and 
otitic  work,  and  surgical  after  the  development  of  the  abscess.  The 
results  of  operative  interference  are  highly  satisfactory.  The  statistics 
vary  with  each  new  series  of  cases.  In  the  hands  of  competent  surgeons 
the  risk  from  operation  is  nil.  The  temporospheniodal  and  frontal 
abscesses  can  be  readily  reached  and  drained.^ 

1  Starr:  Med.  Record,  1906.  Halsted,  in  White  and  Jelliffe:  Modern  Treatment  of 
Nervous  and  Mental  Diseases,  1913. 


CHAPTER  XII. 
DISEASES  OF  THE  BRAIN   (Continued). 

VASCULAR   DISTURBANCES— CEREBRAL    APOPLEXIES. 

The  quantity  and  quality  (viscosity)  of  the  blood,  the  heart  action, 
the  vascular  tension  and  blood-pressure  are  the  chief  factors  which 
govern  the  general  as  well  as  the  cerebral  blood  flow.  Of  all  the 
bodily  structures,  the  brain  is  probably  the  most  highly  vascularized, 
arguing  for  its  great  functional  activity.  Furthermore,  there  is  marked 
plasticity  evident  in  the  compensatory  regulations.  Not  only  is  there 
a  continuous  balance  maintained  between  different  vascular  systems, 
especially  between  those  larger  groups,  such  as  the  portal  system,  the 
vessels  of  the  skin,  the  muscles,  and  the  brain,  but  within  the  brain 
itself  different  balancing  reactions  are  constantly  going  on  between 
speech  areas,  visual  areas,  auditory  areas,  motor  systems,  visceral 
areas,  etc.,  which  are  thrown  into  use  at  different  times  in  specialized 
occupations  or  activities,  or  in  response  to  emotional  stress.^ 

The  anatomical  structures  used  in  regulating  these  compensating 
mechanisms  are  partly  localized  in  the  medulla  and  cord,  as  discussed 
in  the  chapters  on  the  ^'egetative  Xeurolog}'  of  the  Vascular  Appara- 
tus, but  parts  are  within  the  vascular  apparatus  itself;  at  times  within 
the  walls,  again  within  the  circulating  blood  (viscosity  disturbance)  .- 

The  smaller  cerebral  vessels,  in  conformity  with  the  softness  and 
plasticity  of  a  developing  organ,  have  less  rigid  walls,  hence  more 
easily  overfill  (congestion)  and  contract  (fainting),  are  more  liable  to 
spontaneous  rupture,  and,  furthermore,  are  peculiar  in  that  they  have 
few  or  no  anastomosing  branches.  Thus  special  problems  are  con- 
nected with  the  cerebral  circulation.  The  avenues  of  blood  intake 
are  through  the  two  internal  carotids  and  the  two  vertebrals.  The 
course  of  the  left  internal  carotid  is  more  direct,  and  it  has  been  taught 
that  thrombi  are  therefore  more  common  on  the  left  side.  There  are 
no  decisive  facts  to  bear  out  this  a  priori  conjecture. 

The  vertebrals  unite  to  form  the  basilar,  which  bifurcates  into  the 
two  large  posterior  cerebral  vessels  which  supply  the  temporo-occipital 
lobes,  the  corpora  quadrigemina,  crura,  and  parts  of  the  optic  thalami. 
The  posterior  communicating  branches,  usually  small  and  symmetrical 
and  subject  to  great  variation,  pass  forward  to  join  the  internal  carotids, 
and  are  given  oft'  to  the  base  of  the  brain.  The  internal  carotids  form 
the  middle  cerebral  and  the  anterior  cerebral  arteries,  an  anterior 
communicating  branch  completing  the  circle  of  Willis. 

The  middle  cerebral  arteries  lie  in  the  Sylvian  fissure,  giving  oft' 

1  Cannon:     Fear,  Hate,  Rage  and  Anger,  p.  183. 

2  Hirschfeld:     Ztschr.  f.  Neur.  u.  Psych.  Ref.,  iv,  193. 

(633) 


634 


DISEASES  OF   THE  BRAIN 


many  branches  to  the  lenticular  nucleus,  the  internal  capsule,  portions 
of  the  thalamus,  and  superficially  irrigate  the  Island  of  Reil,  Heschl's 
convolution,  and  much  of  the  lateral  aspect  of  the  brain. 

The  anterior  cerebral  arteries  supply  the  frontal  areas,  the  olfactory 
apparatus,  the  upper  margin  of  the  frontal,  parietal  and  occipital 
lobes  on  their  mesial  aspects,  and  much  of  the  corpus  callosum. 


Part  of  marginal  convolution  ;  superior 
and  middle  frontal  convolutions:  upper 
part  of  ascending  frontal  convolution. 


Cotpus  callosum:  gijrnsfomicatus:  inner 
surface  of  first  frontal  convolution: 
upper  part  of  asc.  frontal  convolution. 


Lobus  quadratus  and  adjacent  outer 
surface  of  liemisplicre. 


Third  front,  convolution;  outer 
part   of  orbitai  surface  of 
frontal  lobe. 


Asc.  front,  convolution. 


parietal  convolution:  lower 
rt  of  sup. parietal  convolution. 


Supramarginal  gyrus:  first  temp, 
convolution:  part  of  second  temp, 
convolution:  angular  gyrus. 


Ext.  occip.  comyolution; 
third  temp,  convolution. 


J  Inner  and  outer  surfaces 
of  occipital  lobe. 


Fig.  356.— Circle  of  Willis  and  branches,  with  indication  of  distributions.      Region 
A,  B  most  frequent  sites  for  hemorrhages.      (Greenacre,  from  de  Fursac.) 


The  cerebellum  derives  its  supply  from  the  vertebral  and  basilar 
arteries. 

Partial  compensatory  balance  of  the  circulation  is  brough  about 
chiefly  through  the  circle  of  Willis.  This  circle,  as  well  as  the  branches 
from  it,  shows  a  vast  number  of  anomalies,  and  these  in  part  determine 
many  anomalous  cerebral  disturbances,  possibly  related,  as  Blackburn, 
AYindle,  Bullen  and  others  have  shown,  to  faulty  cerebral  development, 
thereby  lading  the  anatomical  foundation  for  a  neurosis,  a  psychosis 
or  some  aberrant  vascular  disease  (softening,  deterioration,  vicarious 


VASCULAR  DISTURBANCES— CEREBRAL  APOPLEXIES     635 

menstruation,    etc.).     Furthermore,    such    anomalies    are    of    great 
surgical  importance.^ 

The  chief  cerebral  arterial  disorders  arise  from  (a)  temporary 
vascular  instability  (shock  reactions,  cardiac  irregularities,  internal 
secretory  disturbances);  (b)  protracted  regressive  changes  (arterio- 
sclerosis, with  or  without  miliary  aneurisms);  (c)  hemorrhage;  (d) 
thrombosis;  (e)  embolism. 

Venous  changes  are  less  frequent  than  arterial  ones.  Sinus  throm- 
boses are  the  most  important. 

(a)  Vascular  Instability. — This  is  a  very  variable  factor  and  has 
been  discussed  under  the  sections  on  Vegetative  Neurology.  Vago- 
tonia and  vagotropia  occur  in  the  cerebral  vessels  as  well  as  in  the 
somatic  vessels.  The  results  were  previously  grouped  under  the 
concepts  cerebral  anemia  and  cerebral  hyperemia.  Chronic  vago- 
tonic conditions  are  known.  They  should  not  be  called  cerebral 
congestions. 

Anemia  (Shock;  noci-association — Crile)  results  from  loss  of  blood, 
paracentesis,  surgical  handling  of  intestines,  cardiac  weakness,  or 
from  marked  vascular  instability,  often  of  psychogenic  origin.  The 
symptoms  are  faintness,  dizziness,  black  spots  before  the  eyes,  buzzing 
in  the  ears,  and  it  may  be  loss  of  consciousness  with  or  without  nausea 
and  vomiting. 

There  may  be  a  partial  consciousness,  apathy  or  semicoma,  with 
contracted  pupils  and  cold  clammy  skin,  occasionally  loss  of  bladder 
or  bowel  function. 

The  therapy  is  heat  and  bandaging  of  the  extremities,  the  hori- 
zontal position,  camphor,  caffein  and  cardiac  stimulants,  alcohol  and. 
ether.  The  many  problems  of  surgical  shock  must  be  sought  for  in 
surgical  literature. 

Hyperemia. — Hyperemia  may  be  active  or  passive.  The  former  may 
result  from  vascular  instability,  or  is  not  infrequent  after  excessive 
eating  or  drinking.  Sudden  emptying  of  the  peripheral  and  somatic 
vessels  may  occasion  it.  It  is  frequent  in  certain  thyroidisms,  notably 
in  exophthalmic  goiter  and  in  natural  and  artificial  menopause  states, 
which  latter  often  are  accompanied  by  changes  in  the  organs  of  inter- 
nal secretion.     (See  Vegetative  Neurology,  Endocrinopathies.) 

The  symptoms  of  hyperemia  are  redness  and  congestion  of  the 
face  and  eyelids,  pounding  in  the  ears  or  neck  or  head,  headache, 
confusion  and  usually  contracted  pupils.  More  severe  attacks  may 
lead  to  acute  confusion,  rise  in  temperature,  and  the  general  picture  of 
an  acute  meningitis. 

Signs  of  congestion  of  the  face — flushing,  a  sense  of  fulness,  etc., 
must  not  be  taken  to  necessarily  mean  cerebral  congestion.  It 
should  be  borne  in  mind  that  the  vessels  of  the  face  and  those  of  the 
brain  are  controlled  by  separate  mechanisms. 

1  Blackburn  I.  W.:     Jour.  Comp.  Near.,  1907,  x\ai,  93;    1910,  xx,  185. 


636  DISEASES  OF   THE  BRAIN 

Passive  hyperemias  result  from  mechanical  obstruction  to  the 
jugulars,  chiefly  from  new  growths,  emphysema  and  loss  of  compen- 
sation of  heart  disorders.  Pressure  of  the  head,  abnormal  wakefulness 
or  sleepiness,  dizziness,  apathy  or  mild  confusion,  with  anxiety,  are 
the  chief  signs,  combined  with  definite  signs  of  passive  congestion  of 
the  skin,  face  and  mucous  membranes. 

Efficient  therapy  consists  in  ameliorating  the  causative  factors 
whether  active  or  passive.  The  hyperemias  are  purely  secondary. 
Acute  states  may  call  for  bleeding,  mustard  foot-baths,  active 
catharsis. 

(6)  Cerebral  Arteriosclerosis. — This  is  a  widespread  disorder  occur- 
ring chiefly  after  forty  years  of  age  when  with  increasing  years  it  tends 
to  become  more  and  more  prominent.  The  arteriosclerotic  process 
difl'ers  little  in  cerebral  vessels  from  those  located  in  other  organs  of 
the  body,  save,  in  a  tendency  for  a  marked  production  of  miliary 
aneurisms.  These  are  largely  conservative  formations  and  are  not 
to  be  considered  as  productive  of  hemorrhage,  as  taught  by  Charcot 
and  Bouchard.^  Cerebral  arteriosclerosis  is  due  to  the  same  causes 
that  produce  arteriosclerosis  elsewhere  (heredity,  syphilis,  alcohol, 
chronic  hyperadrenalemia  from  psychogenic,  conscious  and  uncon- 
scious, or  other  factors,  etc.),  and  may  be  the  expression  of  a  general 
disease,  or  may  be  sharply  delimited  to  the  cerebral  vessels. 

Symptoms. — The  symptoms  of  early  arteriosclerosis  may  be  general 
or  localized.  The  chiefly  early  symptoms  are  sleeplessness,  restless- 
ness, headaches,  especially  if  there  are  dizzy  attacks,  and  renal  changes, 
neural  fatigue,  increased  emotionalism  and  irritability.  To  this,  at 
times  is  added  hebetude,  read}^  forgetting,  especially  of  new  impres- 
sions, increased  blood-pressure.  These  signs  are  often  loosely  and 
improperly  spoken  of  as  neurasthenic.  Local  signs  may  be  added, 
such  as  temporary  lapses,  marked  somnolence,  tingling  or  numbness 
or  other  signs  of  focal  disease. 

The  chief  neurological  interests  are  focussed  upon  the  distribution 
of  the  focal  lesions  and  are  here  discussed  according  to  the  syndromes 
presented.  The  psychiatric  features  are  only  mentioned  here,  as  they 
are  considered  in  the  special  groupings  under  the  senile,  presenile  and 
-arteriosclerotic  psychoses.  (See  Section  III.)  Thus  only  the  more 
distinctly  neurological  features  are  accented  in  this  place.  (See  tables 
in  chapter  on  Midbrain.) 

These  chief  syndromes  are:^ 
I.  Disorders  due  to  disease  of  the  twigs  and  terminal  branches. 
(a)  Cortical    branches;    irritative    complexes,    Alzheimer's 

disease. 
(6)  Medullary  branches;  lacunar  syndromes, 
(c)   Diffuse  types  (arteriosclerotic  dementias). 

>  See  Pick:     Berl.  klin.  Wchnschr.,  February  21,   1910,  p.  325,  for  bibliography. 
2  Lambert:     States  Hospital  Bull.,  1908,  i,  475. 


CEREBRAL  APOPLEXIES  637 

II.  Disorders  of  chief  branches. 

(a)  Anterior  cerebral  syndromes,  crural  palsies. 
(6)  ]\Iiddle  cerebral  syndromes. 

1.  Palsies:  hemorrhage,  embolism,  thrombosis,  palsies, 

apoplexies. 

(1)  Monoplegias:  facial,  brachial. 

(2)  Combined  palsies.     Apoplexies  in  general. 

(3)  Complete  palsies:  capsular  and  supracapsular. 

(4)  Thalamic  syndromes. 

2.  Aphasias. 

3.  Hemianopsias. 

(c)   Posterior  cerebral  syndromes. 
id)  Inferior,  superior,  posterior,  cerebellafc  syndromes. 
III.  Disorders  of  large  trunks  from  obliterating  lesions. 
1.  Basilar  syndromes. 

CEREBRAL  APOPLEXIES. 

A  tabular  summary  of  all  these  is  to  be  found  at  the  close  of  the 
chapter  on  ]\Iidbrain  Disorders  (g.  ?.). 

Cerebral  Apoplexies. — Hemorrhage,  Thromtosis,  Embolism.— Clean- 
cut  examples  of  these  syndromes  are  rare.  The  patients  present  mixed 
conditions  save  in  the  few  instances  of  embolism  due  to  the  closure  of 
a  main  trunk.  For  this  reason  the  general  rather  than  the  special  type 
of  apoplectic  attack  will  be  described. .  Special  indications  as  to  the 
localization  of  the  region  involved  will  be  noted  later.  Pontine  and 
midbrain  localizations  have  already  been  discussed,  as  have  also 
vascular  disorders  within  the  cerebellum. 

Distribution,  Causation. — ]\len  are  more  often  affected  (seven  to  five) 
than  women,  and  four-fifths  of  the  cases  occur  after  forty  years  of  age. 
Arteriosclerosis,  as  indicated,  plays  the  more  important  role  in  hemor- 
rhage and  in  thrombosis,  and  the  smaller,  rather  than  the  basal  vessels 
are  "responsible.  Cardiac  hypertrophy  with  increased  blood-pressure 
(180  to  225  mm.)  is  the  chief  accompanying  factor,  and  is  closely 
associated  with  the  arteriosclerotic  process.  Chronic  kidney  disease  is 
also  a  frequent  concomitant  factor  (30  per  cent.).  Exciting  causes, 
such  as  great  physical  exertion,  lifting  heavy  weights,  coitus,  vomiting, 
coughing,  sneezing,  etc.,  were  formerly  given  a  prominent  place.  It 
is  doubtful  if  they  have  much  importance.  In  a  large  series  of  cases 
studied  by  Jones,  in  a  considerable  number  the  stroke  occurred  within 
a  few  minutes  after  getting  out  of  bed,  so  that  the  sudden'  change  in 
blood-pressure  on  awakening  and  getting  about  was  chiefly  responsible. 
Many  patients  develop  apoplexy  during  sleep,  although,  other  things 
being  equal,  sleep  is  a  protection.  Psychogenic  factors  play  a  much 
larger  role  than  is  at  present  comprehended.  These  act  chiefly 
through  the  maintenance  of  high  blood-pressure  states  from  emotional 
factors,  conscious  and  unconscious. 

The   localization   of   the   hemorrhage    (thrombus)    varies   greatly. 


638  DISEASES  OF   THE  BRAIN 

Right  and  left  sides  are  about  equally  involved.  ^lorgagni's  early 
dictum  concerning  the  greater  prevalence  of  right-sided  hemorrhages 
has  little  support  from  extensive  statistics.^ 

The  chief  syndromes  are  of  the  middle  cerebral  type.  The  branches 
of  the  lenticulostriate  arteries  supplying  the  internal  capsule,  caudate 
nucleus,  lenticular  nucleus  and  optic  thalamus  are  most  frequently 
involved.  Thus  the  most  frequent  syndromes  are  the  combined 
palsies,  arm  and  leg;  arm,  leg  and  face;  arm,  leg,  face  with  sensory 
symptoms  and  aphasic  complications. 

Cortical  hemorrhages  are  probably  much  more  frequent  than  is 
usually  supposed  since  many  occur  without  the  death  of  the  patient. 
These  result  in  more  limited  syndromes,  such  as  apraxias,  arm  mono- 
plegias (anterior  cerebral  syndrome). 

]\Ionoplegias  of  the  leg,  isolated  aphasias,  lower  quadrant  hemianop- 
sias and  the  thalamic  syndrome  belong  more  especially  to  the  middle 
cerebral  syndrome,  while  mind-blindness  and  homomTnous  hemi- 
anopsias are  the  chief  features  of  the  isolated  posterior  cerebral 
syndromes. 

Midbrain,  pontine,  medullary  and  cerebellar  hemorrhages  are 
comparatively  infrequent,  and  have  a  special  syndromy  described 
elsewhere.     (See  Chapter  VIII,  Diseases  of  the  Midbrain.) 

The  Apoplectic  Attack. — Nausea  and  vomiting  are  the  most  frequent 
precursors  of  the  apoplectic  attack.  In  thrombosis  or  embolic  occlud- 
ing lesions  twitchings  or  even  convulsions  are  more  frequent  as  precur- 
sors of  the  attack  than  in  hemorrhagic  cases.  In  hemorrhagic  cases 
with  convulsions  the  bleeding  is  more  liable  to  have  extended  to  the 
ventricles,  and  can  frequently  be  demonstrated  by  lumbar  puncture. 
This  is  not  invariable,  however. 

The  attack  is  usually  abrupt.  Dizziness,  heaviness,  anxiety,  head- 
ache, paresthesiae  may  be  described  by  those  who  do  not  at  first 
become  suddenly  unconscious,  and  yet  in  whom  a  gradually  develop- 
ing state  of  unconsciousness  occurs,  with  paraphasia  and  gradual  weak- 
ness of  one  side  of  the  body.  Many  patients  are  able  to  describe  the 
beginning  symptoms  with  accuracy  after  recovery  from  even  a  pro- 
found coma  lasting  a  week  or  more.  Loss  of  consciousness  is  usual, 
however,  especially  in  hemorrhagic  cases,  less  often  present  in  throm- 
bosis or  embolism,  especially  in  the  beginning.  In  Jones'  series,  47.7 
per  cent,  of  cases  of  embolism  showed  loss  of  consciousness,  impair- 
ment in  60  per  cent.  Thromboses  show  a  similar  percentage,  while 
hemorrhage  is  accompanied  by  loss  of  consciousness  in  75  per  cent, 
of  the  cases. 

Coma  is  then  apt  to  develop  and  is  accompanied  by  stertorous 
breathing,  and  by  a  slow,  full  and  regular  pulse.  The  patient  is  pale, 
or  the  face  may  be  congested,  the  extremities  are  cold.  The  limbs 
are  usually  completely  relaxed.  Irritative  phenomena  are  usually 
more  indicative  of  cortical,  basal  or  ventricular  hemorrhages.     Minute 

1  See  995  cases  collected  by  Jones,  Brain,  1905, 


CEREBRAL  APOPLEXIES 


639 


variations  in  the  excursion  of  the  two  sides  of  the  chest,  irregularity 
of  the  usually  dilated  and  light-inactive  pupils,  minor  signs  of  irrita- 
bility (]\Iarie-Foix)  may  enable  one  to  locate  the  side  of  the  hemorrhage 
even  in  this  comatose  state.  Other  signs  are  loss  of  corneal  reflexes, 
no  reaction  to  painful  stimuli,  loss  of  reflexes  in  general,  occasionally 
involuntary  urination  and  defecation.  Retention  is  more  apt  to 
occur. 

In  severe  states  of  coma  with  marked  rise  in  temperature,  103°  F. 
to  105°  F.,  with  twitching  or  convulsive  jerks,  very  slow  heart  action, 
later  developing  symptoms  of  irregular  heart  and  breathing,  the 
hemorrhage  is  probably  very  extensive,  tends  to  flow  into  the  ventricles, 
and  death  ensues  in  a  fe«'  hours  or  a  few  davs. 


Fig.  .357. — Cerebral  hemorrhage  within  the  ventricle. 


Thalamic  cases  are  frequently  attended  with  marked  coma,  which 
may  be  very  protracted,  three  or  four  weeks,  without  definite  hemi- 
plegic  signs. 

Jacksonian  convulsive  attacks  usually  indicate  meningeal  bleeding. 
Some  patients  show  a  tendency  to  clouding  of  consciousness  which 
comes  and  goes. 

Reco\ery  from  the  immediate  attack  may  take  place  in  a  few  hours 
or  after  weeks  or  more.  The  residuals  found  will  vary  according  to 
the  location  and  extent  of  the  lesion.  It  is  in  this  stage  that  a  diagnosis 
of  the  localization  and  extent  of  the  lesion  can  be  made. 

Anterior  Cerebral  Syndrome. — In  emboli,  thrombi  and  hemorrhage 
of  the  anterior  cerebral  arteries  the  lesion  may  be  limited  or  be  very 
extensive, 


640 


DISEASES  OF  THE  BRAIN 


Mild  or  severe  and  acute  confusions  with  variously  colored  mental 
defect  states  are  frequently  the  result  of  minute  or  more  extensive 
changes  in  the  vascular  supply  of  the  frontal  lobes,  supplied  by  the 
anterior  cerebrals.  These  patients  not  infrequently  present  lacunar 
symptoms  when  the  medullary  vessels  are  involved.^  The  clinical 
picture  is  protean,  depending  on  the  severity  of  the  hemorrhage,  or 
softening  (from  embolus  or  thrombus)  and  the  distribution  of  these 
focal  softenings.  The  course  is  more  halting;  epileptiform  or  apo- 
plectiform attacks  with  acute  confusions  occur  from  which  the  patient 
is  apt  to  make  a  clinical,  not  anatomical,  recovery.  The  patient 
is  usually  in  the  fourth,  fifth  or  sixth  decade.  There  is  increasing 
sense  of  incompetency.     Vertiginous  attacks  occur,  with  headaches, 


V. 


a>.i>m"-^ 


Fig.  358. — Extensive  ventricular  hemorrhage  from  rupture  of  anomalous  (duplicated) 
anterior  communicating  artery. ^ 

often  migranous  in  character.  Speech  and  thought  associations  are 
interfered  with  and  minor  monoplegias  develop.  Apraxia  maj^  be  an 
isolated  symptom,  or  there  may  be  combined  apraxia  and  crural 
monoplegia. 

In  more  extensive  lesions,  softenings  occur  (lacunar),  the  patients 
seem  to  drop  large  portions  of  their  mental  life  quite  suddenly.  These 
softenings  usually  show  themselves  at  first  as  acute  excited  confusions, 
sometimes  even  manic-like  in  their  general  trend;  others  show  so- 
called  hysterical  coloring;  while  in  the  later  and  more  advanced  cases 
apraxias,  hallucinoses  and  more  global  defects  appear. 

>  Lambert:     State  Hosp.  Bull.,  1909,  ii,  459. 
2  Tilney,  F.:  Neurological  Bulletin,  1918. 


CEREBRAL  APOPLEXIES  641 

General  progression  results  in  marked  mental  enfeeblement — arterio- 
sclerotic deterioration — or  a  sudden  new  and  more  extensive  lesion  may 
cause  sudden  death. 

Irritathe  complexes  from  cortical  vascular  disease  develop  not 
infrequentl}',  even  in  younger  individuals  following  various  infections 
(thrombi,  emboli  of  cortical  vessels),  alcoholism,  syphilis,  lead.  Here 
transitory  mild  shock  may  usher  in  the  disorder  with  twitching,  jerky, 
choreiform  accompaniments,  or  these  motor  distiu-bances  may  later 
develop  as  residuals,  with  or  without  epileptiform  attacks.  Pares- 
thesise,  astereognosis,  weakness  of  an  arm  or  leg,  or  mild  paresis  of  the 
facial  musculature  may  develop.  True  epileptic  residuals  may  be 
permanent  symptoms  of  certain  of  these  cortical  arteriosclerotic  focal 
lesions. 

The  middle  cerebral  syndromes  are  the  most  frequent.  Here  hemi- 
ylegia  is  t.he  most  striking  residual  SATuptom.  In  total  hemiplegia, 
usually  capsular,  supracapsular,  the  face,  arm  and  leg  of  the  same  side 
are  involved.  The  upper  branches  of  the  facial,  wrinkling  of  forehead, 
closure  of  eye  are  not  usually  implicated.  Xor  are  the  eye  movements 
modified  unless  the  lesion  is  located  lower  down,  crura  or  in  the  mid- 
brain, when  another  syndrome  is  present.^  The  face  is  drawn  to  the 
healthy  side,  the  tongue  projects  to  the  paralyzed  side  on  protrusion. 
Dysphagia,  from  hemiparesis  of  the  palate  develops.  The  soft 
palate  .hangs  lower  on  the  paralyzed  side.  The  neck  may  be  involved, 
but  is  less  apt  to  be,  whereas  the  paralysis  of  the  arm  and  leg  are 
characteristic.     (See  Plate  X.) 

The  arm  is  flaccid,  flabby,  apt  to  be  edematous,  blue,  cold  and  boggy. 
The  reflexes  which  during  coma  are  lost,  gradually  return  and  shortly 
become  exaggerated.  Chaddock's  wrist  phenomenon  develops.  There 
is  little  or  no  atrophy  and  the  electrical  reactions  are  normal.  Later 
contractions  may  develop,  or  these  may  occur  closely  following  the 
attack  if  the  lesion  involves  only  a  portion  of  the  arm  fibers  or  en- 
croaches upon  the  thalamus.  The  abdominal  reflexes  are  diminished 
or  lost  on  the  paralyzed  side. 

The  leg  is  flaccid,  is  thrown  about  the  hip  like  a  flail,  may  be  edema- 
tous and  flabby,  and  also  shows  later  the  signs  of  pyramidal  tract, 
upper  motor  neuron  disorder,  i.  e.,  increased  knee-jerks,  ankle-clonus, 
Babinski  and  Chaddock  reflexes,  with  increased  muscle  tone  and 
normal  electrical  responses. 

The  grade  of  the  paralysis  varies  greatly.  With  some  it  is  a  transi- 
tory weakness  which  passes  in  a  few  weeks  o/  a  few  months,  in  others 
it  is  a  complete,  permanent  and  marked  paralysis,  with  later  develop- 
ing contractures  and  total  unilateral  disability.  Any  intermediary 
grade  may  be  expected.  In  the  milder  forms  of  hemiplegia  the  little 
signs — see  Examination  Methods — are  of  great  value  to  clearing  up 
the  situation,  since  some  mild  hemiplegias  may  be  confused  with 
psychogenic  palsies,  especially  in  thalamic  cases. 

'  Millard-Gubler,  Benedict,  etc.,  q.  v. 

41 


642  DISEASES  OF  THE  BRAIN 

Hemianesthesia  may  accompany  the  hemiplegia,  or  the  patient  may 
have  a  hemianesthesia  without  any,  or  with  minimal  hemiplegia. 
This  argues  for  the  implication  of  a  portion  of  the  thalamic  fibers. 

The  patients  complain  of  numbness,  coldness,  and,  depending  on  the 
location  of  the  lesion,  may  lose  their  stereognostic  sense,  may  not  be 
able  to  distinguish  heat,  nor  cold,  or  may  have  subjective  sensations, 
haptic  hallucinations  of  the  limb  area. 

Sensory  disturbances  from  cerebral  lesions  are  extremely  diverse 
and  of  great  importance  in  the  diagnosis  of  brain  disorder,  particularly 
in  arterial  disease  and  in  brain  tumors.  They  are  best  discussed  as 
parts  of  the  thalamic  or  suprathalamic  syndromes. 

The  involvement  of  the  hypothalamic  or  lenticular  regions  may 
cause  muscle  vegetative  signs,  various  tonic  anomalies,  etc. 

The  Thalamic  Syndrome.' — If  the  chief  body  of  the  thalamus  is 
involved  or  with  partial  implication  of  related  extrap\Tamidal  tracts, 
a  characteristic  neurological  complex  results,  termed  by  Dejerine  and 
his  pupil  Roussy  the  thalamic  syndrome.  It  is  one  of  the  middle 
cerebral  artery  combinations. 

The  chief  featiu-es  show  usually  after  an  apoplectic  attack  with 
the  ordinary  signs  of  a  severe  hemorrhage,  thrombosis  or  embolism. 
In  certain  syphilitic  cases  the  attack  may  be  comparatively  slight, 
or  the  syndrome  may  develop  with  no  signs  of  an  attack. 

Practically  the  entire  mass  of  sensory  fibers  carrying  impulses  of 
all  kinds — the  tests  for  most  of  which  have  already  been  outlined — 
have  their  synaptic  junctions  within  the  optic  thalamus.  These 
synaptic  junctions  are  the  thalamus.  Only  the  most  limited  attention 
can  be  given  here  to  the  numerous  fibers  coming  from  the  chemical 
receptors  of  the  respiratory,  gastro-intestinal,  or  genito-urinary  tract, 
or  to  those  from  the  organs  of  internal  secretion.  Some  of  these 
undoubtedly  made  their  synaptic  junctions  in  other  than  thalamic 
structures — globus  pallidus,  putamen,  etc.,  for  example.  The  thalamic 
(or  related)  synapses  of  these  vegetative  pathways  have  not  yet  been 
sufficiently  worked  out  for  didactic  presentation.  (See  Lenticular 
syndromes.) 

The  chief  features  of  the  thalamic  syndrome  are: 

1.  A  persistent  loss  of  superficial  sensation  of  one-half  of  the  body 
and  face.  This  loss  to  touch,  pain,  and  to  temperature  is  more  or 
less  definite,  subject  to  considerable  variation  and  to  partial  recovery, 
but  the  loss  of  deep  sensibility,  deep  pressure,  postural  sense,  etc., 
is  much  more  pronounced,  and  is  more  apt  to  persist.  This  latter 
is  usually  more  marked  distally  and  in  many  instances  diminishes 
proximally. 

2.  There  is  slight  hemiataxia  and  more  or  less  complete  astereog- 
nosis. 

3.  There  are,  in  the  complete  syndrome,  acute  pains  on  the  affected 
side,  which  are  very  persistent,  coming  on  in  paroxysms.     They  are 

1  Jelliffe:     The  Thalamic  Syndrome,  Med.  Record,  February  1,  1910,  for  references. 


THALAMIC  SYNDROME 


643 


frequently  extremely  severe  and  rarely  controlled  by  the  usual  anal- 
gesics. These  pains  may  involve  a  single  member,  may  be  limited 
to  the  side  of  the  face,  constituting  a  thalamic  trigeminal  neuralgia,  or 
they  may  involve  one  whole  side  of  the  body. 


F.  coriico- 
thaJain- 


Medulla 


Dorsal  column - 
sensory  paths 


^^,  Crossed  secondary 

,'  '  sensory  paths 


Fig.  359. — To  show  the  position  and  relations  of  the  optic  thalamus  in  the  central 
sensory  path.  Two  distinct  paths  exist  in  the  spinal  cord:  a  crossed  secondarj'  path  in 
the  ventrolateral  column  which  conveys  impressions  of  pain,  temperature,  and  touch, 
and  a  second  uncrossed  path  in  the  dorsal  column  which  also  carries  touch,  and  in  which 
run  impulses  that  underlie  the  sense  of  position,  the  appreciation  of  movement,  the 
discrimination  of  two  points,  and  the  recognition  of  vibration,  size,  shape,  form,  weight, 
and  consistence.  This  second  path  decussates  in  the  lower  part  of  the  meduUa  oblongata, 
but  runs  separate  from  the  first  path,  at  least  as  high  as  the  pons.  All  these  secondary 
sensory  fibers,  now  crossed,  terminate  in  the  ventrolateral  region  of  the  optic  thalamus. 
The  impressions  they  carry  are  regrouped  here  and,  through  intercalated  neurons,  are 
distributed  along  two  distinct  paths;  the  one  carries  impressions  to  the  cerebral  cortex, 
the  other  toward  the  more  mesial  parts  of  the  optic  thalamus.  The  corticothalamic 
fibers,  which  terminate  in  the  lateral  nucleus  of  the  optic  thalamus,  are  also  shown. 
(Head  and  Holmes.)     (Compare  Plate  XI.) 

4.  There  is  usually  a  more  or  less  distinct,  though  slight  hemiplegia 
which,  in  the  unmixed  syndromes,  rapidly  clears  up.  Contractures 
rarely  develop  in  the  pure  syndrome.  In  the  mixed  syndrome — with 
extension  of  the  lesion  to  the  internal  capsule — contractures  may  be 
present. 

5.  Choreic,  athetoid  or  paralysis-agitans-like  movements  may  be 
present  on  the  att'ected  side. 

These  are  the  chief  symptoms  of  optic  thalamus  disease,  but  in 
addition  to  these.  Head  and  Holmes  have  pointed  out  an  extremely 


644  DISEASES  OF  THE  BRAIN 

suggestive  series  of  affective  reactions  which  are  due  to  lesions  which 
cut  the  optic  thalamus  from  its  cortical  connections.  They  have 
opened  up  an  attack  upon  the  analysis  of  the  sensory  content  of 
emotional  reactions.  They  show  that  in  this  type  of  lesion  there  is 
a  tendency  to  react  excessively  to  pleasant  or  unpleasant  stimuli. 
The  prick  of  a  pin,  painful  pressure,  excessive  heat  or  cold,  all  produce 
more  distress  than  on  the  normal  half  of  the  body.  Thus,  in  one  of 
Head  and  Holmes's  patients,  if  a  pin  was  dragged  lightly  across  the 
face  or  trunk,  from  the  sound  to  the  affected  side,  there  was  felt  an 
excessive  discomfort  as  it  passed  the  middle  line.  The  patient  not 
only  complained  that  it  hurt,  but  the  face  was  contorted  with  pain, 
and  all  this  notwithstanding  the  fact  that  there  was  less  ability  to 
distinguish  head  from  point,  yet  the  prick  was  more  disagreeable. 
This  anomalous  state  of  affairs  is  characteristic  of  a  thalamic  involve- 
ment.^ 

This  excessive  affective  reactivity  is  present  not  only  to  pin-prick, 
but  also  to  deep  pressure,  to  extremes  of  heat  and  cold,  to  visceral 
stimulation,  to  scraping,  roughness,  vibration,  tickling,  to  pleasurable 
stimuli  and  to  ideational  emotional  states.  Not  all  patients  show  all 
of  these  reactions,  but  in  practically  90  per  cent,  of  the  thalamic  cases 
examined  by  Head  and  Holmes,  excessive  affective  response  to  one 
or  more  measured  stimuli  was  found.  For  heat  and  cold  and  other 
forms  of  sensibility  as  well  as  for  pain,  the  excessive  response  may  be 
present,  and  yet  the  patients  are  unable  to  detect — i.  e.,  are  anesthetic 
to — the  stimulus  itself.  So  far  as  the  ideational  affective  reaction  is 
concerned,  these  patients  express  themselves  as  follows:  On  hearing 
affecting  music,  "A  horrid  feeling  came  on  in  the  affected  side,  and  the 
leg  screwed  up  and  started  to  shake."  The  singing  of  a  comic  song 
left  one  patient  absolutely  cold,  but  a  tragic  song  produced  a  very 
distinct  unpleasant  effect.  One  patient  said,  "My  right  hand  seems 
to  crave  sympathy,  my  right  side  seems  more  artistic."  In  practically 
all  of  the  cases  the  increased  affective  reaction  was  accompanied  by 
actual  sensory  loss. 

A  more  detailed  study  of  the  loss  of  sensibility  in  thalamic  disorders 
made  by  Head  and  Holmes  revealed  the  following:  No  sensory  func- 
tions are  so  frequently  affected  as  the  appreciation  of  posture  and  the 
recognition  of  passive  movement.  The  amount  of  this  loss  varies 
greatly  from  a  scarcely  measurable  defect  to  complete  want  of 
recognition  of  the  posture  of  the  limbs  of  the  abnormal  half  of  the 
body. 

Tactile  sensibility  is  frequently  diminished,  but,  excepting  in  a  few 
cases,  where  all  appreciation  of  contact  was  destroyed,  a  threshold 
could  be  obtained.  It  was  always  possible  to  show  that  increasing 
the  strength  of  the  stimulus  improved  the  proportion  of  right  answers 
unless  the  observations  were  confused  by  the  disagreeable  tingling  or 
other  accessory  sensations. 

1  Byrnes,  J,:     Jour-  Nerv.  &nd  Ment.  Pis.,  1919;  Alienist  and  Neurologist,  1919. 


THALAMIC  SYNDROMES  645 

Localization  of  the  spot  touched  was  defective  in  half  the  cases  where 
sensation  was  sufficiently  preserved  to  carry  out  accurate  tests.  This 
inability  to  recognize  the  site  of  stimulation  was  equally  great,  whether 
the  patient  was  pricked  or  touched.  In  cases  where  localization  was 
gravely  affected,  the  disagreeable  sensation,  so  easily  evoked,  tended 
to  spread  widely  on  the  abnormal  half  of  the  body.  A  prick  on  the 
hand  may  cause  an  extremely  painful  sensation  in  the  cheek  or  side, 
and  sometimes  the  patient  simply  recognized  the  stimulus  as  a  change 
within  himself,  and  did  not  refer  the  discomfort  from  which  he  suffered 
to  the  action  of  any  external  agent. 

Sensibility  to  heat  and  cold  may  show  all  degrees  of  change  from 
total  loss  to  a  slight  increase  of  the  neutral  zone.  Heat  and  cold  are 
rarely  dissociated^  and  if  one  form  of  sensation  is  lost,  the  other  will 
be  gravely  disturbed.  The  apparent  exceptions  arise  from  a  misin- 
terpretation of  the  sensation  evoked  by  high  or  low  temperatures  on 
the  affected  half  of  the  body. 

Not  infrequently  the  compass  test  cannot  be  carried  out  because 
of  the  gross  loss  of  sensation  and  inability  to  recognize  contact;  but 
whenever  this  method  can  be  applied  a  threshold  can  be  worked  out, 
and  widening  the  distance  between  the  points  increases  the  accuracy 
of  the  answers. 

The  power  of  estimating  the  relation  between  two  weights  is  fre- 
quently disturbed  on  the  involved  half  of  the  body.  If  the  apprecia- 
tion of  postm-e  and  movement  is  affected,  the  patient  can  no  longer 
recognize  the  identity  or  the  difference  of  two  weights  placed  on  the 
unsupported  hands.  But  so  long  as  tactile  sensibility  is  not  dimin- 
ished, the  patient  can  still  estimate  the  relation  between  weights 
applied  one  after  the  other  on  the  same  spot,  and  can  recognize  the 
increase  or  diminution  in  weight  of  an  object  already  resting  on  the 
hand. 

The  appreciation  of  relative  size  is  often  disturbed  in  these  cases. 
With  care  it  is  usually  easy  to  demonstrate  a  difference  in  the  threshold. 
Shape  and  form  in  three  dimensions  are  frequently  not  recognizable 
on  the  affected  hand.  But,  if  tactile  sensibility  is  not  grossly  affected, 
the  patient  usually  retains  an  idea  that  the  object  possesses  a  form, 
and  may  give  a  considerable  percentage  of  right  answers. 

Vibration  of  the  tuning-fork  is  felt,  in  almost  all  thalamic  cases, 
but  the  length  of  time  during  which  it  is  appreciated  is  usually  shorter, 
and  sometimes  the  rate  of  vibration  is  thought  to  be  slower  on  the 
affected  half  of  the  body. 

Roughness,  as  tested  with  Graham  Brown's  esthesiometer  is  usually 
recognized,  except  in  those  cases  in  which  the  loss  of  all  forms  of  sensa- 
tion is  unusually  severe. 

Partial  syndromes  are  more  frequent  than  the  classical  syndrome 
described.  To  summarize:  the  main  symptoms  of  the  complete 
syndrome  are: 

'  Tilney  and  Cornell,  report  a  Thalamic  Syndrome  with  Thermal  Dissociation, 
Neurol.  Bull.,   1918,  vol.  i. 


646  DISEASES  OF   THE  BRAIN 

1.  Persistent  hemianesthesia,  more  or  less  marked  for  superficial  sen- 
sibility, tactual  pain,  temperatiu-e,  most  marked  for  deep  sensibility 
and  hence  loss  or  diminution  of  postural  sense  and  astereognosis. 

2.  Mild  hemiplegia,  usually  regressive. 

3.  Mild  hemiataxia,  with  choreo-athetoid  movements  either  spon- 
taneous or  on  an  attempted  movement.     Adiadokokinesis  at  times. 

4.  Paroxysmal,  shooting,  neuralgic  pains,  often  very  persistent  and 
severe  and  not  helped  by  the  usual  analgesics, 

5.  Overresponse  to  affective  stimuli,  particularly  on  affected  side, 
with  emotional  hyperactivity  to  varied  sensory  impressions. 

Sensory  Changes  in  Cortical  Lesions,  Suprathalamic  Pathways. — 
The  analysis  of  sensory  stimuli  in  cerebral  and  thalamic  disorders  is 
of  the  greatest  importance  in  cerebral  localization,  hence  an  extended 
presentation  of  contemporary  work  is  desirable.  In  the  chapter  on 
Peripheral  Nerves  the  chief  sensory  syndromes  of  the  first  sensory 
neurons  were  discussed.  Those  of  the  cord  are  presented  elsewhere. 
Redistributions  take  place  in  the  medulla  and  midbrain.  Those 
disturbances  due  to  lesions  within  the  thalamus  have  just  been  pre- 
sented. Finally  the  thalamocortical  pathways  make  a  further  regroup- 
ing of  sensory  qualities  in  the  entire  course  of  the  sensory  pathways. 
(See  Plates  X  and  XI.) 

The  analysis  of  these  phenomena  introduces  complex  factors,  and 
it  is  necessary  to  abandon  all  generalizations,  even,  for  instance,  light 
touch,  cutaneous  sensation.  The  results  of  the  test  must  be  stated 
in  terms  of  the  tests  employed.  (See  Sensory  Examination 
Methods.) 

Using  graduated  tactile  stimuli,  such  as  von  Frey's  hairs  and  the 
pressure  esthesiometer,  the  chief  results  have  been  that  a  cortical 
lesion  may  reduce  the  accuracy  of  response  from  the  affected  part 
to  graduated  tactile  stimuli.  The  form  assumed  by  this  defective 
sensibility  differs  from  that  produced  by  lesions  at  other  levels  of  the 
nervous  system.  The  affected  part  may  respond  to  the  same  graduated 
hair  as  the  normal  part;  but  this  response  is  irregular  and  uncertain. 
Increasing  the  stimulus  may  lead  to  no  corresponding  improvement, 
and  even  the  strongest  tactile  hair  may  occasionally  evoke  less  certain 
answers  than  a  hair  of  much  smaller  bending  strain.  Moreover,  a 
touch  with  the  unweighted  esthesiometer  may  be  as  effective  at  one 
moment  as  the  same  instrument  weighted  with  30  grams  at  another. 
In  such  cases  no  tactile  threshold  can  be  definitely  obtained.  This 
irregularity  of  response  is  associated  with  persistence  of  the  tactile 
sensation  and  a  tendency  to  hallucinations  of  touch.  Where  the 
sensory  defect  is  not  sufficiently  gross  to  abolish  the  threshold,  per- 
sistence, irregularity  of  response  and  a  tendency  to  hallucinate  may 
still  disturb  the  records. 

In  all  cases  where  tactile  sensibility  is  affected,  whether  a  threshold 
can  be  obtained  or  not,  fatigue  is  induced  with  unusual  facility. 
Although  the  patient  may  cease  to  respond  to  tactile  stimuli  over  the 
affected  part  in  consequence  of  fatigue,  his  answers  may  remain  as 


SUBCORTICAL  SENSORY  SYNDROMES  647 

good  as  before  from  the  normal  ])arts.  The  fatigue  is  local  and  not 
general. 

With  stationary  cortical  lesions,  uncomplicated  by  states  of  shock 
or  by  "diaschisis,"  sensibility  to  touch  with  cotton-wool  is  rarely 
lost  over  hair-clad  parts.  Over  hairless  parts,  stimulation  with  cotton- 
wool may  produce  a  sensation  which  seems  "less  plain"  to  the  patient, 
and  his  answers  may  show  the  same  inconstancy  so  evident  when 
he  is  tested  with  graduated  tactile  stimuli. 

A  pure  cortical  lesion  leads  to  no  change  in  the  threshold  to  measur- 
able, painful  or  uncomfortable  stimuli.  Nor  does  the  patient  express 
greater  dislike  to  these  stimuli  on  one  side  than  on  the  other.  A 
prick  may  be  said  to  be  "plainer"  or  "sharper"  on  the  normal  than 
on  the  affected  side;  but  this  is  due  to  a  defective  appreciation  of  the 
pointed  nature  of  the  stimulus  and  bears  no  direct  relation  to  the 
painfulness  of  the  sensation  evoked. 

Temperature  tests  show  that  (a)  the  neutral  zone,  wdthin  the  stimu- 
lus, was  said  to  be  neither  hot  nor  cold,  was  considerably  enlarged  in 
comparison  with  that  observed  on  similar  normal  parts  of  the  same 
patient,  (b)  The  patient  complained  that,  although  he  recognized 
correctly  the  nature  of  the  stimulus,  it  seemed  "less  plain"  than  over 
normal  parts.  His  answers  were  less  constant,  and  less  certain;  a 
temperature  recognized  without  difficulty  at  one  time  seemed  doubt- 
ful at  another,  (c)  The  power  of  discriminating  the  relative  coolness 
of  two  stimuli  or  the  relative  warmth  of  two  hot  tubes  may  be  dimin- 
ished. Thus  20°  C.  may  be  said  to  be  the  same  as  ice,  although  both 
are  uniformly  called  cold,  and  40°  C.  may  seem  as  warm  as  or  even 
warmer  than  48°  C.  The  faculty  of  appreciating  the  relation  to  one 
another  of  two  temperatures  on  the  sarm  side  of  the  scale  is  dis- 
turbed. 

Tests  for  posture  and  for  passive  movements  show  that  (a)  cortical 
lesions  most  frequently  disturb  the  recognition  of  posture  and  of  pas- 
sive movements.  Whenever  sensation  is  in  any  way  affected  in  con- 
sequence of  a  cortical  lesion  these  two  functions  suffer.  (6)  In  all 
their  cases  the  disturbance  in  the  faculty  of  recognizing  posture  and 
passive  movements  was  greater  toward  the  peripheral  parts  of  the 
affected  limb,  (c)  When  a  patient  with  unilateral  disturbance  of  these 
faculties  attempts  to  point  to  some  part  of  his  body,  defective  knowl- 
edge of  its  position  causes  greater  error  than  want  of  recognition  of 
posture  and  mov^ement  in  the  hand  with  which  he  points,  (d)  When 
testing  the  patient's  power  of  appreciating  passive  movement,  the 
answers  are  frequently  uncertain  and  hallucinations  of  movement 
may  occur.  And  yet  the  patient  may  be  remarkably  consistent  and 
accurate  when  normal  parts  are  tested. 

Localization  tests  show  that  (a)  the  power  of  localizing  the  stimulated 
spot  is  not  infrequently  preserved,  although  sensat'on  may  be  other- 
wise disturbed  as  a  consequence  of  cortical  lesions.  (6)  This  faculty 
is  independent  of  the  power  of  recognizing  the  position  of  the  affected 
limb;  appreciation  of  posture  may  be  lost,  although  localization  is 


648  DISEASES  OF  THE  BRAIN 

not  in  any  way  diminished,  (c)  If  the  power  of  locaKzation  is  lost,  the 
patient  will  be  unable  to  recognize  not  only  the  position  of  a  spot 
touched  but  also  the  position  of  a  prick,  {d)  When  localization  is 
defective  in  consequence  of  cerebral  lesions,  the  patient  does  not 
habitually  localize  in  any  particular  direction,  but  ceases  to  be  certain 
where  he  has  been  touched  or  pricked. 

The  compass  test  shows  that  (a)  a  cortical  lesion  may  destroy  the 
power  of  discriminating  two  compass  points,  both  when  applied  simul- 
taneously and  consecutively.  If  this  is  the  case,  no  threshold  can  be 
obtained  for  either  form  of  the  test;  increasing  the  distance  between 
the  points  does  not  constantly  improve  the  accuracy  of  the  answers. 
(6)  This  disturbance  is  not  caused  by  changes  in  tactile  appreciation; 
for  it  can  be  demonstrated  equally  well  with  two  painful  as  with  two 
tactile  stimuli,  (c)  The  condition  of  tactile  sensibility  and  the  accu- 
racy of  the  simultaneous  compass  test  are  closely  associated;  a  dis- 
turbance of  the  tactile  threshold  is  usually  accompanied  by  a  raised 
threshold  for  the  appreciation  of  two  points  applied  simultaneously. 
(d)  Should  the  power  be  preserved  of  recognizing  two  points  when  the 
compasses  are  applied  consecutively,  localization  vvill  be  found  to  hd 
intact.  The  patient's  appreciation  of  the  two  points  when  they  are 
separated  by  an  interval  of  time  is  due  to  the  recognition  of  the  separate 
locality  of  the  two  spots  touched. 

Appreciation  of  weights  shows  that  (a)  the  power  of  estimating  the 
relation  of  two  objects  of  the  same  size  and  shape  is  readily  disturbed 
by  cortical  lesions,  (b)  Though  the  patient  may  retain  sensations  of 
contact  when  the  weight  is  placed  in  his  hand,  all  power  of  recognizing 
the  relative  heaviness  of  the  object  has  disappeared,  (c)  This  faculty 
is  equally  disturbed  in  most  cases  whether  the  weights  are  placed  on 
the  supported  or  the  unsupported  hand. 

From  these  and  related  studies.  Head  and  Holmes  maintain  that 
sensory  impulses  pass  from  the  thalamus  to  the  cortex  in  five  groups: 

1.  Those  concerned  with  the  recognition  of  posture  and  passive 
movement.  If  these  impulses  are  affected  the  power  of  discriminat- 
ing weights  on  the  unsupported  hand  may  be  also  diminished. 

2.  Certain  tactile  elements;  integrity  of  this  group  is  necessary  for 
the  discrimination  of  weights  placed  on  the  fully  supported  hand. 

3.  Those  impulses  which  underlie  the  appreciation  of  two  points 
applied  simultaneously  (the  compass  test) ;  on  this  group  also  depends 
the  recognition  of  size  and  shape. 

4.  Those  which  underlie  the  power  of  localizing  the  situation  of  a 
stimulated  spot.  Recognition  of  the  double  nature  of  two  points 
applied  consecutively  also  depends  on  this  group  of  impulses. 

5.  All  thermal  impulses  are  grouped  together  to  underlie  a  scale  of 
sensations  with  heat  at  one  end  and  cold  at  the  other.  At  the  level 
with  which  we  are  now  dealing  these  impulses  have  already  excited 
the  aflfective  center  and  are  passing  away  to  the  cortex. 

The  functional  integrity  of  the  cortex  enables  attention  to  be 
concentrated  upon  those  changes  which  are  produced  by  the  arrival 


SUBCORTICAL  SENSORY  SYNDROMES  649 

of  afferent  impulses  (Head  and  Holmes).  When  this  is  distm-bed, 
some  impulses  evoke  a  sensation,  but  others,  from  lack  of  attention, 
do  not  affect  consciousness.  Attention  no  longer  moves  freely  over 
the  sensory  field  to  be  focussed  successively  on  fresh  groups  of  sensory 
impressions.  Sensations,  once  evoked,  are  not  cut  short  by  the  moving 
away  of  the  focus  of  attention  as  when  cortical  activity  is  perfect. 
Hence  arise  persistent  sensations  and  hallucinations  which  are  so 
prominent  a  feature  of  lesions  of  the  cortex. 

The  cerebral  cortex  is  the  organ  by  which  attention  may  be  focussed 
upon  the  changes  evoked  by  sensory  irhpulses.  Such  attention  is  to 
further  the  useful  work  in  hand.  A  pure  cortical  lesion,  which  is  not 
advancing  or  causing  periodic  discharges,  will  change  the  sensibility 
of  the  affected  parts  in  such  a  way  that  the  patient's  answers  appear 
to  be  untrustworthy.  Such  diminished  power  makes  the  estimation 
of  a  threshold  in  many  cases  impossible.  Uncertainty  of  response 
destroys  all  power  of  comparing  one  set  of  impressions  with  another 
and  so  prevents  discrimination.     This  interferes  with  function. 

In  addition  to  its  function  as  an  organ  of  local  attention  the  sensory 
cortex  is  also  the  storehouse  of  past  impressions.  These  may  rise  into 
consciousness  as  images,  but  more  often,  as  in  the  case  of  spatial 
impressions,  remain  outside  of  central  consciousness.  Here  they  form 
organized  models  which  may  be  termed  "schemata."  Such  patterns 
modify  the  impressions  produced  by  incoming  sensory  impulses  in 
such  a  way  that  the  final  sensations  of  position,  or  of  locality,  rise  into 
consciousness  charged  with  a  relation  to  something  that  has  happened 
before.  Destruction  of  such  "schemata"  by  a  lesion  of  the  cortex 
renders  impossible  all  recognition  of  posture  or  of  the  locality  of  a 
stimulated  spot  on  the  affected  part  of  the  body. 

In  daily  life  all  stimuli  excite  more  or  less  both  thalamic  and  cor- 
tical centers,  for  most  unselected  sensations  contain  both  affective 
and  discriminative  elements.  But  among  the  tests  employed  in  sen- 
sory analysis,  some  appeal  almost  entirely  to  the  one  or  the  other 
center.  The  test  for  recognition  of  posture,  as  carried  out  by  Head 
and  Holmes,  is  purely  discriminative;  while  the  pain  produced  by 
squeezing  the  testicle,  or  to  a  less  degree  by  the  pressure  algometer, 
appeals  almost  exclusively  to  the  more  affective  center. 

Sensory  impulses  arri\ing  at  the  optic  thalamus  are  regrouped  in 
such  a  way  that  they  can  act  upon  both  what  Head  calls  its  essential 
center  and  the  sensory  cortex.  The  essential  organ  of  the  thalamus 
is  excited  to  affective  activity  by  certain  impulses,  and  refuses  to  react 
to  those  which  underlie  the  purely  discriminative  aspects  of  sensation. 
These  pass  on  to  influence  the  cortical  centers  where  they  are  readily 
accepted.  In  a  similar  way,  the  primary  centers  of  the  cortex  canjiot 
receive  those  components  which  underlie  feeling  tone;  in  this  direction 
they  are  completely  blocked. 

It  has  long  been  recognized  that  sensations  accumulate  feeling 
tone  to  difierent  degrees.  In  those  which  underlie  postural  apprecia- 
tion this  quality  is  entirely  absent,  while  visceral  sensations  are, 


650  DISEASES  OF  THE  BRAIN 

in  some  instances,  little  more  than  a  change  in  a  general  feeling  tone; 
the  former  set  of  impulses  appeals  almost  exclusively  to  the  cortical 
center,  the  latter  to  that  of  the  optic  thalamus.  All  thermal  stimuli, 
however,  make  a  double  appeal.  Every  sensation  of  heat  or  cold  is 
either  comfortable  or  uncomfortable;  the  only  entirely  indifferent 
temperature  is  one  that  is  neither  hot  nor  cold. 

In  the  same  way,  some  unselected  tactile  stimuli  appeal  both  to 
the  sensory  cortex  and  to  the  optic  thalamus.  For  not  only  is  a  touch 
always  related  to,  and  distinguished  from,  something  that  has  gone 
before  it,  but  it  is  known  that  contact,  especially  of  an  object 
moving  over  hair-clad  parts,  is  capable  of  exciting  thalamic  activity. 
Vibration  of  the  tuning-fork  also  makes  a  double  appeal,  for  when 
the  cortical  parts  are  cut  the  amplitude  of  the  vibration  must  be 
greater  in  order  that  it  may  be  appreciated;  on  the  other  hand,  the 
vibratory  effect  may  be  stronger  on  the  abnormal  side  in  those 
thalamic  cases  where  the  affective  response  is  excessive. 

But  these  two  centers  of  consciousness  are  not  coequal  and  inde- 
pendent. Under  normal  conditions  the  activity  of  the  thalamic 
center,  though  of  a  different  nature,  is  dominated  by  that  of  the  cortex. 
When  the  sensation  normally  produced  by  a  prick  is  examined  it  is 
recognized  that  the  pain  develops  slowly  and  lasts  a  considerable  time 
after  the  stimulus  has  ceased.  Moreover,  the  same  intensity  of  stimu- 
lation will  produce  a  different  effect  on  the  same  spot  on  different 
occasions.  A  long  latent  period,  persistence  and  want  of  uniformity 
are  characteristic  of  all  painful  sensations.  This  is  seen  in  an  exag- 
gerated form  in  cases  where  the  thalamic  center  has  been  freed  from 
control.  The  response  to  prick  is  slow,  but  persists  long  after  the 
stimulus  has  ceased.  Moreover,  the  reaction,  when  it  occurs,  tends 
to  be  explosive;  it  is  as  if  a  spark  had  fired  a  magazine  and  the  conse- 
quences are  not  commensurate  with  the  cause.  Head  aptly  describes 
the  peculiarity  of  thalamic  activity  as  being  characterized  by  extensity 
rather  than  intensity;  the  whole  or  nothing  reaction,  affective  tone 
rather  than  sensory  quality  being  the  main  result  of  the  stimulus; 
absence  of  projections  and  relation  of  the  stimulus  to  the  body  rather 
than  to  an  external  object.  These  same  peculiarities  are  common  to 
protopathic  sensibility. 

On  the  contrary,  the  sensations  normally  produced  by  moderate 
tactile  stimuli  are  characterized  by  a  short  latent  period,  and  disappear 
almost  immediately  on  the  cessation  of  the  stimulus.  A  lesion  of  the 
sensory  cortex  disturbs  both  these  characteristics.  Tactile  sensa- 
tions becomes  uncertain  and  incalculable,  and  no  threshold  can  be 
obtained;  persistence  and  hallucinations  mar  the  uniformity  of  the 
records. 

The  work  of  Head  and  Holmes  tends  to  show  that  the  sensory  cortex 
is  the  organ  by  which  attention  can  be  concentrated  on  any  part  of 
the  body  that  is  stimulated.  The  focus  of  attention  is  arrested  at  any 
one  spot  by  the  changes  produced  by  cortical  activity.  These  are 
sorted  out  and  brought  into  relation  with  other  sensory  processes, 


CORTICAL  SENSORY  SYNDROMES  651 

past  and  present.  Then  the  focus  of  attention  sweeps  on,  attracted  by 
some  other  stimuhis. 

All  stimuli  which  reach  as  high  up  as  the  thalamic  center  have  a  high 
threshold.  They  must  reach  a  high  intensity  before  they  can  enter 
consciousness,  but  once  they  have  risen  above  the  threshold  they  tend 
to  produce  a  change  of  excessive  amount  and  duration,  and  this  it  is 
the  business  of  the  cortical  mechanism  to  control.  The  low  intensity 
of  the  stimuli  that  can  arouse  the  sensory  cortex,  and  its  quick  reaction 
period,  enable  it  to  control  the  activity  of  the  cumbersome  mechanism 
of  the  thalamic  center. 

This  view  of  the  sensory  mechanism  explains  many  of  the  facts 
recognized  by  students  of  disorders  of  the  nervous  system.  It  enables 
one  to  understand  how  integrations  can  occur  at  all  afferent  levels  of 
the  nervous  system,  and  make  development  possible  even  in  the 
individual.  The  aim  of  human  evolution  is  the  control  of  feeling  and 
instinct  by  discriminative  mental  activities.  This  struggle  on  the 
highest  plane  of  mental  life  is  begun  at  the  lowest  afferent  level,  and 
the  issue  becomes  more  clearly  defined  the  nearer  sensory  impulses 
approach  the  field  of  consciousness.  Finally,  in  terms  of  Bergson, 
"the  cerebral  mechanism  is  arranged  just  so  as  to  thrust  back  into 
the  unconscious  almost  the  whole  of  our  past  and  to  allow  beyond  the 
threshold  onlv  that  which  will  further  the  work  in  hand,  to  do  useful 
work." 

Cortical  Sensory  Disturbances. — Continuing  his  researches  to  the 
cortex,  Head,^  from  an  analysis  of  a  large  number  of  war  injuries 
of  the  brain,  first  calls  attention  to  the  fact,  that  a  gross  lesion  of  the 
so-called  "sensory  cortex"  does  not  abolish  any  of  the  four  primary 
qualities  of  sensation,  as  does  a  lesion  of  the  brain-stem  or  spinal 
cord,  and  yet  in  a  case  of  such  cortical  injury  the  answers  of  the  patient 
to  tests  show  that  these  qualities  are  in  some  w^aj'  disturbed.  It  is 
to  the  precise  examination  of  these  disturbances  that  Head  has  applied 
his  experience,  and  he  finds  that  if  the  sensibility  of  the  hand,  for 
example,  be  carefully  tested,  disturbances,  perhaps  confined  to  certain 
digits,  will  be  revealed.  These  disturbances  will  affect,  not  crude 
recognition  of  touch,  pain,  heat  and  cold,  but  three  discriminative 
faculties:  (1)  The  recognition  of  spatial  relations,  in  particular  the 
recognition  of  passive  movement  of  the  affected  part.  Discrimination 
of  two  compass  points  and  the  topical  localization  of  stimuli,  which 
come  within  this  faculty,  may  also  be  disturbed,  but  in  lesser  degree. 
(2)  Appreciation  of  differences  in  intensity  of  pain,  touch,  heat  and 
cold.  To  test  capacity  in  estimating  these  differences  is  hard. 
"Appreciation  of  heat  and  cold,  as  one  is  forced  to  examine  it  clinic- 
ally, depends  comparatively  little  on  cortical  activity.  For  while  it 
is  easy  to  say  whether  an  object  is  hot  or  cold,  it  requires  an  intelli- 
gent patient  and  unusually  favorable  conditions  to  test  the  relation  of 
two  warm  stimuli  to  one  another,  or  to  determine  the  threshold  for 

•Head:     Brain,  1918,  Pt.  II. 


652 


DISEASES  OF   THE   BRAIN 


heat  and  cold  with  approximate  accuracy.  These  difficulties  are  mul- 
tipled  a  hundredfold  in  the  case  of  pain."  In  the  case  of  touch,  how- 
ever, useful  results  may  be  obtained  by  measurement  with  tactile 
hairs  of  graduated  strength.  (3)  The  recognition  of  similarity  and 
difference,  thus,  the  size,  shape  and  weight  of  objects  and  the  texture 
of  stuffs,  apart  from  their  purely  tactile  qualities.  These  are  the  three 
faculties  which  fundamentally  depend  upon  the  activity  of  the  sensory 
cortex. 


Fig.  360  Fig.  361  Fig.  362 

Figs.  360,  361  and  362. — Topography  of  the  sensory  disturbance,  in  a  case  of  thalamic 
syndrome,  right  side,  of  six  years'  duration  in  a  woman,  aged  fitty-five  years.  There 
is  mild  hemiplegia  of  the  right  side  with  intense  choreo-athetoid  movements  of  the 
hand  and  foot.  There  is  a  marked  ataxia  in  all  voluntary  movements  of  the  same 
side.     Exaggeration  of  the  tendon  reflexes  of  the  right  side.     No  Babinski  reflex. 

There  are  very  active  and  severe  spontaneous  pains  over  the  entire  right  side;  tactile 
pain  and  thermal  sensibility  is  diminished,  but  not  abolished,  on  the  entire  right  side. 
Compass  discrimination  markedly  affected.  Complete  astereognosis.  Taste,  smell 
and  hearing  are  diminished  on  the  right  side.  Vision  is  unimpaired  and  there  is  no 
hemianopsia.  Deep  sensibiUty  is  more  altered  than  superficial.  The  sense  of  position 
is  altered,  bony  sensibility  is  much  diminished.     (Thomas  and  Chiray.) 

How  does  the  cortex  handle  and  utilize  the  sensory  material  it 
receives?  In  doing  this  the  observer  must  rely  upon  the  study  of 
dissociated  sensibility,  of  the  consequences  produced  by  interferences 
at  various  known  points  in  the  nervous  sj^stem,  where  some  impulses 
are  intercepted,  while  others  pass  on  to  reach  the  highest  receptive 
centers  and  form  the  underlying  basis  of  an  abnormal  sensation.  "The 
clue  to  the  situation  is  given  by  the  behavior  of  the  optic  thalamus. 
Until  recently  the  part  played  by  this  organ  was  unknown.  But  it  is 
now  recognized  that  it  is  the  seat  of  those  physiological  processes 


APRAXIA  653 

which  underhe  crude  sensations  of  contact,  pain,  heat  and  cold, 
together  with  the  feehng  tone  they  evoke.  The  essential  organ  of  the 
optic  thalamus  is  the  center  for  the  affective  aspect  of  sensation,  while 
discrimination  and  spatial  projection  are  the  product  of  cortical 
activity."  And  it  follows  that  if  a  lesion  be  subcortical  a  mixed  dis- 
turbance will  result,  one  having  both  thalamic  and  cortical  characters, 
either  dominating  in  accordance  with  propinquity  to  the  respective 
levels.  Touching  the  term  "level,"  Head  is  careful  to  point  out  that, 
following  Hughlings  Jackson,  he  uses  the  term  in  its  physiological 
rather  than  its  anatomical  sense.  In  addition  to  sensory  changes, 
an  uncomplicated  lesion  of  the  sensory  cortex  may  produce  hypotonia; 
this  is  a  slight  but  unmistakable  change,  best  seen  in  the  hands,  com- 
pletely different  from  paralytic  hypotonia  and  closely  associated 
with  the  failure  to  recognize  posture  and  passive  movement.  Con- 
cerning the  anatomical  localization  of  these  cortical  sensory  functions, 
they  may  be  placed  in  the  pre-  and  postcentral  convolutions,  the  anter- 
ior part  of  the  superior  parietal  lobule  and  the  angular  g^Tus.  But  it 
is  the  functions  rather  than  the  anatomical  relations  of  any  one  part 
of  the  body  that  are  represented  in  these  centers.  Hence,  those 
portions,  such  as  the  hand,  which  are  endowed  with  the  highest 
powers  of  discriminative  sensibility,  are  most  exclusively  represented. 
Next  in  order  comes  the  sole  of  the  foot,  which  constantly  exerts  a 
discriminative  action  in  walking.  Accordingly,  a  cortical  lesion  may 
disturb  the  sensibility  of  the  hand  and  foot  without  of  necessity 
affecting  the  elbow,  shoulder  or  knee. 

Apraxia. — This  term  was  first  used  by  Gogol  in  1873,  in  a  Breslau 
thesis  on  Ap.hasia.  His  patient  ate  his  soap,  urinated  in  his  water 
pitcher,  and  was  described  as  having  lost  his  understanding  for  objects. 
Such  defects  had  been  noted  before,  and  it  is  worthy  of  note  that 
Hughlings  Jackson,  in  1SG6,  called  attention  to  a  similar  type  of 
phenomenon,  and  attached  much  importance  to  it.  Quaglino,  in 
1867,  described  a  case;  Finkelnburg,  in  1870,  another,  in  which  recog- 
nition of  things  and  people  was  lost,  and  created  the  term  asymbolia. 
Wernicke,  in  1874,  expanded  the  term  asymbolia;  while  Freud  finally 
utilized  the  term  agnosia,  to  cover  all  types  of  loss  of  sensory  or  motor 
object  images,  apraxia  then  being  arranged  as  a  form  of  loss  of  knowl- 
edge of  objects,  really  a  form  of  visual  agnosia.  The  superficial 
observer  calls  these  patients  "demented." 

From  this  early  use  of  the  term  apraxia,  there  has  been  a  distinct 
variation,  brought  prominently  into  the  foreground  by  Liepmann 
in  1900.  He  defined  the  disturbance  as  a  lack  of  knowledge  of  the 
use  of  objects,  although  there  was  no  true  agnosia,  or  loss  of  recog- 
nition of  what  they  were.  Out  of  the  later  studies  of  Liepmann, 
Pick,  von  Monakow,  d' Hollander  and  others  has  come  the  following 
general  definition  of  apraxia. 

It  consists  in  an  inability  to  i)erform  certain  subjectively  purposeful 
movements,  or  movement  combinations,  the  motor  power,  sensation, 
and  coordination  being  intact.     Such  an  inability  will  naturally  depend 


654 


DISEASES  OF   THE  BRAIN 


upon  at  least  three  factors;  one  may  be  unable  to  recognize  the  object 
which  is  to  be  used,  in  which  case  there  is  a  sensory  apraxia,  in  the 
same  sense  as  one  speaks  of  a  sensory  aphasia,  or  a  visual  agnosia. 
Should  the  patient  recognize  the  object,  call  it  perhaps  by  name, 
state  its  use,  and  yet  in  attempting  to  use  it  totally  fail  in  proper 
motor  act,  it  is  termed  a  motor  apraxia;  it  being  understood  here 
that  there  is  no  necessary  change  in  the  motor  arc,  either  on  the  incom- 
ing sensory  or  outgoing  motor  side.  Thus  one  can  speak,  as  Wilson 
has  done,  of  a  motor  aphasic,  as  having  an  apraxia  of  his  speech  mus- 
culature. In  grave  intracerebral  changes  the  knowledge  of  the  proper 
kinetic  images  to  carry  out  purposeful  actions  in  the  arms  and  legs 
may  be  complexly  involved.  This  is  termed  an  intrapsychic  apraxia. 
Clinically  it  is  usually  overlaid  in  the  general  psychic  loss,  and  is  often 
included  in  the  loose  and  unprecise  term  dementia. 


Liepmaim 
(Reg.  rat.) 


Bychowsld 


Kroll 
.  V.  Stauffenberg    (Krykow) 


KroU 
(Iwanow) 


V.  Monakow 
(Vet..r) 


+  +  + 
V.  Bychowsk 


Fig.  363. — Localization  of  apraxia.  Positive  cases  from  lesion  in  the  left  supra- 
marginal  gyrus  and  (a,  b,  c,  d)  small  metastatic  tumors  from  one  case  of  total  bilateral 
apraxia.     In  this  case  foci  were  also  found  in  both  optic  thalami.      (von  Monakow.) 


Apraxia  may  be  then  either  sensory  or  motor;  it  may  be  unilateral 
or  bilateral;  it  may  be  extensive,  involving  many  muscular  groups 
or  may  be  limited  to  a  few,  such  as  an  inability  to  protrude  the  tongue 
on  demand  or  close  the  eyelids,  etc.,  with  perfect  power  in  other 
movements. 

Liepmann's  celebrated  case  was  able  to  do  things  wit.h  his  left  hand, 
but  failed  entirely  with  his  right.  When  told  to  brush  the  examiner's 
coat,  he  picked  up  a  corner  of  it  carefully  in  his  left  hand,  then  picked 
up  the  brush  in  his  right  hand,  with  which  he  made  movements  as  if 


APRAXIA 


655 


to  brush  his  hah.  Asked  to  pour  water  into  a  glass  from  a  carafe,  he 
grasped  the  carafe  with  his  left  hand,  to  pour  water  into  the  glass 
held  in  the  right  hand,  after  which  the  glass  was  brought  to  the  mouth 
without  any  water  in  it.  These  patients  fail  to  carry  out  the  simple 
commands  to  blow  a  kiss,  make  a  threatening  fist,  soldier's  salute,  etc. 


rtmann  III  ^_ 


Hartmann  I 


Monakow 
(Gloor.  b) 


Rhein 


Rhein 


Fig.  364. — Site  of  the  lesion  in  20  cases  of  apraxiii  marked  according  to  the  author's 
localization.  The  shaded  places  in  the  illustration  indicate  the  spreading  of  the  lesion 
in  certain  cases.  Sir,  corpora  striata;  VH,  anterior  horn  of  ventricle;  HH,  posterior 
horn,     (von  Monakow.) 


In  ideomotor  apra.xia  the  situation  is  more  complicated.  One 
patient  given  a  tooth-brush  recognized  it,  then  began  to  brush  his 
beard  with  it  clumsily;  another  being  given  a  pistol,  which  he  named 


656  DISEASES  OF   THE  BRAIN 

correctly,  on  being  told  to  shoot  it,  grasped  the  barrel,  blinked  and 
put  the  muzzle  into  his  left  eye.  Another  patient,  being  given  a  cigar 
and  a  match-box  opened  the  latter,  stuck  the  cigar  in  it,  and  tried  to 
shut  the  box  as  though  it  were  a  cigar  cutter.  Then  taking  the  cigar 
out  rubbed  it  on  the  side  of  the  box  as  though  it  were  a  match.  The 
entire  order  of  procedure  was  badly  devised. 

Apraxia  is  usually  an  accompaniment  of  certain  apoplectic  attacks. 
It  may  also  be  present  from  tumors.  The  lesions  center  about  the 
fibers  of  the  corpus  callosum  and  the  medullary  fiber  areas  from  the 
second  parietal  region. 

In  left  hemisphere  disease,  the  apraxia  may  be  homo-  or  hetero- 
lateral,  and  in  homolateral  apraxias  the  corpus  callosum  is  usually 
involved.  In  left  frontal  disease  apraxia  has  been  found  especially 
with  lesions  of  F.l  and  F.2.  Anything  that  brings  about  an  isolation, 
diaschisis,  of  the  left  frontal  area  from  the  right  frontal  area  will 
seem  to  bring  about  an  apraxia. 

Hemianopsia  is  an  infrequent  sign  occurring  as  a  result  of  apoplexy. 
As  a  symptom  of  middle  cerebral  disease  it  may  result  from  thalamic 
lesions  (pulvinar,  geniculates)  and  is  usually  a  permanent  residual. 
It  is  rarely  a  temporary  condition  but  may  show  marked  improve- 
ment. The  type  is  a  bilateral  homonymous  hemianopsia.  Quadrant 
hemianopsias  more  often  belong  to  the  middle  cerebral  syndromes, 
although  limited  lesions  of  the  cuneus  may  cause  quadrant 
hemianopsias.^ 

The  visual  fields  will  vary  greatly  according  to  the  tract  involve- 
ment. In  posterior  cerebral  syndromes  occipital  cortical  types  of 
hemianopsia  are  present.  These  show  very  irregular  visual  fields. 
Psychical  blindness  (loss  of  visual  memories)  may  also  result  from 
posterior  cerebral  involvements.  Here  the  portions  of  the  occipital 
cortex  about  the  calcarine  fissure  and  cuneus  are  implicated.  It  is 
usually  an  accompanying  symptom  of  the  apoplectic  attack  but  may 
be  isolated  and  often  combined  with  a  hemianopsia. 

Ayhasia  is  a  frequent  result  of  the  hemiplegic  attack  (right  hemi- 
plegia in  right-handed  persons;  left-sided  in  left-handed  persons). 

The  type  of  aphasia  depends  entirely  upon  the  location  of  the 
lesion  and  the  areas  served.  Transitory  aphasias  are  extremely 
common,  occurring  in  at  least  one-half  of  the  attacks.  Permanent 
aphasias  are  rarer,  about  one-half  of  the  patients  recovering.  The 
different  forms  of  aphasia  and  the  localization  significance  are  dis- 
cussed under  Disorders  of  Speech.     (See  page  357.)^ 

Papillary  edema,  even  choked  disk,  may  be  a  symptom  of  apoplexy, 
and  is  found  on  the  side  affected.  It  is  most  frequent  with  large 
lesions.^ 

1  Compare  Wilbrand  and  Saenger:  Die  Neurologie  des  Auges,  for  complete  discus- 
sion and  bibliography;  also  Henschen,  in  Lewandowsky's  Handbuch  der  Neurologie. 

2  Comp.  V.  Monakow's  Die  Lokalization  im  Grosshirn,  1914,  for  most  recent  discussion 
of  the  complicated  aphasia  question. 

^Uhthoff:     Neurol.  Centralbl.,   1909. 


DIAGNOSIS  OF  APOPLECTIC  SYNDROMES  657 

Diagnosis.- — In  the  matter  of  the  middle  cerebral  syndromes  chiefly, 
a  differentiation  of  the  apoplectic  attack  in  terms  of  causation — hemor- 
rhage, thrombosis  or  embolism — is  highly  desirable,  although  it  is 
not  always  possible.  The  end-results  may  be  identical,  but  the  ante- 
cedent conditions  vary.  Cerebral  hemorrhage  is  comparatively  rare 
in  the  young,  but  it  may  be  seen  at  all  decades,  and  even  in  intra- 
uterine life.  When  occurring  in  early  youth  it  is  usually  a  result  of 
parental  syphilis  or  of  some  acute  illness.  In  cases  of  cerebral  hemor- 
rhage the  patient  is  usually  over  forty,  shows  signs  of  arterial  disease, 
frequently  with  hypertrophied  heart,  and  often  has  nephritis.  The 
attack  is  usually  sudden,  often  preceded  by  emotional  disturbance 
or  sudden  change  in  position  that  modifies  the  blood-pressure.  The 
attack  is  more  likely  to  be  accompanied  by  unconsciousness  (75  per 
cent.).  Severe  ventricular  hemorrhages  show  blood  on  lumbar  punc- 
ture, but  this  procedure  is  rarely  called  for  save  in  supposed  traumatic 
cases. 

Thrombosis  occurs  also  in  older  individuals,  and  in  s^-philitics  par- 
ticularly. Unconsciousness  is  less  apt  to  occur,  or  develops  in  pro- 
gressive stages  as  it  were.  The  prodromata  already  mentioned  are 
more  apt  to  have  been  present.  Markedly  atheromatous  arteries 
speak  for  thrombosis,  and  mild  signs  of  deterioration — lacunar  syn- 
dromes— speak  for  thrombotic  t}'pes  of  disease. 

Embolism  is  almost  invariably  associated  with  some  acute  disease — 
typhoid,  acute  septic  infections  (articular  rheumatism,  gonorrhea, 
scarlet  fever,  malaria,  etc.).  Acute  endocarditis  is  often  present  and 
the  individuals  are  apt  to  be  young.  Unconsciousness  is  less  apt  to 
occur;  when  occurring  it  is  likely  to  develop  very  suddenly,  and  is 
more  often  present  in  basilar  and  carotid  occlusions  than  when  other 
arteries  are  blocked. 

Hemorrhages,  especially  when  small,  frequentlj-  show  the  maximum 
symptoms  early,  with  gradual  betterment,  while  thromboses  usually 
show  the  reverse,  the  symptoms  having  a  tendency  to  spread  or  to 
deepen. 

Ophthalmoscopic  examination  is  always  imperative.  Dilatation  of 
the  pupil,  usually  present,  can  be  obtained  by  cocain  in  a  few  minutes. 
Diffuse  retinitis,  so-called  retrobulbar  neuritis,  is  highly  indicative  of 
basal  syphilis  or  syphilitic  endarteritis.  In  hemorrhage  the  vessels 
of  the  disk  are  apt  to  be  engorged.  This  is  not  so  in  embolism  nor  in 
thrombosis.     Choked  disk  points  to  a  neoplasm. 

The  blood-pressure  affords  diagnostic  criteria.  It  is  apt  to  be  high 
in  hemorrhage  and  in  embolism,  but  low  in  thrombosis,  also  in  neo- 
plasms, syphilitic  endarteritis  and  cerebral  abscess. 

Other  disease  processes  to  be  distinguished  are  hysterical  hemiple- 
gias, syncope,  epileptic  attacks,  general  paresis,  uremic,  alcoholic,  or 
encephalitic  coma. 

Hysterical  hemiplegias  are  rarely  accompanied  by  unconsciousness. 
Hysterical  delirium  may  complicate  the  picture.     Later  the  signs  of 
pyramidal  tract  involvement,  such  as  increased  reflexes,  Babinski, 
42 


658  DISEASES  OF   THE  BRAIN 

Chaddock,  loss  of  abdominal  reflexes,  Grasset  and  Hoover  phenomena 
are  not  present  in  hysterical  hemiplegia.  In  certain  mild  thalamic 
cases  the  sensory  changes  may  be  thought  of  as  of  psychogenic  origin, 
but  careful  summing  up  of  the  results  outlined  on  pages  642  et  seq.  will 
show  the  somatic  signs  of  thalamic  involvement.  Personal  consulta- 
tion experience  has  shown  that  many  thalamic  cases  are  diagnosed  as 
psychogenic. 

Certain  lacunar  syndromes,  especially  in  the  anterior  cerebral 
distribution,  which  are  mild  and  which  present  momentary  confusion 
(worse  at  night),  emotional  instability,  irritability,  tendency  to 
weeping,  etc.,  are  frequently  mistaken  for  hysteria.  Suggestive  treat- 
ment of  such  a  case,  as  by  a  metronome  as  personally  observed,  is  not 
likely  to  succeed. 

Lacunar  syndromes  with  softenings  in  t.he  frontal  areas  are  also 
mistaken  for  manic  attacks.  There  is  confusion,  some  disorientation, 
excitement,  and  after  a  short  time  the  whole  thing  may  clear  up, 
leaving  only  slight  defect,  easily  overlooked,  unless  careful  intelligence 
tests  are  applied  (presbyophrenic  excitements,  presenile  excitements 
— see  Senile  and  Presenile  Psychoses.) 

Uremic  coma  is  usually  of  more  gradual  onset.  There  are  preceding 
signs  of  heaviness  and  toxemia;  convulsive  movements  are  not  infre- 
quent. But  signs  of  inequality  of  respiration  of  the  two  sides  of  the 
chest,  possibly  pupillary  inequalities,  possible  variations  in  response 
to  sensory  stimuli  on  the  two  sides,  involuntary  reflex  motor  responses 
to  joint  squeezing  and  finger  squeezing  (Marie-Foix)  are  usually  absent 
in  uremic  coma.  The  general  odor  of  the  patient  is  often  characteristic 
in  uremia.     Some  uremic  patients  develop  apoplectic  attacks  as  well. 

Diabetic  coma  shows  similar  difficulties.  Acetone  odor,  large 
amounts  of  sugar-containing  urine,  usually  slow  onset  and  the  previous 
history  must  be  relied  upon. 

Alcoholic  coma  is  frequently  diagnosed  by  the  police  as  apoplexy, 
since  many  alcoholics  do  have  apoplectic  attacks.  Some  severe 
intoxications  resemble  apoplectic  coma  very  closely,  but,  as  a  rule, 
the  coma  is  less  profound  in  alcoholism;  the  reflexes  are  often  pre- 
served, in  a  measure,  and  the  uniformity  in  the  bilaterality  of  the 
relaxation  is  evident  (absence  of  Marie-Foix  signs).  One  should  be 
on  one's  guard,  however,  and  look  for  all  of  the  little  signs  of  hemi- 
plegia.^ 

Epileptic  and  syncopal  attacks  rarely  present  great  difficulties.  The 
history  of  a  previous  attack,  the  scarred  body  or  head  and  tongue  of 
the  epileptic  is  often  evident,  while  in  syncope  the  coma  is  usually 
shallow  and  the  feeble  respirations  and  superficial  heart  action  point 
to  the  difficulty. 

An  apoplectiform  attack  may  be  the  first  sign  of  paresis.  Such 
attacks  are  usually  due  to  cerebral  edema,  and  may  be  at  first  indis- 
tinguishable from  an  apoplexy  (non-paretic) .     The  later  history  and 

'Dejerine:  Semiologie,  1914,  2d  ed.  Jelliffe:  Little  Signs  of  Hemiplegia,  Post- 
graduate, 1912. 


PROGNOSIS  OF  APOPLECTIC  SYNDROMES  659 

examination  will  usually  establish  a  diagnosis,  although  the  pseudo- 
paresis  of  arteriosclerotic  softening  is  often  only  distinguishable  from 
paresis  by  the  cytobiological  reactions.  (See  chapter  on  S\-philitic 
Diseases  of  the  Nervous  System.) 

Prognosis. — Recovery  from  the  attack  and  amelioration  of  the 
residual  symptoms  are  separate  problems. 

Cerebral  hemorrhage  is  usually  more  immediately  fatal  than  either 
thrombosis  or  embolism.  Deepening  coma,  Cheyne-Stokes'  respira- 
tion, irritative  phenomena,  jerking,  convulsions,  blood-pressure  (very 
high,  over  230  mm.  or  very  low,  under  90  mm.),  marked  rise  in  tem- 
perature are  the  usual  lethal  signs.  General  convulsions,  retinal 
hemorrhage,  blood  on  puncture,  bilateral  paralysis  are  unfavorable 
signs.  After  recovery  from  coma,  continued  temperatiu-e,  advancing 
symptoms,  restlessness,  delirium,  loss  of  sphincter  control,  trophic 
disturbances,  indicate  a  grave  prognosis  and  probable  death  in  from 
two  to  three  weeks. 

Early  attacks  of  lacunar  softening  are  rarely  fatal,  but  indicate 
that  a  fatal  termination  from  a  more  severe  t\'pe  of  attack  will  be 
probable  in  from  one  to  three  years.  Inasmuch  as  this  is  a  form  w^hich 
is  frequently  mistakenly  diagnosed  as  hysterical  in  the  early  mild 
attacks,  sometimes  as  a  mild  manic  attack  (excitement  and  confusion), 
one  should  be  on  one's  guard  in  this  not  infrequent  syndrome. 

The  recovery  from  the  residual  symptoms  will  vary  greatly  upon 
the  nature,  localization  and  extent  of  the  lesion.  A  careful  plotting 
of  the  entire  symptomatology  will  determine  the  area  involved  in  the 
destructive  process,  and  the  effects  of  diaschisis  must  first  be  separated 
out  from  tliose  of  actual  tissue  destruction,  since  the  former  are  more 
apt  to  disappear. 

Aphasias  are  usually  recovered  from,  almost  invariably  in  left- 
sided  hemiplegias  in  the  right-handed.  In  right-handed  hemiplegias 
aphasias  are  recovered  from  in  about  one-half  of  the  patients,  espe- 
cially in  the  intelligent  who  will  make  an  effort  to  reeducate  them- 
selves. If  the  lesion  lies  directh'  within  the  aphasic  area  the  chances 
for  recovery  are  less. 

Hemianopsias  are  not  infrequently  diaschitic.  When  so  they  pass 
within  a  few  weeks.  In  thalamic  lesions  they  are  apt  to  be  permanent, 
as  are  cortical  hemianopsias  persisting  over  a  few  weeks. 

Hemiplegia,  if  total,  is  apt  to  persist  in  some  degree  at  least,  although 
most  patients  are  able  to  get  about  in  three  months  or  more.  Help- 
lessness persisting  over  six  months  or  a  year  has  been  partially  recovered 
from.  Facial  palsies  usually  recover.  Leg  palsy  is  rarely  as  persistent 
as  arm  palsy,  but  both  react  favorably  to  appropriate  treatment.  Early 
contracture  usually  means  bad  therapy. 

Thalamic  involvement  is  a  bad  prognostic  feature — the  pain  and 
irregular  movements  (choreo-athetoid),  hemianesthesia,  etc.,  usually 
persist. 

Mental  defects  may  clear  up  almost  entirely,  especially  when  slight, 
but  careful  intelligence  tests  (see  Chapter  on  Mental  Examination) 


660  DISEASES  OF   THE  BRAIN 

should  be  utilized  in  all  cases,  especially  to  decide  medicolegal  prob- 
lems which  may  arise — testamentary  or  contract  capacity,  respon- 
sibility, etc.  A  sensory  or  motor  aphasia  alone  is  not  necessarily  a 
sign  of  an  intelligence  defect;  many  aphasics  are  very  intelligent. 
They  simply  cannot  utilize,  in  speech,  the  knowledge  they  have. 
(See  Chapter  on  Senile  and  Presenile  Mental  States.)  A  motor  aphasic 
who  cannot  get  his  symbols  over,  as  it  were,  may  not  be  any  more  of 
a  dement  than  an  Englishman  trying  to  make  a  Chinaman  understand 
what  he  is  saying. 

Treatment. — Prophylaxis  applies  particularly  to  those  over  fifty 
years  with  arteriosclerosis,  and  sustained  high  blood-pressure.  The 
tendency  to  hemorrhage  here  is  great.  Such  patients  should  slow  down 
somewhat  in  their  work,  if  strenuous  and  calling  for  intense  and  sus- 
tained effort,  and  especially  if  emotional  calls  are  frequent.  A  mod- 
erate amount  of  work  and  freedom  is  desirable.  Invaliding  a  pros- 
pective hemorrhagic  case  is  inadvisable.  A  partial  psychoanalysis  in 
intelligent  patients  who  have  not  crystallized  all  their  ideas,  to  show 
faulty  handling  of  the  unconscious  emotional  reactions  may  be  highly 
beneficial,  especially  in  hypertensive  states. 

Dietary  faddism  is  to  be  avoided.  Excessive  eating  is  to  be  stopped 
and  all  alcohol  should  be  restricted.  Protein-free  diet — vegetable 
proteins  are  the  same  as  animal — keeps  down  the  blood-pressure  in 
many  cases.  Some  search  should  be  made  to  see  if  specific  protein 
sensitization  exists. 

Careful  regulation  of  the  gastro-intestinal  tract  is  called  for,  the 
kidney  functions  should  be  scrutinized,  and  the  liver  metabolism 
regulated. 

It  is  doubtful  if  drug  therapy  is  of  any  service  in  prophylaxis.  The 
iodides  have  been  used  widely,  but  their  utility  is  still  undecided. 

Treatment  of  the  attack,  even  if  there  is  no  unconsciousness,  requires 
immediate  rest  in  bed  if  possible.  If  striken  away  from  home  the 
patient  should  be  moved  as  little  as  possible.  With  high  tension,  hot 
foot-packs  will  help  to  reduce  it  (not  hot  enough  to  burn  the  uncon- 
scious patient).  In  cases  in  which  the  blood-pressure  rises  steadily, 
keeps  above  250  mm.  and  with  very  deep  coma,  blood-letting  (10  to 
12  oz.)  is  advisable. 

Hypodermic  medication  by  blood-pressure  reducing  drugs  in  hemor- 
rhage is  alone  advisable  in  coma,  and  only  very  small  quantities  of 
water  should  be  used,  as  water  raises  blood-pressure.  Hydrochlorate 
of  gelsemine  in  doses  of  tV  grain  is  fairly  active  and  reliable.  The 
nitrites  are  not  available. 

If  the  patient  can  swallow,  tincture  of  aconite  in  lUv  doses  may  be 
given,  watching  the  blood-pressure.  The  dose  may  be  repeated  in  an 
hour.  Pressure  should  be  kept  below  200  mm.  if  possible.  Tincture 
of  veratrum  viride  in  TUv-xv,  every  two  hours,  or  the  fl.  ext.  of  gelse- 
mium  in  same  doses  at  similar  intervals. 

Pressure-reducing  drugs  should  be  used  with  caution.  The  high 
pressure  following  hemorrhage  is  usually  compensatory  and  for  the 


TREATMENT  OF  APOPLECTIC  SYNDROMES  661 

purpose  of  keeping  up  an  effective  A'ascular  irrigation  of  the  medullary 
nuclei  following  a  rupture  in  the  arterial  pipe-line.  These  drugs  should 
be  used  only  when  it  is  known  that  a  high  pressure  preceded  the  attack. 

Early  purgation  is  desirable;  2  gtt.  of  croton  oil  is  useful  in  states 
of  deep  coma.  This  may  be  placed  with  butter  on  the  back  of  the 
tongue. 

If  there  is  marked  excitement,  chloral,  gr.  y-xv,  or  paraldehyde, 
oj-ij,  may  be  given  by  mouth  or  by  rectum.  Tepid  sponge  baths 
help  restlessness.  Veronal  and  trional  are  useful  in  the  restlessness 
of  lacunar  softenings. 

Care  of  the  mouth,  drooling,  moving  the  patient,  removal  of  all 
obstructions  to  breathing,  should  be  attended  to  at  once. 

Nourishment  may  be  omitted  in  the  early  stages.  A  purin-free 
diet,  mostly  milk  and  gruels,  to  which  sugar  and  eggs  may  be  added 
later,  should  constitute  the  standard  diet.  In  the  presence  of  diffi- 
culty in  swallowing,  milk  enemas  are  to  be  given. 

In  cardiac  atonic  cases,  with  low  blood-pressure — chiefly  throiiiboses 
— vasodilators,  camphor  and  adrenalin  may  be  necessary. 

Surgical  treatment  may  be  advisable  for  meningeal  or  subcortical 
hemorrhages.  Certain  nephritic  cases  are  helped  by  lumbar  puncture, 
and  the  withdrawal  of  20  to  50  c.c.  of  fluid.  The  patients  should  be 
kept  quiet  several  weeks,  attention  being  paid  to  giving  ease  and  com- 
fort by  cushions,  props,  supports,  and  frequent  changes  of  position. 

Late  Treatment  of  Hemiplegia.^ — The  recent  investigations  on  the 
functions  of  the  p^Tamidal  tracts  (von  Monakow%  Sherrington,  Franz) 
make  the  outlook  for  the  reeducational  treatment  of  hemiplegia  much 
more  hopeful.  It  would  seem,  as  a  result  of  this  work,  that  the  p\Tami- 
dal  tracts  are  not  the  exclusive  carriers  of  volitional  motor  control. 
Destruction  of  the  motor  cortex  of  the  cat,  dog  or  monkey  causes 
only  about  two-thirds  of  the  pjTamidal  tract  to  degenerate.  The 
indications  are  that  there  is  a  large  extrarolandic  motor  area,  probably 
in  the  frontal  and  parietal  lobes,  particularly  for  the  face  and  upper 
extremities.  In  addition  to  these  facts  are  the  further  ones  brought 
out  by  stimulation  of  the  motor  cortex,  viz.,  the  so-called  motor  centers 
do  not  correspond  in  extent  in  different  animals  of  the  same  species; 
they  are  not  of  the  same  extent  on  the  two  sides  in  the  same  animal; 
repeated  stimulation  does  not  always  produce  the  same  movements, 
sometimes  there  are  added  movements  and  sometimes  the  movements 
are  the  exact  opposite  as  extension  where  there  was  formerly  flexion. 
All  this  indicates  that  the  motor  cortex  is  by  no  means  as  fixed  in  the 
relation  of  its  cells  to  a  definite  muscular  function  as  has  heretofore 
been  supposed.  On  the  contrary,  it  would  seem  that  it  had  retained  a 
considerable  fluidity,  a  large  capacity  for  adjustment  so  that  the 
movement  resulting  from  stimulation  of  any  particular  part  was 
dependent  upon  the  functional  set  of  the  associated  neurons  at  the 
moment  of  stimulation.  If  this  is  true  it  can  be  seen  how  systematic 
motor  reeducation  may  serve  to  facilitate  discharge  through  paths 
not  heretofore  consciously  used  both  by  bringing  into  use  associated 


662  DISEASES  OF  THE  BRAIN 

cortical  motor  cells  and  pathways  nearby  and  also  by  drawing  upon 
the  extrarolandic  regions. 

The  application  of  the  principles  elucidated  have  in  fact  been  pro- 
ductive of  most  excellent  results  in  the  treatment  of  hemiplegias.  A 
most  detailed  scheme  of  movements  calculated  to  overcome  the  motor 
defects  should  be  mapped  out  and  persisted  in  daily.  This  plan  should 
be  begun  a  few  days  after  the  injury,  practically  as  soon  as  the  patient's 
mental  state  will  permit  of  cooperation.  It  should,  at  first,  be  limited 
to  a  few  minutes  and  later  prolonged  with  care  to  avoid  excessive 
fatigue.  The  nurse  can  finally  learn  what  is  to  be  done  and  exercise 
the  patient  perhaps  an  hour  daily,  adding  diversions  to  the  daily 
routine  that  include  a  utilization  of  the  newly  acquired  motions. 

Such  exercises  must  get  away  from  the  hackneyed  and  stupid  calis- 
thenics, etc.,  which  have  prevailed  in  the  past.  The  psyche  of  the 
patient  must  be  put  into  his  work  and  the  exercises  worked  out  along 
proper  kinetic  lines  (Mensendieck  exercises  and  the  like)  employed. 

In  certain  cerebral  hemorrhage  diplegias  the  contractures  may  be 
helped  by  the  Foerster  operation  or  some  modification  of  it.  (See 
Little's  disease.) 

Electricity  is  of  doubtful  service.  Galvanism  aids  some  of  the  pains, 
the  anode  should  be  over  the  painful  area. 

The  general  care  of  the  invalid  will  depend  largely  upon  his  economic 
status.  Travel  is  helpful  in  supplying  a  mental  stimulus  and  the 
warmer  climates  and  more  interesting  foreign  resorts  are  enjoyable, 
and  thus  of  direct  therapeutic  value.  The  proper  exercises  must  be 
kept  up,  however.  Travel  alone  has  no  particular  benefit.  Varied 
occupations  suited  to  the  individual's  temperament  and  habits  should 
be  sought  for,  wherein  resourcefulness  is  a  great  asset. 

Speech  training  for  aphasia  may  accomplish  much.  Its  details, 
as  well  as  many  others,  cannot  be  entered  into  here.^ 

SINUS   THROMBOSIS. 

There  are  three  main  varieties  of  sinus  thrombosis,  the  so-called 
marasmic  variety,  occurring  in  debilitated  individuals,  and  as  a  result 
of  cardiac  weakness,  cachexia,  chlorosis,  etc.  Thrombosis  of  a  sinus 
may  also  be  dependent  on  injury  as  a  result  of  fracture  of  the  skull  or 
injury  to  the  skull  during  difficult  labor.  The  most  important  form 
of  sinus  thrombosis,  how^ever,  is  that  dependent  upon  infection,  and 
the  most  frequent  varieties  are  sigmoid  thrombosis,  from  mastoid 
disease,  and  lateral  thrombosis.  Primary  thrombosis  of  the  jugular 
bulb  sometimes  follows  middle  ear  infections. ^ 

Symptoms. — The  general  symptoms  of  sinus  thrombosis  are,  in 
accordance  with  the  above,  those  of  infection,  namely,  chills  and  fever, 

1  Consult  White  and  Jelliffe:  Modern  Treatment  of  Nervous  and  Mental  Disease, 
vol.  ii,  for  detailed  discussion  of  all  of  the  features  of  treatment  in  chapters  by  F.  Tilney 
and  S.  A.  K.  Wilson. 

2Braun,  A.:  Diagnosis  of  Sinus  Thrombosis,  New  York  Med.  Jour.,  December  21, 
1918. 


SINUS  THROMBOSIS 


663 


and  with  the  breaking  down  and  liquefication  of  the  thrombus  and 
its  distribution  in  the  general  circulation  there  may  be  pyemia,  with 
abscess  formation.     These  abscesses  may  occur  in  the  brain,  cerebrum 


Fig.  365. — Schema  showing  venous  sinuses  of  the  head.     (After  Macewen.) 


and  cerebellum,  or  the  infection  may  extend  directly  from  the  sinus 
and  produce  a  local  or  a  general  meningitis. 

The  local  symptoms  vary  with  the  sinus  involved.     In  thrombosis 
of  the  lateral  sinus,  dependent  upon  middle-ear  disease,  there  is  usually 


664  DISEASES  OF   THE  BRAIN 

a  venous  congestion  and  edema  over  the  mastoid  process,  and  the 
thrombus  may  extend  into  the  jugular  vein  and  be  palpable  (see  p. 
663). 

The  chief  symptoms  in  lateral  sinus  disease  are:  (1)  Pain,  at  times 
merged  in  the  mastoid  pain.  In  certain  cases  a  marked  euphoria, 
from  temperature,  masks  the  pain  and  may  even  be  diagnostic  of  a 
thrombosis.  (2)  Optic  hyperemia  is  not  infrequent.  (3)  Sixth  nerve 
paralysis  occasionally.  (4)  Perijugulitis,  with  pain  on  moving  the 
head  or  in  swallowing.  (5)  Glossopharjaigeal  implication  with  loss 
of  power  in  swallowing  from  palatal  paresis.  (6)  Vagus  implication 
rarely  as  seen  in  hoarseness  and  bradycardia.  (7)  Spinal  accessory 
irritation  or  paralysis.  These  are  the  chief  results  of  the  inflam- 
matory process  in  the  sinus  wall.  The  chief  signs  of  interference 
wuth  the  venous  circulation  are:  (1)  Griesinger's  sign,  painful  swelling 
at  the  middle  of  the  posterior  margin  of  the  mastoid;  (2)  Painful 
swelling  in  the  posterior  triangle  of  the  neck;  (3)  Gerhardt's  sign,  an 
unequal  fulness  of  the  jugular  veins,  not  vers-  constant. 

Cavernous  sirrns  thrombosis,  owing  to  the  drainage  of  the  ophthalmic 
vein  backward  into  this  sinus,  produces  quite  characteristically  marked 
exophthalmos,  venous  congestion  and  edema  of  the  lids,  chemosis; 
these  are  often  mistaken  for  orbital  disease  and  are  fairly  constant. 
To  these  may  be  added  choked  disk,  infrequently,  together  with 
retinal  hemorrhages,  oculomotor  palsies,  dilated  immovable  pupils, 
involvement  of  the  fifth  nerve,  with  hyperesthesia  in  its  area  of  dis- 
tribution. Involvement  of  this  sinus  rarely  remains  unilateral  but 
soon  becomes  bilateral  by  spread  of  the  affection  by  way  of  the  circular 
sinus. 

In  hiigitudinal  sinus  thrombosis,  distended  fontanelles  (in  children), 
nosebleed,  caput  medusee,  and  swelling  of  the  frontal  and  parietal 
veins  are  the  chief  local  symptoms. 

Treatment. — ^The  treatment  of  these  conditions  is  essentially  surgi- 
cal. Septic  involvement  of  the  cavernous  sinus  is  generally  fatal. 
The  condition  is  usually  inaccessible  to  surgical  interference. 

Thrombosis  of  the  lateral  sinus  is  not  an  infrequent  complication 
of  the  middle-ear  disease,  and  if  seen  early,  is  susceptible  of  successful 
operation.     The  sinus  may  be  opened  and  the  clot  cleared  out. 

The  other  sinuses  of  the  dura  may  of  course  become  involved,  but 
generally  secondarily,  and  do  not  present  such  characteristic  clinical 
pictures. 


CHAPTER  XIII. 
TUMORS  OF  THE  BRAIN. 

TiTMORS  of  the  brain  are  relatively  infrequent.  They  occur  at  all 
ages,  are  found  in  ever\-  conceivable  location  within  the  cranial  cavity, 
and  are  of  a  greatly  varied  pathology.  Extensive  monographs  have 
been  published,  and  no  feature  of  disease  of  the  nervous  system  has 
attracted  more  attention,  not  only  by  reason  of  its  practical  impor- 
tance, but  also  because  of  the  contribution  to  cerebral  localization 
and  function.  The  chief  literature  to  1919  may  be  foimd  in  Starr,^ 
von  Monakow,^  Oppenheim,^  Tooth^  and  Redlich.^ 

In  18,000  cases  of  nervous  disease  occurring  in  ten  years  at  the 
Vanderbilt  Clinic  there  were  48  brain  tumors.  This  is  a  minimum 
computation.^  Bruns  states  it  as  high  as  2  per  cent.,  Cushing  as 
0.75  per  cent.,  Redlich,  from  4000  cases  of  nervous  disease,  the  same. 

Etiology. — ^The  causes  for  certain  tumors,  such  as  tuberculoma, 
syphiloma,  actinomycoses,  are  well  known.  For  both  s\'philitic  and 
tuberculous  tumors,  and  possibly  other  tumors,  traumatism  may  be 
an  additional  element  for  their  special  localization.  Metastatic 
tumors  follow  from  their  prim  an,'  sources.  Certain  teratomata, 
dermoids,  angiomata  are  congenital  conditions,  while  cholesteatomata, 
chordoma,  chondroma,  lipoma  and  myxoma  are  also  developmental 
anomalies. 

The  traumatic  genesis  of  tumors  in  general,  or  of  any  one  t^'pe  in 
particular,  apart  from  aneurisms,  is  highly  problematical,  yet  if  the 
trauma  and  symptom  development  are  related  in  specific  and  very 
definite  ways  the  causative  role  may  be  debated.  Thus,  if  the  injury 
is  sufficiently  intense  to  definitely  injure  the  skull,  the  time  interval 
between  the  accident  and  the  development  of  the  symptoms  not  too 
great,  and  the  localization  of  the  probable  tumor  near  to  the  site  of 
the  injury-,  the  relationship  may  justifiably  be  open  to  serious  con- 
sideration. 

Varieties. — The  chief  forms  met  with  may  be  classified  as:  (1)  True 
tumors,  (2)  infectious  tumors,  (3)  parasitic  cysts,  (4)  aneurisms,  (5) 
vascular  cysts. 

1.  True  Tumors. — Of  these  gliomata  are  the  most  frequent.  They 
preponderate  over  any  other  class  in  adults.     In  Tooth's  summary-  of 

*  Text-book  of  Nervous  Diseases,  4th  ed.,  Brain  Surgery. 

*  Gehirnpathologie,  2d  ed.,  Die  Lokalisation  im  Grosshim,  1914. 

'  Die  Geschwiilste  des  Gehims,  2d  ed.  ♦  Brain,  1912,  xxxv,  61. 

'  Handbuch  der  Neurologie,  Lewandowsky,  1912,  vol.  iii.  Also  consult  Current  Lit- 
erature in  Journal  of  Nervous  and  Mental  Disease. 

•See  Report  of  Clinic  of  Prof.  M.  Allen  Starr,  1900-1909,  by  Jelliffe  and  Brill. 

(665) 


666 


TUMORS  OF   THE  BRAIN 


500  cases,  49  per  cent,  were  gliomata.  Children  rarely  come  to 
the  National  Hospital  in  London,  hence  Tooth's  low  percentage  of 
tuberculous  tumors,  Gliomata  occur  throughout  the  brain  as  circum- 
scribed or  as  diffuse  tumors,  and  are  extra-  as  well  as  intracerebral. 
The  general  tendency  is  toward  diftuseness,  and  degeneration  with 
hemorrhage  and  fatty  and  cystic  formations  take  place  principally  in 
intracerebral  growths.  Thus  the  symptoms  are  apt  to  develop  slowly 
and  intermittently  in  cortex  or  basal  ganglia  tumors,  occasionally 
from  those  of  the  ependjuia.  The  tumors  may  invade  an  entire 
hemisphere. 

Sarcomata  are  less  frequent,  restricting  the  term  sarcoma  more 
strictly  than  is  usual,  and  rejecting  the  compromise  gliosarcoma. 
Nearly  all  gliomata  show  elements  indistinguishable  from  sarcoma. 
They  usually  develop  slowly  in  the  brain. 

Chloromata  are  leukemic  in  origin  and  are  rare.  They  involve  the 
periosteum  or  the  base  and  thus  cause  compression  phenomena  which 
often  persist  for  some  time. 


Fig.  366. — -Infiltrating  glioma  of  basal  ganglia. 


Fibromata  (neurofibromata)  are  comparatively  common  brain 
tumors  (10  per  cent,  in  Tooth's  collection,  including  fibroglioma). 
They  develop  chiefly  about  the  cerebellopontine  angle  (acoustic)  but 
may  develop  along  other  cranial  nerves.  Occasionally  they  are 
multiple.     They  develop  slowly.^ 

Endotheliomata  are  comparatively  frequent  (14  per  cent,  in  Tooth's 
collection).  They  seem  to  confine  themselves  chiefly  to  the  anterior 
fossae.  They  are  usually  small  and  multiple,  develop  slowly  and 
chiefly  in  the  falx  region .^ 

Chordomata  are  infrequent,  and  only  rarely  reach  a  considerable  size.^ 

1  Gushing:     Tumors  of  the  Nervus  Acusticus,  1918. 

'Haeger:     Berlin,  1911. 

'  Jelliffe  and  Larkin:     Jour.  Nerv.  and  Ment.  Dis.,  .January,  1912. 


TUMORS  OF   THE  BRAIN 


667 


Carcinomata  (5.8  per  cent. — Tooth),  closely  related  to  the  endo- 
theliomata  are  usually  secondary  (metastatic),  rarely  primary. ^ 

Psammomata,^  lipomata,  enchondromata,  angiomata,  osteomata, 
adenomata,  cysts,^  cholesteomata,  hypophyseal  tumors,  teratomata 
(pineal),^  and  dermoids  are  among  the  rarities. 

2.  Infectious  Tumors. — These  are  tuberculous,  s^-philitic,  actino- 
mycotic. 

Tuherculoviata  are  possibly  the  commonest  of  all  tumors,  certainly 
in  children  (Starr,  193  in  600  tumors  recorded).  They  are  extremely 
rare  after  forty  years.  They  are  frequently  conglomerate  or  multiple 
in  type,  hence  giving  rise  to  mixed  syndromes.    There  may  be  a  few 


Fig.  367. — Chordoma  of  base.     (Jelliffe  and  Larkin.) 

very  small  miliary  tubercles  or  a  large  broken-down  tubercle  mass,  with 
every  conceivable  intermediary  stage.  They  develop  chiefly  in  the 
cerebellum,  peduncle,  basal  ganglia,  pons  and  cortex.  In  Zappert's 
group  of  89,  37  were  in  the  cerebellum,  29  in  the  cerebral  cortex,  13 
in  the  basal  ganglia,  5  in  the  pons.     They  have  a  bad  prognosis. 

Syphilomata.- — Gummata  are  not  infrequent.^  They  are  practically 
limite<l  to  adults  and  are  rarely  recorded  from  congenital  syphilis. 
Patients  with  congenital  s\philis  may  develop  symi)toms  of  tumor 


1  Levin:     Metastasis  of  Cancer  in  the  Central  Nervous    System,  Jour.   Nerv.  and 
Ment.  Dis.,  June,  1917. 

2  Roussy:     Aroh.  g6n.  de  M6d.,  ii,  82. 

3  Meyer:     Berlin,  1913. 

<  Bailey  and  Jelliffe:     Tumors  of  the  Pineal  Body,  Arch.  Int.  Med.,  December,  1912. 
'  Tooth:     Gumma  Cerebri,  Tr.  Royal  Soc,  April  17,  1913. 


668 


TUMORS  OF  THE  BRAIN 


pressure.  They  may  appeal  from  one  year  to  thirty  years  after 
infection.  They  occur  chiefly  as  flat,  infiltrating,  irregular  masses — 
less  often  as  definite  nodular  masses,  chiefly  at  the  base  of  the  brain. 
They  are  discussed  more  fully  in  the  chapter  on  Syphilis  of  the  Nervous 
System  {q.  v.). 

Actinomycosis  of  the  brain  is  a  rarity. 

3.  Parasitic  Cystic  Tumors. — Cysticercvs  of  the  brain  is  a  rarity  and 
is  secondary.^ 

4.  Aneurismal  Tumors. — Aneurisms  are  very  frequent  in  cerebral 
vessels.  They  are  mostly  small,  but  large  aneurisms  occur  at  times 
and  give  s^Tnptoms  of  pressure.  They  occur  in  patients  usually  from 
forty  to  seventy  years  old,  and  are  mostly  of  the  basilar.  They  cause 
pressure  symptoms  at  times,  with  obstructive  sjTuptoms — basilar  syn- 
drome— or  they  rupture  and  produce  symptoms  of  cerebral  hemor- 
rhage.2 


Fig.  368. — Gumma  of  brain. 

Symptoms. — Brain  tumors,  even  of  a  large  size,  may  be  found  at 
autopsy,  and  yet  not  have  given  rise  to  any  recognized  symptoms. 
Tuberculomata  are  thus  frequently  found  in  children.  With  more 
precise  investigation  such  latent  tumors  are  becoming  rarer,  especially 
since  the  importance  of  mental  symptoms — psychoses,  so-called  hys- 
terias, etc. — unaccompanied  by  sensorimotor  syndromes,  is  becoming 
recognized.  Many  small  tumors,  especially  osteomata,  psammomata, 
slowly  developing  and  circumscribed  gliomata,  cholesteatomata  may 
cause  very  few  symptoms.  Occasionally  a  tumor  will  show  mono- 
symptomatically,  as  by  epileptic  convulsions,  mili  speech  disturbances, 


iLewy:  Beitrag  zur  Kenntnis  der  zerebrospinal  Zystizerkose,  Breslau,  1913.  Lloyd: 
Philadelphia  Med.  Jour.,  March  19,  1898. 

2  Beadles:  Brain,  1907,  p.  285.  Reinhardt:  Ueber  Hirnarterienaneurysmen  und 
ihre  Folgen,  Mitt.  a.  d.  Grenzg.,  d.  Med.  u.  Ch.,  1913,  xxvi.  Booth:  Jour.  Nerv.  and 
Ment.  Dis.,  September,  1909. 


GENERAL  SYMPTOMS 


669 


mild  sensory  defects,  optic,  olfactory,  auditory  hallucinations  or 
hemianopsia,  without  being  recognized. 

The  s^Tuptoms  are  best  considered  as  (1)  general  and  (2)  local  or 
focal. 

The  general  symptoms  are  indicative  of  the  effects  of  the  tumor 
as  a  whole,  irrespective  of  its  special  nature  or  localization.  They  are 
due  in  general  to  the  effects  of  increased  intracranial  pressure,  which 
in  certain  tumors,  notably  of  the  posterior  fossa  and  cerebellum,  appears 
early  and  is  usually  marked  even  with  small  tumors,  whereas  tumors 
elsewhere  often  may  show  little  of  such  pressure  symptoms.  Sometimes 
the  focal  s^Taptoms  appear  before  the  general  ones.    General  s;vTaptoms 


Fig.  369. — Aneurism  of  basilar  artery.     (Larkin.) 

rarely  have  any  localizing  diagnostic  value.  Some  of  the  general  symp- 
toms of  pressure,  cranial  nerve  palsies  for  example,  may  even  tend  to 
mislead  one  as  to  a  localizing  diagnosis.  Furthermore,  in  cerebral 
tumors,  acute  swellings,  not  identical  with  but  related  to  edemas, 
often  give  rise  to  very  anomalous  symptoms  and  tend  to  obscure  the 
diagnosis  and  render  it  uncertain.^ 

The  chief  symptoms  of  general  value  are  headache,  nausea,  vomiting, 
dizziness,  respiratory  and  cardiac  disturbances,  metabolic  changes, 
mental  signs  of  sleeplessness,  sometimes  drowsiness,  optic  nerve 
changes,  and  convulsive  phenomena.    These  general  symptoms  have 


iSpiller:     Jour.  Nerv.  and  Ment.  Dis.,  1914,  xli,  184.     Miiller,  E.:  D.  Z.  f.  N.,  xlvii, 
48.     Marburg;     Wien,  med,  Wchnschr.,  1911,  No.  40, 


670  TUMORS  OF  THE  BRAIN 

a  tendency  to  be  progressive,  but  may  vary  considerably  in  their 
intensity  from  time  to  time,  especially  in  syphilomata,  tuberculomata, 
and  gliomata.  At  times  they  remain  stationary,  again  they  may 
regress  and  disappear. 

Headache.- — This  is  frequent.  Most  patients  will  have  headache, 
especially  if  the  tumor  is  of  protracted  growth.  Headache  is  an  early 
sign.  Practically  every  patient  with  a  headache  should  be  questioned 
and  examined  for  the  possibility  of  its  being  caused  by  a  cerebral 
tumor.  The  headache  usually  starts  more  or  less  irregularly,  is  fre- 
quently intermittent  in  the  early  stages  and  then  becomes  persistent, 
being  dull  or  severe;  if  dull,  with  periods  of  excruciating  exacerbation. 
Diurnal  variations  may  be  noted,  and  emotional  excitement  or  general 
causes  for  increasing  intracranial  pressure  increase  it,  often  with  addi- 
tional symptoms,  such  as  vertigo,  vomiting  or  even  agitated  or  coma- 
tose confusions.  Aneurismal  tumors  may  reveal  pulsation  by  auscul- 
tation. Migraine-like  headaches  are  very  frequent  in  early  states,  par- 
ticularly in  basal  cases,  pituitary,  basal  gummata,  neurofibromata, 
or  there  may  be  migraine-like  exacerbations  on  a  dull,  heavy,  gray 
background  of  pain.  Children  usually  respond  to  such  variations  by 
attacks  of  screaming,  pulling  the  hair,  or  beating  the  head. 

Later,  somewhat  mentally  dulled  patients  may  even  deny  any 
headache,  or  even  forget  having  had  a  period  of  great  distress. 

The  headache  of  cerebral  tumor  is  mostly  diffuse,  but  it  may  be 
localized,  in  which  case  it  may  serve  to  indicate  the  general  site  of  the 
tumor.  Such  is  rendered  more  probable  if  further  substantiated  by 
percussion  tenderness,  a  highly  important  procedure,  percussion  dul- 
ness,  and  x-ray  shadow.  The  site  of  a  headache  is  a  very  uncertain 
guide  for  localization  purposes,  however.  Frontal  tumors  often  give 
rise  to  occipital  headaches  and  vice  versa;  right-sided  tumors  to  left- 
sided  pains  and  vice  versa.  The  general  drift  for  localized  pain  is,  how- 
ever, in  favor  of  a  similarly  located  tumor.  Among  head  symptoms 
may  be  mentioned  the  occasionally  found  auscultation  notes  of  aneu- 
rismal tumors;  the  presence  of  enlarged  head  (hydrocephalus),  and  the 
overfilling  and  increased  tortuosity  of  the  veins  on  the  forehead,  face, 
conjunctiva,  etc.,  of  the  affected  side. 

Trigemmal  neuralgic  attacks  may  be  general  or  at  times  a  focal 
(cerebellopontine  angle)  sign. 

Nausea,  vomiting,  and  dizziness  are  frequent  in  late  stages  of  a  cere- 
bral tumor,  and  more  often  found  in  children  and  in  those  patients 
with  rapidly  increasing  signs  of  intracranial  pressure — posterior  fossa 
tumors  particularly.  Such  vomiting  may  occur  spontaneously — pro- 
jectile in  type — or  as  an  accompaniment  of  the  headache  crises,  espe- 
cially when  migrainous  in  type.  Vomiting  often  is  absent  entirely 
even  with  large  tumors.  When  present  it  more  often  occurs  in  the 
morning,  and  at  times  it  is  so  persistent  as  to  lead  to  inanition,  exhaus- 
tion, and  death. 

In  cerebellar,  peduncle,  pontme,  and  medullary  tumors,  and  those 
causing  pressure  on  the  superior  cerebellar  peduncle,  or  its  incoming 


GENERAL  SYMPTOMS  071 

pathways,  the  nausea  and  vomiting  may  be  accompanied  by  unilateral 
vertigoes  or  with  tendencies  to  turn  or  to  fall  in  one  direction.  Here 
general  and  focal  symptoms  coincide.  Frontal  tumors  may  occasion- 
ally cause  such  one-sided  vertigoes  from  implication  of  the  frontal 
extensions  of  the  cerebellar  pathways.  Vertigoes  from  ocular  palsies 
are  localizing  s\Tnptoms  (quadrigeminal  SAndromes — occasionally 
cortical) . 

Cardiac  and  Respiratory  Signs. — Slowness  of  the  pulse,  at  times 
marked — 30  to  40 — is  a  general  sign  of  intracranial  pressure,  and  more 
especially  in  marked  grades.  Hence  it  is  apt  to  be  a  late  rather  than 
an  early  s\Tiiptom,  unless  one  of  direct  irritation  of  the  vagus  (medullary 
pressure).  The  bradycardia,  at  times  arrhythmia,  may  appear  period- 
ically during  headache  exacerbations,  or  at  times  independent  of  the 
same  (acute  swelling  reaction). 

^Medullars'  tumors  cause  respiratory  changes,  at  times  slowness, 
again  irregularity,  and  Che^Tle-Stokes — with  acute  pressure  SNTiiptoms 
(hydrocephalus  internus).  Hiccough,  yawTimg,  and  related  respiratory 
signs  are  occasionally  present. 

Metabolic  Didurhances. — These  are  irregular  in  their  development 
and  evolution.  Fever  is  infrequent  save  as  a  complication  of  the 
late  stages. 

Cachexia  and  marasmus  are  present  with  certain  carcinomata. 
Marked  adiposity,  ovarian  and  in  particular  testicular  aplasias,  are 
frequent  m  certain  hj^jophyseal  (pituitary)  tumors  or  those  causing 
internal  hydrocephalus  by  possible  implication  of  the  infundibular 
region  through  general  pressure  (pineal  and  corpora  quadrigemina 
tumors).  Acromegaly  is  a  special  case,  as  are  also  the  dystrophia 
adiposogenitalis  s\iidromes  which  are  discussed  in  the  chapter  on  the 
Disorders  of  the  Endocrinous  Glands. 

Mental  Signs. — These  are  of  great  value,  both  general  and  localizing, 
in  from  60  to  So  per  cent,  of  the  cases.^  They  var^-  considerably, 
and  are  particularly  prominent  late  in  the  disease,  although  here 
masked  under  the  general  symptoms  of  apathy,  confusion  or  coma. 
Tumors  of  any  region,  large  and  small,  and  independently  of  their 
pathological  nature,  may  cause  psychical  changes.  Certam  localities 
cause  special  psychical  alterations  to  be  discussed  under  focal  and 
localizing  symptoms. 

In  the  early  stages,  slight  impairment  of  attention,  with  slowTiess 
and  difficulty  in  grasp,  retardation  in  motor  response,  and  a  confusion 
or  bewilderment  may  be  present.  Ready  forgetting,  slight  esthetic 
lapses,  and  moral  breaks — with  the  telling  of  shady  stories,  showing 
of  bad  taste,  exhibitionist ic  fancies,  even  gross  lapses — such  as  open 
masturbation,  etc. — occur.  These  are  the  precursors  of  a  more  marked 
grade  of  retardation  of  mental  function,  leading  to  apathy,  listlessness, 
lack  of  initiative,  at  time^  with  confusional  episodes — getting  lost — 
fugues,  fussiness,  emotionalism,  etc.     In  marked  states  of  confusion 

>  Gianelli,  Schuster  and  others:  See  Bibliography  in  Redlich,  Hirntumor,  Lewandow- 
sky's  Handbuch  der  Neurologie. 


672  TUMORS  OF  THE  BRAIN 

and  disorientation  a   typical   Korsakov's  syndrome    (g.  v.),  without 
polyneuritis,  may  be  present. 

Later  stages  show  typical  pictures,  not  distinguishable  from  those 
of  arteriosclerotic  deterioration,  or  paresis — i.  e.,  so  far  as  the  purely 
mental  picture  is  concerned. 

Certain  less  general  symptoms  often  present  themselves  and  are 
of  a  certain  localizing  value.  Hallucinations  of  smell  speak  for  olfac- 
tory lobe  or  olfactory  tract  involvement.  Those  of  sight  for  occipital 
cortex  localization,  those  of  sound  for  temporal  lobe  trouble. 

The  tendency  to  joke,  be  facetious,  show  manic  idea  associations, 
even  flight  of  ideas  with  euphoria,  is  at  times  present.  This  is  more 
often  found  in  prefrontal  tumors,  especially  left-sided,  but  may  occur  in 
tumors  of  other  regions,  usually,  however,  all  reaching  to  and  involv- 
ing the  cortex.  These  symptoms  are  possibly  thalamic  overresponses 
from  thalamocortical  interference  at  cortical  levels. 

Certain  patients  show  definite  depressed  states — with  hypochon- 
driasis or  even  melancholic  suicidal  ideas.  Others  show  manic  pictures 
with  wild  flight  or  marked  maniacal  delirium.  Certain  patients 
develop  delirium  during  certain  of  their  headache  paroxysms.  Para- 
noid trends  also  manifest  themselves  in  a  few  instances.  So-called 
hysterical  symptoms  are  frequently  encountered.  Careful  analysis 
shows,  however,  no  psychical  conversions.  Emotionalism  and  delirium 
are  incorrectly  termed  hysterical  because  of  a  loose  application  of  the 
term  hysteria.  The  type  of  response  at  the  symbolic  level  is  probably 
conditioned  by  the  personality  make-up;  patients  showing  manic, 
depressed,  paranoid  tendencies  or  what  not  in  accordance  with  their 
characteristic  personality  trends  as  has  been  shown  in  other  organic 
conditions  (paresis).^ 

Optic  Nerve  Changes. — These  are  of  the  greatest  importance  in  diag- 
nosis of  brain  tumor.  The  general  features  are  discussed  in  the  chapter 
on  Cranial  Nerves.  From  60  to  80  per  cent,  of  all  patients  show  optic 
nerve  changes,  which  vary  largely,  depending  upon  the  grade  of  intra- 
cranial pressure  and  the  size  and  location  of  the  tumor.  Those  tumors 
causmg  great  intracranial  pressure  (posterior  fossa  particularly) 
naturally  cause  choked  disk  and  optic  neuritis  earlier  and  in  more 
marked  degree.  Optic  nerve  changes  may  be  absent  even  with  large 
tumors,  and  small  tumors  of  the  pons,  medulla,  motor  area,  basal 
ganglia,  corpus  callosum,  and  pituitary  may  give  rise  to  no  optic 
nerve  changes.  The  optic  nerve  changes  develop  gradually.  They  are 
unilateral,  later  bilateral  or  develop  bilaterally  synchronously.  As  a 
rule  tumors  of  one  side  show  beginning  nerve  changes  on  the  same 
side  earlier  and  more  markedly  than  on  the  opposite  side.  The  reverse 
can  also  be  true  and  the  localizing  value  must  not  be  overestimated. 
The  general  trend,  however,  is  as  stated  (Gunn). 

The  visual  power  may  not  be  lost  even  with  marked  grade  of  swelling 
or  of  atrophy,  but  there  is  later  a  gradual  loss  of  vision — often  seen 

'  Osnato:  Jour.  Nervous  and  Mental  Disease,  May,  Jqne,  1919,  vol,  \,  Kempf,  1.  c. 


FRONTAL   TUMORS 


673 


in  early  signs  by  the  irregularity  of  the  color  field  loss  (interlacing 
phenomena,  scotomata,  etc.)-  The  hemianopic  changes  which  are  not 
infrequent  in  chiasm,  posterior  tract,  pulvinar,  and  posterior  parts 
of  the  optic  pathways  are  discussed  in  Chapter  V. 

Motor  Phenomena. — These  are  local  or  general.  Epileptiform  attacks 
are  frequent — ^particularly  in  children.  WTien  limited  Jacksonian 
attacks  are  present,  the  localizing  value  in  the  motor  cortex  is  evident, 
save  for  the  few  exceptions  of  pontine,  cerebellar,  and  peduncular 
Jacksonian  attacks.  Petit  mal  attacks  are  also  not  infrequent.  Some 
patients  die  in  the  convulsive  seizures. 

Focal  or  Local  S3nnptoms. — These  may  be  among  the  first  of  the 
s^Tiiptoms  encountered,  especially  in  circumscribed  cortical  tumors, 
but,  as  a  rule,  the  focal  symptoms  develop  after  the  general  ones;  again 
they  advance  together.  They  are  best  considered  with  reference  to 
the  areas  involved,  such  as  the  symptoms  of  the  frontal  localizations, 
central  convolutions,  parietal  lobes,  temporal  etc. 


Fig.  370. — Frontal  lobe  tumor.     (Larkin.) 

Frontal  lobe  tumors  are  those  located  ahead  of  the  precentral  sulci. 
Those  of  the  frontal  poles,  foot  of  third  frontals,  are  frequently  termed 
prefrontal  tumors."^  The  functions  of  the  frontal  areas  are  chiefly 
those  of  coordination  of  psychical,  chiefly  intellectual  processes.^  They 
contain  motor  areas  for  the  innervation  of  the  muscles  of  the  neck, 
throat  and  abdominal  muscles  and  the  third  frontal  convolution — 
Brocas'  convolution — is  a  motor  aphasia  area,  INIarie  and  his  pupils 
notwithstanding.    On  the  base  the  olfactory  and  optic  pathwaysmay 


'  For  early  literature  see  Grimm:  Tumoren  an  den  vorderen  Hirnlappen,  Greifswald 
Diss.,  1868.  Newmark:  Prefrontal  Tumors,  California  State  Jour.  Med.,  November, 
1913. 

*  Franz;  Functions  of  the  Frontal  Lobes,  Archives  of  Psychology,  1907. 
43 


C74 


TUMORS  OF   THE  BRAIN 


bs  involved  by  direct  or  indirect  injury,  and  pressure  posteriorly  causes 
pyramidal  tract  symptoms.  Cerebellar  pathway  projections  also  lie 
in  the  frontal  poles. 

Thus  the  symptoms  of  tumors  lying  within  the  frontal  lobes  may 
show  considerable  variation  according  to  their  site  and  their  size, 
many  of  the  symptoms  being  due  not  to  the  local  involvement,  but  to 
pressure  upon  surrounding  structures  (neighborhood  symptoms). 
Many  small  tumors  located  deep  in  the  cortex  are  apparently  symp- 
tomless from  the  neurological  point  of  view.  Paranoid  states,  occasion- 
ally resulting  from  such  tumors,  are  readily  overlooked,  also  mild 
depressed  states  which  are  called  neurasthenia.^  One  symptom  of 
special  importance  is  a  tendency  to  make  jokes  (Witzelsucht),  or  a 
tendency  to  talk  or  answer  beside  the  point — at  times  an  apparently 
intentional  effort  to  mislead.    One  does  not  necessarily  locate  a  tumor 


Fig.  371. — Frontal  lobe  tumor  removed.  This  patient  had  loss  of  smell  on  the 
tumor  side.  Witzelsucht,  staggering  gait,  and  loss  of  control  of  feces  at  times,  with 
otherwise  unimpaired  intelligence.     Natural  size. 

in  the  frontal  lobes  by  reason  of  this  tendency  to  joking  alone.  At 
times  the  behavior  is  infantile  and  churlish,  and  diagnosed  hysteria. 
Again  patients  are  irritable,  excitable,  childish,  even  have  furious 
outbreaks  of  wrath  and  are  violent,  capricious,  or  the  picture  of 
gradually  advancing  stupidity,  with  inability  to  grasp,  loss  of  initia- 
tive, slowness  of  power  of  application,  is  seen.  In  right-handed 
tumors,  the  psychical  disturbances  are  more  frequent — Schiister,^  (80 
per  cent.),  Pfeiffer,^  Miiller.^  ^ 

Orientation  for  the  external  world,  time  and  space,  is  apt  to  be 
involved  more  than  personal  orientation.  Complete  disorientation, 
as  in  Korsakov's  syndrome,  is  occasionally  found. 


'Dynan:     New  York  Med.  Jour.,  November  4,  1911. 

2  Thesis,  Stuttgart,  1902.  '  Arch.  f.  Psychiat.,  vol.  xlvii. 

*  Deutsch.  Ztschr.  f.  Nervenh.,  vols,  xxi,  xxii,  xxiii. 

^  Blackburn:     Intracranial  Tumors  among  the  Insane. 


FRONTAL   TUMORS  C75 

At  times  hallucinations  of  smell  appear  from  pressure  on  the  olfac- 
tory pathways,  or  hallucinations  of  sight,  photomata,  from  similar 
pressure  on  optical  pathways  at  the  base. 

Vertigo,  with  a  drunken  gait,  may  be  ascribed  probably  to  the  higher 
association  of  space  perceptions  and  indicates  frontal  mvolvement 
of  cerebellar  components,  the  gait  being  closely  related  to  that  of 
cerebellar  SAiidromes — the  patient  staggers  to  the  tumor  side;  adia- 
dokokmesis  and  asAnergia  are  usually  absent  here,  however.  (See 
Cerebellar  S^mdromes.) 

Involuntary  defecation  or  urination  occurs  at  times ;  most  frequently 
with  somnolent  patients.  The  patient  whose  tumor  is  here  figured 
had  a  marked  cerebellar  gait,  was  keen  and  active,  jocular  and  happy, 
with  occasional  loss  of  bladder  and  rectal  function  and  hallucinations 
of  smell.    The  tumor  occupied  the  left  frontal  pole  (Fig.  371). 


Fig.  372. — Depression  in  brain  after  removal  of  a  frontal  tumor.      (Goodhart.) 

Apraxic  disturbances  are  occasionally  met  with  in  frontal  tumors, 
and  those  involving  or  pressing  upon  Brocas'  convolution,  left  side, 
cause  mild  (paraphasia),  or  severe  motor  aphasias  in  right-handed 
individuals,  usually  of  gradual  onset  and  often  remittent  ui  character. 

Other  motor  signs  are  stifi'ness  in  the  neck  with  forward  and  back- 
ward fixations  of  the  hand  and  tendencies  to  tremor  of  the  hand  on 
the  side  of  the  tumor.  In  a  third  of  the  cases,  epileptiform  attacks, 
often  Jacksonian,  occur,  from  pressure  on  the  motor  area. 

Central  Conwluiions. — The  functions  chiefly  involved  are  those 
of  the  voluntary  muscular  activity,  hence  paresis,  paralysis,  spasms. 


676 


TUMORS  OF  THE  BRAIN 


Tumors  of  this  region  are  never  latent.  Irritative  phenomena,  spasms, 
convulsions,  speak  for  cortical  location;  paralysis  for  deeper-seated 
lesions  involving  the  pyramidal  paths  from  the  motor  areas.  Small 
tumors,  cortically  located,  cause  isolated  Jacksonian  attacks;  the 
more  extended  the  tumor  the  more  widespread  the  muscular  involve- 


FiG.  373. — Multiple  sarcoma  of  brain.     (Larkin.) 


ment;  even  small  tumors,  however,  may  cause  widespread  Jacksonian 
or  grand  mal  symptoms.  Often  the  first  observed  motion  accompanied 
by  tingling  afTords  a  clue  as  to  the  more  definite  localization  of  the 
tumor;  again  an  orderly  and  uniform  progression  in  the  development 
of  a  Jacksonian  attack  is  valuable  in  localization. 


PARIETAL  AND   TEMPORAL  TUMORS  677 

Monoplegise^  and  monopareses  are  not  infrequent  from  small  lesions, 
and  in  the  beginning  of  the  tumor  growth.  The  slow  extension  of  the 
paresis  or  paralysis  is  of  diagnostic  moment.  Advancing  hemiplegia 
is  often  accompanied  bv  the  vasomotor  phenomena  seen  in  hemor- 
rhage— especially  in  deep-seated  lesions.  The  usual  signs  of  an  organic 
paralysis  [q.  v.)  are  present. 

Psychical  symptoms  of  general  nature  are  not  infrequent.  Occa- 
sionally large  tumors  will  cause  a  Korsakov  sjTidrome.  Katatonic 
symptoms  may  also  appear.  Sensory  phenomena  are  frequent  in 
postcentral  convolution  tumors.  The  phenomena  have  been  exten- 
sively described  in  the  chapter  dealing  with  sensory  changes  due  to 
cortical  and  subcortical  lesions,     (page  646  et  seq.) 

Central  convolution  tumors  are  among  those  more  readily  localizable, 
and  usually  more  accessible  to  operative  relief. 

Parietal  Lobes. — ^\Vhen  the  tumors  press  forw'ard  toward  the  pos- 
terior central  lobes  sensory  signs  are  produced  similar  to  those  already 
mentioned.  Left-sided  tumors,  in  right-handed  persons,  especially  of 
the  inferior  parietal  lobes,  cause  cortical  sensory  aphasia  of  Wernicke 
of  various  grades.  x\lexia  and  agraphia  may  also  be  fomid  in  left-sided 
lesions. 

Epileptic  attacks  with  hallucmatory  auras  of  taste  occur;  also  a 
general  Korsakov  sjiidrome  may  develop.  Other  pathways  from  the 
sensory  areas  may  be  cut  off  by  tumors  in  this  region;  hence  either 
hemianopsias,  optic  agnosias  or  optical  aphasias  (gyrus  angularis). 
Apraxic  disturbances  are  of  value  in  localizing  left-sided  tumors. 
Katatonic  s^Tidromes,  confusion,  apathy,  or  general  loss  of  orientation 
may  be  encountered  but  are  equally  present  in  right-sided  and  left- 
sided  cases. 

Occasionally  parietal  tumors  give  rise  to  ptosis,  paresis  of  the 
lateral  movements  of  the  head  and  the  conjugate  motion  of  the  eyes 
to  the  opposite  side.  (Cephalorotary  and  oculorotary  paralysis,  see 
Plate  VIII.) 

Deep-seated  lesions  may  impinge  upon  or  involve  the  motor  path- 
ways. 

Temporal  Lobes. — ^The  cortical  end-stations  of  the  auditory  path- 
ways which  are  both  crossed  and  uncrossed  are  chiefly  contained  in 
the  first  and  second  temporal  lobes.  Cortical  deafness  is  practically 
impossible  in  unilateral  lesions  but  has  resulted  from  bilateral  involve- 
ment. 

Word-deafness  is  the  most  striking  result  in  left-sided  lesions.  This 
is  a  progressive  affair,  often  beginnmg  with  difficulty  in  finding  words, 
paraphasia,  and  resulting  in  more  severe  forms  m  alexia,  agraphia, 
logorrhea,  and  total  word-deafness.  Lai'ge  tumors  also  cause  hidirect 
symptoms  and  may  lead,  by  pressure  on  motor  areas — Broca — to 
total  aphasia,  and  epileptiform  convulsions. 

Auditory  hallucinations  are  not  infrequent,  showing  as  aurte  in 

•Bergmark:     Monoplegia,  Brain,  1910. 


678 


TUMORS  OF   THE  BRAIN 


generalized  grand  mal  attacks.  Gustatory  and  olfactory  phenomena 
of  similar  nature  result  from  hippocampal  or  closely  related  lesions — 
uncinate  fits. 


Fig.  374. — Glioma  of  temporal  lobes. 


Fig.  375. — Tumor  of  temporo-occipital  lobes.      (Larkin.) 

Tumors  of  the  under  surface  may  cause  hemianopsia,  through  press- 
ure on  the  optic  tracts,  and  by  pressure  on  the  pyramidal  or  fillet 
tracts  cause  hemiparesis  or  hemianesthesia. 


OCCIPITAL  LOBE  TUMORS  679 

In  certain  cases,  as  Knapp^  has  sho^NTi,  there  may  be  an  orderly 
progression  of  symptoms  due  to  direct  or  indirect  action  from  temporal 
lobe  tumors.  The  most  striking  of  these  is  a  homo-  or  contralateral 
temporary  or  persistent  oculomotor  palsy,  chiefly  ptosis  and  mydriasis, 
crossed  hemiplegia,  and  cerebellar  ataxia. 

Occipital  Lobe. — The  end-projections  of  the  optic  tracts  are  located 
here  particularly  in  and  about  the  calcarine  fissure.  Complete  homony- 
mous hemianopsia  is  the  chief  symptom  of  tumors  of  this  area.  This 
hemianopsia  usually  spares  the  papillomacular  bundles  and  is  often 
unperceived  by  the  patient.     Quadrant  hemianopsia  is  also  found .^ 


Fig.  376. — Tumor  arising  from  the  meninges  and  pressing  down  to  the  corpus  callosum. 


(Baldwan.3) 

Tumors  may  exist  and  hemianopsia  be  absent.  It  is  most  often 
present  with  tumors  of  the  median  aspect  of  the  occipital,  also  with 
those  lying  on  the  con\'ex  surface,  and  hence  the  more  readily  remov- 
able. Various  stages  of  blindness  may  also  result,  and  there  may  exist 
a  mind-blindness  from  left-sided  tumors,  also  alexia,  agraphia  and 
sensorv'  aphasia. 

*  Die  Geschwiilste  d.  rechten  u.  linken  Schlafenhippens,  1905;  Mvinchen.  med. 
Wchnschr.,  1908;    Deutsche  med.  Wchnschr.,  June  27,  1918. 

^Bramwell:  Lesions  of  Occipital  Lolx;  and  Affections  of  Vision,  Clinical  Studies. 
Holmes  and  Lister,  loc.  cit.     See  Chapter  on  Cranial  Nerves,  p.  307. 

5  Jour.  Nerv.  and  Ment.  Dis.,  1901. 


680 


TUMORS  OF  THE  BRAIN 


Other  optic  signs,  such  as  phonemes,  various  scintillating  scotomata, 
optical  hallucinations  and  illusions  occasionally  result  from  occipital 
tumors,  and  these  may  exist  as  auree  preceding  general  epileptic 
convulsions. 

Tumors  on  the  inferior  surface,  by  compression  of  the  cerebellum, 
will  cause  cerebellar  signs,  and  occasionally  palsies  result  from  tumors 
lying  on  the  external  surface  of  the  occipital  lobe. 

Corpus  Callosum} — Isolated  callosal  lesions  are  extremely  rare. 
Schuster  gathers  37;  Williams  38.  Congenital  absence  is  known  with 
no  definite  symptoms.  Apraxia  (dyspraxia)  of  the  left  hand  is  an 
occasional  symptom  due  to   interruption   of  forward-lying  callosal 


Fig.  377. — Tumor  with  atrophy  of  the  cerebellum. 

fibers,  especially  those  between  the  sensorimotor  areas,  from  left  to 
right,  and  is  of  special  significance  in  diagnosis.  Possibly  mind-blind- 
ness results  from  lesions  of  those  callosal  fibers  uniting  the  two  optic 
fields  (Brodmann  18,  19),  also  termed  the  visual  psychic  area.  This 
is  not  yet  established  definitely .^ 

Paretic  syndromes  of  the  extremities  occur  with  callosal  tumors. 
Dupre  locates  them  in  the  anterior  callosal  region  if  the  paretic  symp- 
toms are  in  the  muscles  of  the  face  and  tongue;  pareses  and  ataxias 
of  the  upper  extremities  from  midcallosal  fibers;  paresis,  ataxia  and 


1  Ayala:     Riv.  d.  patol.  Nerv.  and  Ment.,  1915,  p.  449. 

2  Van  Valkenberg:     Brain,  November,  1913. 


Hammacher:     Kiel,  1910. 


TUMORS  OF   THE  BRAIN  681 

hemianopsia  from  posterior  callosal  fibers.  Van  Valkenberg's 
extensive  studies  of  the  callosum  do  not  at  all  corroborate  Dupre's 
deductions. 

Tumors  of  the  callosum  are  more  apt  to  show  complex  syndromes 
due  to  involvement  of  the  neighboring  parts ;  if  lying  fonv^ard,  frontal 
signs  are  added;  midcallosal  region,  pyramidal  tracts,  epilepsies,  etc. 
General  psychical  symptoms  are  usually  present,  and  appear  early. 
They  approach  the  t;ype  due  to  general  pressure.  Korsakov  and 
paretic  sjiidromes  develop.  There  is  little  of  diagnostic  significance 
in  the  mental  signs. 

Tumors  of  the  Optic  Thalamus. — The  symptoms  are  discussed  under 
the  section  on  the  Thalamic  Syndrome  {q.  v.).  Involvement  of  the 
lenticular  nucleus  (see  Wilson's  disease),  tumors  of  the  midbrain, 
pons  and  medulla  have  been  discussed.  Those  of  the  cerebellum  and 
cerebellopontine  angle  will  be  found  under  Cerebellar  S\Tidromes. 

Diagnosis. — Multiple  sclerosis,  paresis,  arteriosclerotic  disease,  all 
forms  of  headache,  tuberculous  meningitis,  chronic  hydrocephalus, 
and  hysteria  are  the  chief  conditions  causing  difficulty. 

Multiple  sclerosis,  if  the  patches  are  solely  cerebral,  may  cause 
confusion,  especially  in  the  acute  cases,  as  described  by  Marburg  and 
others.  The  bitemporal  pallor  of  the  disks  in  this  disorder  differs 
from  the  usual  pressure  changes  in  the  disk.  Nystagmus  is  not  a 
frequent  brain-tumor  sign.  Other  signs  of  pyramidal  tract  implica- 
tion may  be  identical.  Headaches  are  usually  absent,  also  nausea  and 
vomiting.     • 

Paresis  with  marked  focalization  of  the  pathological  process  may  for 
a  time  closely  simulate  the  picture  of  tumor. 

Pseudoparetic  and  arteriosclerotic  psychical  syndromes  (Korsakov's 
psychosis)  are  frequentlj-  confused  with  cerebral  tumor,  i.  e.,  the  cause 
of  the  mental  picture  is  overlooked.  Thus  a  tumor,  which  might  have 
been  removed,  has  been  missed  under  the  psychotic  disguise.  Eye- 
ground  changes  are  usually  positive  in  these  cases,  yet  may  be 
absent. 

Headache  should  always  be  scrutinized  carefully.  A  postinfluenzal 
occipital  headache  which  is  very  frequent  and  extremely  severe  and 
persistent,  is  frequently  highly  suggestive  of  brain  tumor.  The  head- 
aches from  lead  poisoning,  anemia,  and  nephritis  are  also  to  be  excluded. 

Brain  abscess  and  tuberculous  meningitis  must  be  excluded  on  the 
ground  of  their  difference  in  development  of  symptoms.  Symptomati- 
cally  speaking,  they  may  be  considered  as  tumors.  This  is  also  true 
of  chronic  hydrocephalus. 

Cerebral  puncture,  withdrawing  a  small  plug  of  brain  tissue  through 
a  trephine  opening  and  cannula,  is  often  of  great  aid  in  diagnosing  very 
puzzling  cases. 

Prognosis. — No  definite  prognosis  can  be  laid  down.  All  depends 
on  the  site  and  nature  of  the  tumor.  In  general,  apart  from  surgical 
relief  and  from  medicinal  treatment  of  syphilomata,  the  outlook  is 
pessimistic.    Sudden  death  is  not  infrequent  and  lumbar  puncture  is 


682  TUMORS  OF  THE  BRAIN 

an  extremely  dangerous  procedure  with  brain  tumor,  often  leading  to 
sudden  collapse  and  death,  especially  with  tumors  of  the  posterior  fossa. 

SA-philomata  and  gummata  of  the  brain  have  a  fair  prognosis.  Better 
results  are  obtained  with  mercury  by  inunction  and  by  iodides  than 
by  salvarsan  in  the  beginning  treatment.  Salvarsan  may  be  used 
later  to  attempt  to  kill  off  all  the  spirochetes,  but  with  well-advanced 
s^-philomata,  salvarsan  is  apt  to  set  up  a  dangerous  reaction.  Round, 
hard  gummata  do  not  absorb,  as  a  rule,  and  are  best  considered 
surgically. 

Treatment. — ^Medicinal  treatment,  excepting  for  syphilomata,  is 
useless,  and  involves  a  waste  of  valuable  time.  The  chief  objects  to  be 
attained  are  early  diagnosis,  immediate  exclusion  of  s;v'philis  by  sero- 
logical tests,  eye-ground  examination  with  particular  study  of  the 
color  fields,  exact  localization  as  soon  as  possible,  and  surgical  removal 
or  palliation  (decompression)  to  save  the  eyesight  or  to  gain  time  for 
a  more  exact  localization. 

The  details  for  applying  these  principles  have  already  been  noted. 
The  results  to  be  expected  in  any.  particular  case  are  problem,  atical, 
yet  from  10  to  20  per  cent,  of  all  brain  tumors  (seen  in  the  large) 
have  been  removable,  with  at  least  in  10  per  cent,  practical  recovery. 
Even  with  such  chances  against  him  the  patient  should  have  the 
benefit  of  the  doubt  if  a  competent  surgeon  is  available.  Surgical 
skill  is  a  very  large  factor  in  the  results;  a  good  abdominal  surgeon 
is  not  necessarily  a  good  brain  surgeon.  The  brain  is  semifluid  and  an 
intricate  switchboard  of  highly  important  structures;  there  are  no 
unimportant  areas  in  the  brain.  Many  surgeons  have  treated  it  in  the 
past  as  though  it  were  an  abdominal  viscus;  the  results  have  been 
disastrous. 

Most  brain  operations  are  best  done  in  two  stages.  Decompression, 
usually  subtemporal,  alone  is  often  the  only  possible  procedure.  It 
often  relieves  a  recently  acquired  blindness. 

The  situation  referable  to  brain  surgery  for  cerebral  tumors  is 
rapidly  advancing,  and  better  and  better  results  are  being  obtained 
and  regions  hitherto  impossible  to  reach  (pituitary,  etc.)  have  been 
approached  with  results  which  a  decade  ago  would  have  been  impos- 
sible.^ Notwithstanding  all  this,  the  general  attitude  should  be  one 
of  extreme  caution. 

>  See  Starr:  Brain  Surgery.  Halsted:  Treatment  of  Brain  Tumor.  White  and 
Jelliffe:     Modern  Treatment  of  Nervous  and  Mental  Diseases. 


CHAPTER  XIV. 
NEUROSYPHILIS. 

Syphilis  of  the  nervous  system  is  so  protean  in  its  clinical  forms, 
that  the  significance  of  syphilis  as  an  etiological  factor  in  nervous 
diseases  is  frequently  overlooked.  The  physician  with  his  eye  riveted 
upon  a  clinical  picture,  be  it  an  amyotrophic  lateral  sclerosis,  a  failing 
memon-,  a  persistent  nervous  weakness,  an  isolated  cranial  nerve 
palsy,  a  progressibe  muscular  atrophy  of  the  arm  muscles,  or  a  pro- 
tracted sciatica,  may  readily  overlook  the  fact  that  syphilis  is  the 
unique  cause  for  these  different  syndromes. 

There  is  no  field  in  medicine  wherein  similar  disease  pictures  may 
arise  from  so  many  differing  causes  as  in  the  domain  of  the  nervous 
system.  Nor,  on  the  other  hand,  where  a  single  etiological  factor  may 
give  rise  to  so  many  dissimilar  clinical  pictures.  Hence  the  com- 
plexity of  the  whole  subject,  and  the  need  for  iterating  and  reiterating 
the  advice  that  in  nearly  all  of  the  clinical  pictures  which  have  or  have 
not  been  given  descriptive  terms  in  nervous  or  mental  disease,  the 
possibility  of  syphilis  as  a  direct  etiological  factor  or  as  a  complica- 
tion, or  as  causing  confusion,  should  be  borne  in  mind.  Hence  the 
necessity  for  detailed  and  minute  inquiries  into  all  of  the  possible 
symptomatology  of  hereditary  or  acquired  syphilis,  which  in  all 
questionable  cases  should  be  supplemented  by  complete  serological 
and  cytological  examinations. 

The  formerly  veiy  distinct  dividing  lines  between  the  lesions  of 
hereditary  syphilis,  acquired  syphilis,  and  metasyphilis  are  slowly 
being  obliterated,  and  in  time  it  may  probably  seem  strange  that  it 
could  have  been  thought  that  spinal  and  cerebral  syphilis  on  the  one 
hand  should  ever  have  been  considered  different,  let  us  say,  from  tabes 
and  general  paresis  on  the  other. 

Since  it  has  been  demonstrated  that  both  tabes  and  general  paresis 
are  syphilitic  in  nature,  the  variety  of  SA-philitic  disorders  showing 
fairly  clear  clinical  entities  has  been  enlarged.  Further,  with  the 
recognition  of  many  acute  and  subacute  psychoses  due  to  syphilis 
the  psychiatric  borders  have  been  further  extended. 

In  the  present  chapter,  then,  the  discussion  will  concern  itself  chiefly 
with  clinical  forms,  without  any  extended  attempt  behig  made  to 
differentiate  tjpes,  which  in  reality  are  so  kaleidoscopic  that  they 
defy  description. 

At  the  same  time  it  needs  to  be  emphasized  that  such  intermedian*- 
forms  are  ever-present  realities.  Classical  pictures  of  a  disease  are 
largely  literarj-  efforts.    They  are  the  product  mainly  of  the  descriptive 

(683) 


684  NEUROSYPHILIS 

art.  The  actual  processes  going  on  in  nature  in  the  conflict  of  man 
against  the  spirochete  do  not  show  classical  types,  but  rather  a  multi- 
plicity of  variations,  with  here  and  there  the  statistical  prominence 
of  this  or  that  trend  in  the  reaction,  which  is  called  the  disease  and 
given  a  label. 

History. — Leoncino  as  early  as  1497  described  paralysis  as  a  con- 
sequence of  sj^philitic  infection.  He  here  referred  to  what  is  known 
as  hemiplegia,  which  may  result,  as  is  well  known,  within  a  few  years, 
even  a  few  months,  after  infection. 

Joseph  Griinbeck  (1503),  Emser  (1511),  Ulrich  von  Hutten  (1519), 
all  lay  writers,  mention  paralyses  of  the  limbs,  as  due  to  the  disease. 
Emser  speaks  of  his  patient,  a  syphilitic  paralytic,  and  with  a  psychosis, 
as  having  made  a  remarkable  recovery,  under  treatment  by  Bruno, 
by  making  a  vow.  Paracelsus  (1530),  although  still  confusing  gon- 
orrhea with  syphilis,  as  had  been  and  is  still  being  done,  through  the 
senseless  use  of  the  term  "venereal"  disease,  left  indications  of  a 
description  of  syphilitic  meningitis,  and  in  speaking  of  the  syphilitic 
virus  said  that  it  affected  all  of  the  organs  of  the  body.  Nowhere 
in  Fracastorius  (1521),  who  gave  the  name  syphilis,  are  direct  refer- 
ences to  the  nervous  system  to  be  found.  Nicolaus  Massa  (1556) 
gave  an  early  description  of  syphilitic  neuralgias.  Borgarutius  (1567) 
also  described  neuralgic  pains  due  to  syphilitic  disease  of  the  meninges. 
Amatus  Lusitanus  (1561)  described  headaches  due  to  intracranial 
osteitis  of  syphilitic  origin.  Botalli  (1563)  made  an  observation  that 
blindness  might  be  due  to  syphilitic  disease  of  the  brain.  Ferrus  (1537) 
Morgagni  (1600)  noted  the  arthropathies. 

During  the  following  century  many  references  have  been  found 
showing  the  recognition  of  the  relationship  of  syphilis  to  nervous 
disease.  Only  a  few  can  be  mentioned  here.  Thus,  Guarinoni  (1610) 
described  epileptic  attacks  from  syphilis  of  the  brain.  Vidus  Vidius 
(1611)  described  epilepsies  as  due  to  syphilitic  cranial  caries.  Thiery 
de  Hery  (1634)  and  Zechius  (1650)  also  called  attention  to  syphilitic 
spasms  as  well  as  epilepsies.  Zacutus  Lusitanus  (1644)  described 
cases  of  blindness  due  to  gumma  of  the  brain,  quoting  Botalli  a  century 
ahead  of  him .  In  1696  a  special  treatise  on  syphilitic  pains  was  written 
by  Blagny. 

Attention  might  be  called  to  the  works  of  Scholtzius  (1610)  and 
Willis  (1672)  a  propos  of  the  subject  of  general  paresis.  To  Willis 
has  always  been  ascribed  the  honor  of  the  first  description  in  which 
one  could  definitely  recognize  general  paresis. 

By  the  end  of  this  (seventeenth)  century  a  fairly  broad  view  of 
syphilitic  nervous  disease  had  been  obtained.  Syphilitic  headaches 
were  described  by  Felix  Plater  (1641).  Rhodius  (1657)  described 
gummata  of  the  dura  and  syphilitic  hemiplegias;  Ballen  (1663) 
spinal  syphilitic  disease  and  spasms  in  the  facial  region;  Cummius 
(1684),  diplopias  and  eye  palsies.  Astruc  has  reviewed  these  writings 
completely. 

During   the    eighteenth    century    the   picture    expanded    rapidly. 


HISTORICAL  685 

Intercostal  neuralgias  (1762),  deafness,  loss  of  smell,  caries  of  base  of 
skull  (1762),  sciatica  (1745),  psychoses.  S}-philitic  mania  was  described 
by  Sanche  in  1777,  amaurosis  (1748),  facial  palsy  (1758),  leptomenin- 
gitis (1766),  syphilitic  arterial  disease  (1766),  paraplegias  (myelitis) 
(1771),  and  a  number  of  other  conditions  were  described,  and  may 
be  consulted  in  Lagneau's  interesting  monograph  in  which  234  case 
histories  are  collected.  Astruc,  Bonet,  and  Morgagni  offer  the  richest 
literary'  sources. 

It  may  be  recalled  in  this  place  that  John  Hunter,  in  1787,  stated 
that  he  never  observed  syphilis  in  the  internal  organs,  including  the 
brain.  The  weight  of  his  authority  retarded  progress  for  many  years, 
especially  in  England.  Indeed,  it  was  not  until  Ricord's  sound  obser- 
vations were  published  that  Hunter's  enormous  blunder  was  fully 
remedied. 

Virchow's  studies  (1847)  on  phlebitis  and  arteritis  had  laid  the 
foundation  for  the  modern  knowledge  of  bloodvessel  syphilis,  although 
it  may  be  recalled  that  Morgagni  (1766)  and  Home  (1782)  both  made 
extremely  important  studies  on  vascular  sj^hilis.  These  have  been 
fully  developed  by  Huebner  (1874)  and  Alzheimer  (1904). 

The  studies  of  Virchow  on  the  formation  of  gummatous  granulo- 
mata  and  related  syphilitic  phenomena,  practically  established  the 
modern  era  of  study  of  the  pathology  of  this  disease. 

The  succeeding  years  have  filled  in  the  picture  with  a  number  of 
details,  the  chief  additions  having  been  those  of  Nissl  and  Alzheimer, 
who  have  established  the  highest  criteria  for  the  pathology  of  this 
disease  so  far  as  the  nervous  system  is  concerned. 

The  latest  chapter  in  this  interesting  history  is  that  dealing  with 
the  discovery  of  the  inciting  agent,  and  the  final  clearing  up  of  the 
entire  subject  of  etiology  and  modes  of  infection.  Schaudinn^  (March 
3,  1905)  found  the  parasite  which  he  first  called  Spirocheta  pallida. 
Its  synonymy  has  varied  to  accord  with  principles  of  botanical  and  zoo- 
logical nomenclature.  Treponema  palHdum  has  the  best  sanction, 
which  in  zoological  nomenclature  is  not  governed  by  usage  but  by 
definite  principles.  Doele  (1892)  is  thought  to  have  first  seen  the 
parasite,  but  Schaudinn,  then  Epaschen,  Fischer,  Metchnikoff,  and 
Roux  established  its  identity  and  its  affiliations  with  the  protozoa.- 

It  is  found  in  congenital  syphilis  of  the  nervous  system,  in  syphilitic 
gumma  of  the  brain  and  spinal  cord,  in  syphilitic  meningitis,  even  in 
the  cerebrospinal  fluid,  both  of  congenital  and  acquired  syphilitics. 
Moore  (1913),  Xoguchi,  Nichols,  and  Hough  found  it  in  the  paretic 
brain,  and  it  has  been  found  in  the  spinal  meninges  of  tabetics. 

The  final  studies  of  Xeisser,  Metchnikoff,  Roux,  and  others  have 
laid  bare  the  entire  story  of  the  inoculability  of  the  disease  and  its 

'  Arbeiten  aus  dem  Kaiserlichen  Gesundheitsamte,  April  23,  1905. 

'See  Noguchi:  "Spiroche<^es,"  Jour.  Lab.  and  Clin.  Med.,  March,  1917.  Also  con- 
sult Stein,  J.  B.:  Med.  Record,  April  13,  1912,  for  an  interesting  history  of  the  details 
of  the  discovery  of  Treponema  pallidum,  illustrated  by  photographs  of  the  chief  investi- 
gators of  the  problem  to  that  date. 


686  NEUROSYPHILIS 

transmission  from  animal  to  animal,  while,  utilizing  the  knowledge 
gained  by  Bordet  and  Gengou,  Wassermann  and  his  pupils  have 
elaborated  a  serobiological  technic  which  has  made  one  largely  inde- 
pendent of  clinical  or  anamnesic  data  relative  to  a  knowledge  on  the 
part  of  the  patient  of  the  infection. 

Thus,  in  the  past  decade,  a  flood  of  light  has  been  thrown  upon  the 
disease  and  its  relation  to  other  protozoan-caused  diseases,  notably 
trypanosomiasis,  which  has  illuminated  and  made  clear  the  entire  path 
which  has  been  so  busily  travelled  since  Columbus  brought  this  most 
portentious  exotic  to  the  new  world. 

Diagnosis.- — The  diagnosis  of  syphilis  of  the  nervous  system  presents 
certain  difficulties  which  rapidly  increasing  perfections  in  laboratory 
technic  are  resolving  with  considerable  success.  These  laboratory 
findings,  combined  with  those  of  the  neurological  examination,  permit 
an  almost  certain  diagnosis  of  this  disease  in  the  nervous  system,  either 
as  congenital,  acquired,  or  as  so-called  para-  or  metasyphilis. 

The  chief  features  in  such  diagnosis  are:  (1)  search  for  the  organ- 
isms; (2)  serological  investigation  of  the  blood  and  cerebrospinal  fluid; 
(3)  cytological  examination  of  the  cerebrospinal  fluid;  (4)  chemical 
examination  of  the  cerebrospinal  fluid;  (5)  clinical  examination  of  the 
pupillary  reflexes;  (6)  general  neiu-ological  and  psychiatric  signs. 

1.  Search  for  Organisms. — The  parasite  has  been  found  in  the 
cerebrospinal  fluid,  but  as  yet  in  but  few  instances.  It  has  been 
cultivated  from  the  cerebrospinal  fluid  (Nichols  and  Hough).  More 
recent  work  by  injecting  the  fluid  into  the  testicle  of  rabbits  has  added 
considerable  information.^  The  results  thus  obtained  added  to  those 
previously  on  record  show  positive  findings  in  primary  and  secondary 
syphilis,  without  nervous  manifestations,  in  a  total  of  2  cases  (none 
before  the  eighth  week) ;  in  secondary  syphilis,  from  the  third  to  the 
twelfth  month,  in  7  cases;  never  in  tertiary  syphilis.  In  the  cases  with 
objective  nervous  symptoms:  in  early  syphilitic  meningitis;  once; 
neurorecurrence  (eight  months),  once;  apoplexy  and  hemiplegia 
(seven  months),  once;  (one  year),  once;  syphilitic  meningitis,  once; 
spinal  syphilis  (ten  years),  once;  tabes,  2  cases,  and  progressive  par- 
alysis, 5  cases.  In  inherited  syphilis,  soon  after  birth,  2  cases;  syphilitic 
leptomeningitis  later  in  life,  once,  and  juvenile  paralysis,  once.  This 
compilation  includes  Friihwald  and  Zaloziecki's  23  syphilitic  patients 
tested  in  this  way.  Spirochetes  were  found  in  only  4  of  the  total  23; 
once  in  recent  secondary  syphilis,  once  in  older  secondary  syphilis 
(without  nervous  symptoms),  once  in  early  meningitis,  and  once  in 
progressive  paralysis.  They  add  that  large  numbers  of  others  with 
tabes,  paralysis  and  brain  syphilis  were  also  tested,  with  constantly 
negative  results.  The  inoculation  lesion  in  the  rabbit  testicle  was  so 
slight  that  only  the  microscope  disclosed  its  positive  nature.  Fildes^ 
found  the  organism  once  in  15  fluids  investigated.  The  pleocytosis 
was  1000  cells  per  c.mm.  in   one   patient  only   twenty  weeks  after 

*  Friihwald,  Zaloziecki:     Berl.  klin.  Wchnschr.,  January  3,  1916. 
2  American  Arch.  Neur.  and  Psych.,  1919,  i,  231. 


CEREBROSPINAL  FLUID  687 

infection.  The  data  presented  show  that  the  cerebrospinal  fluid  only 
rarely  contains  the  spirochete.  The  fluid  otherwise  may  or  may  not  be 
normal,  and  there  may  or  may  not  be  a  positive  seroreaction  and  symp- 
toms on  the  part  of  the  nervous  system.  There  is  thus  no  regularity 
in  the  findings. 

2.  Biological  or  Serological  Tests  of  Blood  arid  Cerebrospinal  Fluid. 
—(a)  Blood. — Practically  all  forms  of  early  s^'philis  of  the  nervous 
system  should  show  a  positive  Bordet-Wassermann  reaction  in  the 
blood.  There  are  exceptions,  but  s}^hilis  of  the  nervous  system,  like 
syphilis  elsewhere,  gives  a  positive  reaction  in  early  cases. 

Whereas,  the  number  of  positive  findings  should  be  as  high  as 
100  per  cent,  in  cases  of  early  neurosj^philis,  in  its  secondary  and 
tertiary  stages  the  number  of  positive  results  may  fall  to  even  70 
per  cent.,  and  in  the  latent  period  may  sink  to  50  per  cent.  Whether 
these  reduced  percentages  speak  for  the  good  results  of  treatment  or  are 
dependent  upon  other  as  yet  uncertain  factors  is  yet  to  be  decided. 
The  alcohols  interfere  with  the  reaction.  Hence  the  results  of  a  Wasser- 
mann  test  are  apt  to  be  unreliable  if  the  patient  has  taken  alcohol 
within  forty-eight  hours  of  the  time  of  making  the  test. 

(6)  Cerebrospinal  Fluid. — The  beha"^'ior  of  the  cerebrospinal  fluid 
to  the  Wassermann  reaction  is  of  special  significance  in  the  diagnosis 
and  treatment  of  neurosyphilis.  It  is  almost  uniformly  positive  in 
general  paresis,  even  when  small  quantities  (0.02  c.c.)  of  the  serum  are 
employed.  By  the  use  of  small  quantities  Hauptmann  and  Hossli 
attempted  to  show  that  paresis  might  be  separated  from  other  types 
of  neurosyphilis,  all  of  which  will  cause  a  positive  result,  with  larger 
quantities  of  cerebrospinal  fluid  (0.4  to  0.8  c.c).  Later  studies  tend 
to  show  that  whereas  this  may  be  a  useful  differential  at  times,  it  is  not 
reliable,  for  the  intensity  of  the  serological  and  cytological  reactions 
seem  to  depend  more  upon  the  localization  and  intensity  of  the  process 
than  upon  the  kind.  Thus  meningeal  involvements  of  the  cord  and 
brain  tend  to  show  marked  positive  reactions,  while  intracerebral 
vascular  lesions  tend  to  run  low  in  respect  to  both  cellular  and  sera 
criteria.  Central  parenchymatous  tj^pes  almost  always  show  positive 
serological  and  cytological  changes. 

Syphilis  without  nervous  involvement  usually  give  a  negative  reac- 
tion, even  when  large  quantities  of  the  flu'd  are  used  but  many  cases 
of  undoubted  neurosyphilis  will  show  both  pleocytosis  and  positive 
Bordet-Wassermann  in  the  fluid,  long  before  evident  neurological  or 
psychiatric  symptoms.^  - 

It  must  constantly  be  borne  in  mind  in  the  diagnosis  of  syphilis  of 
the  nervous  system  that  the  reaction  of  the  cerebrospinal  fluid  in  the 
Wassermann  test  is  purely  monosymptomatic.  The  positive  or  nega- 
tive results  must  always  be  interpreted  in  association  with  other 
laboratory  and  clinical  tests.     As  Xonne  has  well  said,  the  Wasser- 

'  See  Kaplan:     Serology  in  Nerv^ous  Syphilis,  Philadelphia,  1915. 
^Compare  study  of  Fildes  et  al,  loc.  cit.     Cornaz:     Jour.   Nerv.   and   Ment.   Dis., 
1919.     Head  and  Fearnsides:    Brain,  vol.  xxxvi. 


688 


NEUROSYPHILIS 


mann  reaction  is  only  a  symptom.  Like  other  symptoms  in  a  syndrome 
it  may  or  may  not  be  present  without  affecting  the  validity  of  the 
syndrome  from  its  diagnostic  aspects. 

3.  Cytological  Examination  of  the  Cerebrospinal  Fluid. — ^The  technic 
of  punctm-e  cannot  be  entered  into  here,  but  one  point  should  be 
borne  in  mind:  the  procedure  is  not  always  without  danger.  It 
should  be  done  with  care,  the  fluid  being  withdrawn  very  slowly,  drop- 
wise  in  some  cases,  and  the  patient  should  rest  in  bed'  several  hours, 
preferably  both  before  and  after  the  operation.  One  of  the  functions 
of  the  cerebrospinal  fluid  is  to  maintain  an  equality  in  the  intracerebral 
pressure,  and  any  sudden  alteration,  such  as  is  produced  by  the  with- 


FiG.  378.- 


-Lymphocytes  in  normal  (left)  and  paretic  (right)  cerebrospinal  fluid. 
(Kraepelin.) 


drawal  of  5,  10,  or  15  c.c.  of  the  fluid,  is  apt  to  disturb  such  equilibrium. 
Headache,  nausea,  vomiting,  dizziness,  are  among  the  unpleasant 
effects  in  those  who  react  badly.  Such  are  few,  but  they  exist,  and 
care  is  imperative.  Some  are  helped  by  lying  quiet,  with  foot  of  bed 
elevated  and  with  aspirin,  10  grains,  repeated  if  necessary.  In  patients 
in  whom  brain  tumor  is  suspected  special  care  should  be  taken,  as 
here  a  few  cases  of  sudden  death  have  been  reported  following  lumbar 
puncture  with  withdrawal  of  fluid. 

In  pathological  cerebrospinal  fluid  an  increase  in  lymphocytes  is 
the  most  striking  feature.  Over  7  to  10  lymphocytes  to  the  cubic 
millimeter  indicates  pathological  fluid.  Other  ceUs  may  also  be  found, 
such  as  polymorphonuclear  leukocytes,  plasma  cells,  and  occasionally 
eosinophile  and  endothelial  cells.  Red  blood  cells  usually  come  from 
the  wound  of  the  puncture. 


CEREBROSPINAL   FLUID  689 

The  fluid  is  best  fixed  and  imbedded,  and  then  stained  by  the 
Alzheimer  method.  This  is  the  most  complete  and  satisfactory 
method  thus  far  devised,  as  it  permits  of  the  counting  and  study  of  all 
the  cells.  The  Fuchs-Rosenthal  counting  chamber,  however,  is  that 
used  in  the  greater  number  of  instances.  Its  results  are  more  quickly 
obtained,  although  they  lack  the  finality  of  the  Alzheimer  method. 

A  normal  cerebrospinal  fluid  is  a  clear  liquid.  It  has  a  specific 
gravity  of  1006,  a  slightly  alkaline  reaction,  and  is  almost  free  from 
cellular  constituents — 1  to  5  lymphocytes  per  cubic  millimeter,  as 
estimated  by  the  Fuchs-Rosenthal  method,  may  be  considered  normal.^ 

Changes  in  Cerebrospinal  Fluid. — In  cerebrospinal  s^-philis,  in  tabes, 
and  m  general  paresis  there  is  usually  an  increase  in  the  number  of 
Ij-mphocytes.  They  may  run  up  into  the  hundreds,  especially  in 
some  cases  of  acute  meningomyelitis;  also  in  some  fulminating  cases 
of  paresis.  In  fact,  most  meningitic  processes  are  accompanied  by 
increase  in  cellular  content.  Polynuclear  cells  are  frequent  in  the 
acute  types,  sometimes  tuberculous  meningitis  excepted,  and  as  the 
process  tends  to  chronicity  there  is  a  tendency  to  lymphocytosis  and 
loss  of  other  cell  t^-pes.  The  pleocytosis  of  s^-philis  has  usually  been 
attributed  to  a  meningitic  process.  It  has  been  suggested  as  due  to  a 
periarteritis  as  well  (Szecsi). 

Certain  general  variations  may  be  recorded.  The  earlier  students 
were  more  didactic  in  their  statements  concerning  specific  differences 
in  the  number  of  cells  as  distinguishing  cerebrospinal  syphilis,  tabes, 
and  paresis.  Further  extension  of  the  studies  shows  them  to  have 
been  in  part  unwarranted.  Thus  it  has  been  said  that  the  lower 
numbers  point  to  cerebrospinal  syphilis,  the  higher  nmnber  to  tabes, 
and  the  highest  to  paresis.  This  may  at  times  be  so,  but  it  is  not  an 
absolute  rule  by  any  means.  The  number  of  cells  seems  to  bear  a  more 
definite  relation  to  the  activity  of  the  underlying  irritation  or  inflam- 
matory process  than  to  its  kind.  Thus  a  stationary  tabes  may  show 
few  cells,  also  a  paresis  in  remission,  whereas  an  acute  cerebrospinal 
syphilis  or  an  acute  meningomyelitis  may  show  many  cells. 

A  fluid  rich  in  polymorphous  cells  is  indicative  of  a  very  active 
process,  syphilitic  or  othen\'ise. 

In  neurosy})hilis  in  general,  in  tabes,  and  paresis  in  particular,  it  is  of 
importance  to  note  that  a  pleocytosis,  oftentimes  of  very  marked  grade, 
may  antedate  all  neurological  symptoms  of  the  after-coming  disorder. 
This  has  been  shown  repeatedly  by  Sicard,  Ravaut,  Fildes,  Cornaz 
and  many  others.  This  preparetic  period  has  been  diagnosticated  as 
long  as  two  \'ears  before  the  onset  of  the  disease.  This  point  is  well  to 
bear  in  mind  when  one's  advice  is  asked  as  to  the  advisability  of  mar- 
riage of  syphilitics  in  the  forties.  It  is  not  at  all  an  infrequent  experi- 
ence to  find  the  outbreak  of  paresis  occurring  in  men  of  from  forty  to 
forty-fi\e,  who  have  been  from  one  to  three  years  married,  and  many 
have  waited  this  long,  feeling  that  because  of  an  early  syphilis  it  were 
wiser  to  defer  marriage  until  a  safe  period.    A  return  will  be  made  to 

•  See  Thomsen,  Hill,  Halliburton:     Proc.  Roy.  See,  vol.  Ixiv,  for  normal  fluid, 
44 


690  NEUROSYPHILIS 

the  prophylactic  features  later  m  the  more  detailed  discussion  of  the 
therapy. 

How  soon  after  infection  by  syphilis  may  lymphocytosis  appear  in 
the  cerebrospinal  fluid?  Varying  answers  are  available,  but  Ravaut 
has  reported  its  presence  at  least  two  months  after  infection .  In  one 
patient  of  Fildes  a  positive  Wassermann,  1000  cells  and  the  spirochete 
were  found  in  the  cerebrospinal  fluid  twenty  weeks  after  infection 
with  no  other  symptoms  of  neurosyphilis.  This  author  reports  a 
series  of  624  unselected  cases  of  s^-philis  in  all  stages  of  the  disease. 

Eighteen  per  cent,  of  these  had  10  cells  or  over  per  c.mm.  and  12 
per  cent,  had  from  5  to  9  cells  per  c.mm.  In  80  per  cent,  of  individuals 
who  had  abnormal  cerebrospinal  fluid  there  was  no  clinical  sign  nor 
symptom  of  nervous  disease,  although  29  patients  had  over  100  cells 
per  c.mm.,  9  had  over  300  cells  per  c.mm.,  and  3  had  1000  cells  or  over 
per  c.mm.  Many  of  the  other  20  per  cent,  had  symptoms  so  slight  that 
they  were  of  uncertain  value  in  pointing  one  to  neurosyphilis. 

An  interesting  study  by  Cornaz^  is  worth  quoting  at  length  in  this 
connection.  The  figures  are  based  on  an  investigation  of  about  354 
cases.  Cornaz  accepts  9  lymphocytes  per  c.mm.  as  the  upper  limit  of 
the  normal  number. 

1.  In  yrimary  syphilis,  hard  chancres  with  no  secondary  manifes- 
tations, there  were  76,  all  punctured  before  beginning  treatment.  In 
24  patients  the  puncture  was  made  at  a  time  when  the  Wassermann 
reaction  in  the  blood  was  still  negative.  The  average  lymphocytosis 
in  these  cases  was  only  2.4;  it  was  increased  only  twice.  Of  the  52 
punctures  made  when  the  blood  Wassermann  was  already  positive,  the 
average  liyinphocytosis  was  6.1  it  was  increased  in  35  per  cent,  of 
the  cases.    Pleocytosis  rising  to  34  was  found  once. 

Wassermann  reaction  in  the  fluid  was  tested  for  59  times;  58  times 
it  was  negative  and  only  once  positive  with  a  patient  whose  blood  was 
equally  positive  and  who  presented  a  lymphocytosis  of  9. 

The  albumin,  precipitated  by  nitric  acid  and  measured  approxi- 
mately by  means  of  the  scale  of  Bloch,  remained  generally  within 
normal  limits,  0.2  or  0.3  per  thousand.  It  was  plainly  increased  (0.7 
per  cent.)  in  only  two  instances.  In  one  of  these  cases  the  lympho- 
cytosis was  1  and  in  the  other  8;  there  was  therefore  no  parallelism 
between  content  of  albumin  in  the  fluid  and  the  number  of  lympho- 
cytes. Nonne's  reaction,  disclosing  the  presence  of  globulins,  is,  so  to 
speak,  always  negative  at  the  beginning  of  the  luetic  infection. 

2.  Roseola  (with  or  without  persistance  of  chancre). — 84  cases. 
These  were  also  patients  punctured  before  treatment.  The  Wasser- 
mann was  positive  in  the  blood  every  time.  The  average  lymphocytosis 
was  8.2  per  c.mm.;  it  was  increased  in  40  per  cent,  of  the  cases.  The 
Wassermann  reaction  was  tested  for  in  the  fluid  71  times;  ID  times  it 
was  positive.  For  these  10  cases  the  average  lymphocytosis  was  17.1. 
In  the  61  fluids  in  which  the  Wassermann  reaction  was  negative  the 
lymphocytosis  had  an  average  of  only  5.6.    There  exists  a  parallelism 

1  Cornaz,  G. :  Study  of  Cerebrospinal  Fluid  in  Different  Periods  of  Syphilis,  Jour. 
Nerv.  and  Ment.  Dis.,  April,  1919. 


CEREBROSPINAL  FLUID  691 

between  the  two  phenomena  in  the  sense  that,  when  the  reaction  of 
fixation  of  complement  is  positive  in  a  fluid,  that  fluid  presents  most 
often  a  lymphocytosis  clearly  augmented.  The  greatest  pleocytosis 
was  67,  observed  in  a  fluid  in  which  the  Wassermann  was  positive. 
The  albumin  was  increased  in  45  per  cent,  of  the  cases.  As  a  rule, 
if  the  albumin  is  abundant  there  is  also  augmentation  in  the  number 
of  hTnphocytes.  The  ^Yassermann  in  the  fluid  may  be  positive  with  a 
normal  quantity  of  albumin. 

3.  Mucous  plaques  (without  exanthematous  lesions). — Twenty-five 
patients  punctured.  They  all  presented  only  mucous  plaques  and  not 
large  condylomata  nor  remains  of  roseola.  The  blood  Wassermann 
w'as  positive  24  tim.es  (96  per  cent.).  The  lymphocytosis  w^as  increased 
8  times  (32  per  cent,  of  the  fluids) ;  this  was  an  average  of  5,  therefore 
inferior  to  the  degree  attained  in  the  roseola.  The  fluid  Wassermann 
was  positive  only  once  (4  per  cent.);  in  this  case  the  lymphocytosis 
was  4.    Increase  of  albumin  was  observed  in  30  per  cent. 

4.  Large  Condylomata. — ^Thirty  patients  presented  neither  roseola 
nor  mucous  plaques.  Blood  Wassermann  was  positive  in  100  per  cent, 
of  the  cases.  Lymphocytosis  was  increased  in  27  per  cent,  of  the  cases 
with  an  average  of  5.1.  Fluid  Wassermann  was  positive  four  times 
(13  per  cent.).  In  one  instance  a  negative  Wassermann  had  become 
positive  when  a  second  puncture  was  made  after  a  complete  treatment 
with  neosalvarsan.  The  albumin  content  averaged  0.5  per  cent., 
increased  in  30  per  cent,  of  the  cases. 

5.  Latent  Syphilis. — This  treats  of  long-standing  syphilis,  65  cases, 
already  treated  previously  or  of  patients  in  whom  the  lues  was  inci- 
dentally discovered  without  their  presenting  other  signs  of  neuro- 
syphilis. In  those  of  them  in  whom  the  blood  Wassermann  was 
positive  (70  per  cent.),  the  lymphocytosis,  increased  in  40  per  cent, 
of  the  cases,  showed  an  average  of  8;  for  the  patients  in  whom  the 
blood  Wassermann  was  negative  the  average  was  only  4.7  (increase 
in  30  per  cent,  of  the  cases).  Fluid  Wassermann  was  positive  5  times 
in  64  examinations  (7.8  per  cent.).  In  one  instance  we  found  the 
Wassermann  negati^'e  in  the  blood  and  positive  in  the  fluid.  This  was 
in  a  longstanding  syphilitic  treated  on  several  occasions.  The  albumin, 
increased  in  35  per  cent,  of  the  cases  showed  an  average  of  ().(>  per  cent. 

6.  Hereditary  Lues. — Only  6  observations.  One  small  patient 
presented  hydrocephalus  with  idiocy.  His  lymphocytosis  was  67. 
If  this  special  case  be  excluded  there  was  for  the  5  others  a  normal 
lymphocytosis  averaging  3.9.  The  blood  Wassermann  was  positive 
in  100  per  cent,  of  the  cases,  the  fluid  negative  6  times  at  the  first 
puncture.  In  one  instance  it  had  become  positive  at  a  second  puncture 
made  after  treatment  the  lymphocytosis  remaining  normal. 

7.  Tertiary  Syjjhilis  (gummata  and  ulcerations). — F'orty-five 
patients  were  punctured.  The  blood  Wassermann  was  positive  for 
77  per  cent,  of  the  cases.  Lymphocytosis,  increased  in  37  per  cent, 
of  the  cases,  averaged  7.1.  It  was  evidently  the  same  whether  the 
blood  Wassermann  was  positive  or  negative.  The  fluid  Wassermann 
was  positive  3  times  (6.6  per  cent.).    Here  also  he  found  in  one  instance 


692 


NEUROSYPHILIS 


the  Wassermann  reaction  positive  in  the  fluid  and  negative  in  the 
blood.  Increase  in  albumin  was  noted  in  40  per  cent,  of  the  cases;  it  is 
most  often  parallel  with  the  pleocytosis. 

8.  Cerebral  Syphilis  and  Meningomyelitis. — Six  cases.  Blood 
Wassermann  was  positive  4  times  (66  per  cent.).  There  was  marked 
pleocytosis  in  100  per  cent,  of  the  cases.  This  averaged  51  lympho- 
cytes per  c.mm.,  one  case  presenting  an  exceptional  pleocytosis  of 
235,  the  5  others  giving  an  average  of  15  lymphocytes.  The  fluid 
Wassermann  was  positive  every  time,  even  in  the  two  cases  in  which 
the  blood  reaction  was  negative.  The  quantity  of  albumin  surpassed 
0.6  per  cent,  only  in  the  patient  noted  above  who  presented  a  lympho- 
cytosis of  235;  it  reached  in  this  case  1.5  per  cent. 

9.  Tabes. — Punctured  patients  13.  The  blood  Wassermann  was 
positive  9  times  (69  per  cent.).  The  average  lymphocytosis,  which  was 
30,  was  increased  in  11  instances  (85  per  cent.).  It  was  still  normal 
with  the  two  patients  presenting  clear  symptoms  of  tabes  at  the  outset. 
Fluid  Wassermann  was  positive  9  times  at  the  beginning,  but  after 
treatment  by  neosalvarsan  or  novarsenobenzol  it  became  positive 
in  the  13  patients  (100  per  cent.).  Once,  in  a  case  of  demonstrated 
tabes,  the  fluid  Wassermann  had  become  positive  after  treatment 
while  remaining  completely  negative  in  the  blood.  The  average  quan- 
tity of  albumin  is  0.6  per  cent.  Here  its  increase  is  generally  propor- 
tional to  the  lymphocytosis. 

10.  General  Paresis. — Only  4  cases  are  reported. 

The  Wassermann  reaction  was  positive  all  4  times  in  the  blood 
and  the  fluid  (100  per  cent.).  The  average  lymphocytosis  was  28, 
it  was  increased  in  every  case.  The  quantity  of  albumin,  0.8  per  cent, 
on  the  average  was  in  each  instance  equally  augmented.  It  attained 
a  maximum  of  1.5  per  cent,  in  a  case  w^here  the  lymphocytosis  was  12. 
On  the  other  hand  in  another  patient  it  was  only  0.8  per  cent,  for  a 
lymphocytosis  of  51,  which  shows  that  the  parallelism  between  the 
quantity  of  albumin  and  the  number  of  lymphocytes  is  not  by  any 
means  constant. 

The  results  may  be  tabularized  as  follows: 


Average 
Lymphocytosis. 

Lymphocytosis 

increased  in, 

per  cent. 

Wassermann  fluid, 
positive, 
per  cent. 

Blood 

Wassermann, 

positive, 

per  cent. 

Hard  chancre  (Wa.  +) 

6.1 

35 

1.6 

1        68 

Hard  chancre  (Wa.  — ) 

2.4 

8 

0.0 

Roseola         

8.2 

40 

14.0 

100 

Mucous  plaques 

5.0 

32 

4.0 

96 

Large  condylomata 

5.1 

27 

13.0 

100 

Latent  lues  (Wa.  +)      . 

8.0 

40 

8.8 

1        70 

Latent  lues  (Wa.  — )     . 

4.7 

30 

f 

5.0 

5.0 

16.6 

/ 

Hereditary  hies 

3.9 

« 

100 

I 

after  treatment 

J 

Tertiary  lues      .... 

7.1 

37 

6.6 

77 

Meningeal  lues 

51.0 

100 

100.0 

66 

Tabes 

30.1 

85        / 

69  after  neo. 

1        69 

\ 

100.0 

General  paresis 

28.0 

100 

100.0 

100 

CEREBROSPINAL  FLUID  693 

His  observations  permit  him  to  confirm  fully  that  which  Dind^ 
pointed  out  in  1914,  that  is,  how  frequent  and  early  is  the  extension 
of  the  syphilitic  infection  into  the  meninges,  since  in  35  per  cent,  of 
the  cases  of  hard  chancre  the  number  of  leukocytes  was  already 
increased  at  a  time  when  the  cutaneous  or  mucous  manifestations 
were  still  totally  absent  or  at  least  could  not  be  demonstrated  by  the 
means  of  investigation.  It  is  the  lymphocytosis  which  at  first  betrays 
the  modifications  in  the  fluid,  when  the  quantity  of  albumin  is  still 
normal  and  when  the  generalization  of  the  infection  has  not  yet  been 
demonstrated  by  the  Wassermann  reaction.  In  the  exanthematous 
stage  14  per  cent,  of  the  fluids  gave  a  complement  fixation.  This 
figure  is  met  with  again  much  the  same  (13  per  cent.)  in  the  cases  of 
large  condylomata  when,  among  the  patients  showing  mucous  plaques, 
the  fluid  Wassermann  was  positive  only  once  in  25  cases.  This  curious 
difference  is  not  easily  explained.  Are  there  anatomico-histological 
conditions  favoring  the  passage  in  the  fluid  of  bodies  capable  of  becom- 
ing fixed  in  the  antigen  especially  among  the  carriers  of  the  condylo- 
mata or  indeed  do  the  condylomata  result  in  a  more  abundant  for- 
mation of  these  bodies?  The  modifications  of  the  fluid  seem  at  least 
to  be  in  accord  with  the  reactions  of  the  skin  and  it  may  be  asked  if 
the  phenomenon  of  allergic  is  not  present.  This  conclusion  would 
support  the  idea  which  Dind  has  been  one  of  the  first  to  defend,  that 
allergic  is  an  essentially  cutaneous  phenomenon.  The  condylomata, 
cutaneous  lesions,  would  provoke  a  violent  reaction  of  the  organism 
when  the  lesions  of  the  mucous  membrane  alone  would  not  excite  the 
individual  to  defense  in  the  same  degree. 

As  soon  as  the  syphilis  localizes  itself  in  the  central  nervous  system, 
the  cerebrospinal  fluid  constanth'  presents  important  modifications 
and  it  is  of  great  value  in  investigation  for  fixing  the  diagnosis  or  for 
controlling  the  course  of  treatment.  The  Wassermann  reaction 
should  be  specially  made  in  these  cases  for  it  is  relatively  frequent  that 
it  is  positive  in  the  fluid  and  negative  in  the  blood.  The  presence  of 
l}-mphocytes  almost  invariably  speaks  for  the  beginning  of  neuros^-philis. 

With  active  therapy  the  pleocytosis  is  apt  to  disappear.  Are  those 
patients  who  retain  their  lymphocytosis  on  the  way  to  tabes  or  paresis? 
This  is  an  important  problem  which  has.  been  answered  yes  and  no. 
Its  definite  answer  is  yet  to  come,  as  the  necessary  time  for  deter- 
mining the  full  significance  of  the  results  of  cytological  study  of  the 
fluid  has  not  yet  elapsed. 

From  the  therapeutic  point  of  view  then  the  whole  subject  of  pleo- 
cytosis of  the  cerebrospinal  fluid  is  full  of  significance,  and  in  need 
of  constant  attention.  In  this  connection  it  should  again  be  empha- 
sized that  lymphocytosis  alone  does  not  mean  syphilis  alone.  It  can 
be  said  that  absence  of  lymphocytes  at  least  negatives  tabes  and 
paresis — to  this  generalization  there  are  but  few  recorded  negative 
observations.^ 

'  Revue  d'Urologie,  1914. 

*Klieneberger:  Archiv  f.  Psychiat.,  1911.  Foster,  Lewandowsky :  Handbuch  der 
Neurologie. 


694  NEUROSYPHILIS 

Lymphocytosis  is  not  limited  to  neurosyphilis,  however.  It  is 
marked  in  sleeping  sickness,  an  allied  disease  (Spielmeyer,  Mott).  It 
is  often  also  high  in  tuberculous  meningitis,  but  is  here  usually  compli- 
cated by  the  presence  of  polymorphonuclear  leukocytes.  In  a  few 
cases  of  multiple  sclerosis  lymphocytes  have  been  found.  In  the 
acute  stages  of  poliomyelitis  lymphocytes  may  be  found,  also  in 
epidemic  cerebrospinal  meningitis  and  in  herpes  zoster.^ 

4.  Chemical  Examination. — Here  the  presence  of  a  reducing  agent 
(Fehling's)  and  of  globulin  is  to  be  estimated.  Most  fluids  contain 
the  former.  When  present  in  large  quantities  it  may  point  to  a  tuber- 
culous meningitic  process. 

Increased  globulin  content  is  a  characteristic  feature  of  paresis. 
In  tabes  increased  globulin  is  the  rule,  as  is  also  the  case  in  cerebro- 
spinal sj^hilis,  but  to  a  less  extent.  Markedl}'  increased  globulin 
content  is  not  infrequent  in  spinal  cord  tumors,  gummatous  or  non- 
syphilitic.  Increased  globulin  is  also  a  feature  of  the  acute  stage  of 
thrombotic  softening  of  arteriosclerosis  when  the  softened  area  touches 
the  meningeal  sac.  The  globulin  reaction  is  apt  to  run  along  with  the 
lymphocytosis.  It  has  no  apparent  alliances  with  the  findings  of  the 
Wassermann  test. 

Summary  of  Laboratory  Findings. — Four  Eeactions. — Before  passing 
to  the  diagnostic  significance  of  the  clinical  examination  of  the  eye 
refiexes  a  word  should  be  said  relative  to  the  value  of  these  "four 
reactions,"  as  Nonne  has  called  them.  It  has  been  said  that,  taken 
alone  they  may  mean  nothing  positive,  so  far  as  a  differential  of  the 
different  trends  in  neurosyphilis  is  concerned,  but  when  read  together 
they  afford  important  guides  to  diagnosis,  to  localization,  and  to 
treatment. 

Expressed  schematically,  which  schemes,  as  he  well  remarks,  are 
not  products  of  nature  but  of  man,  the  following  results  of  the  four 
reactions  are  to  be  expected.     (I)  blood  and  (II)  fluid. 
I.  Blood  examination. 
Wassermann  reaction. 

(a)  Positive.  Is  characteristic  of  syphilis  with  few  exceptions 
(already  noted).  A  positive  Wassermann  of  the  blood 
serum  says  nothing  further  than  that  the  individual  has 
come  in  some  manner  in  contact  with  syphilis,  either 
through  heredity  or  by  infection.  It  does  not  say  that 
the  disease  from  which  he  suffers  is  due  to  syphilis. 
(6)  Negative.  Is  differentially  diagnostic,  against  paresis, 
since  it  is  only  very  rarely  that  the  blood  in  paresis  gives 
a  negative  reaction. 
II.  Study  of  cerebrospinal  fluid. 

(a)  Normal  fluid.  Pressure  90  to  130  mm.  water.  Globulin 
reaction  negative — not  over  5  or  6  cells  to  c.mm.  (Fuchs- 
Rosenthal). 

^  See  study  by  Schaller,  loc.  cit.,  on  Herpes  Zoster  and  the  Cerebrospinal  Fluid. 


SUMMARY  OF  LABORATORY  TESTS  695 

(6)  Pathological  fluids. 

1.  Increased  pressure — over  15  c.mm.  water. 

2.  Positive  phase  I.     Globulin  reaction. 

3.  Increased    cell    count.       (These  three  symptoms,  in 

coordination  or  alone,  indicate  the  presence  of    an 
organic  nervous  disorder,  s\'philitic  or  non-syphilitic.) 
(c)  If  the  disease  of  the  nervous  system  is  s^'philitic,  then  the 
Wassermann  test  of  the  fluid  will  show.     If  the  Wasser- 
mann  reaction  (original  method — 0.2  c.c.  of  the  fluid)  is 
positive,  there  is  great  probability  that  the  patient  is  a 
paretic,  or  a  taboparetic,  much  less  often  a  cerebrospinal 
syphilitic,  or  a  pure  tabetic.    In  nearly  all  cases  of  cerebro- 
spinal  syphilis   and   of  tabes   the  Wassermann    reaction 
becomes  positive  by  using  0.4  to  1  c.c.  of  fluid. 
Nonne's  tj^iical  findings  are  as  follows 
I.  Paresis  or  taboparesis. 

1.  Wassermann   reaction    in    blood   positive    (100  per  cent.). 

Pressure  increased. 

2.  Phase  I,  globulin  reaction  positive  (95  to  100  per  cent.). 

3.  Lymphocytosis  (95  per  cent.). 

4.  Wassermann  in  fluid. 

(a)  Positive — about    85    to    90    per    cent,  with  original 

method  and  0.2  c.c.  fluid. 
(6)  Positive   in   100  per  cent.,  with   larger  quantities  of 
fluid. 
IT.  Tabes  without  paresis. 

1.  Wassermann  reaction  in  blood  positive  (60  to  70  per  cent.). 

Pressure  usually  increased. 

2.  Phase  I,  reaction  globulin  and  positive  (90  per  cent.). 

3.  Lymphocytosis  positive  (90  per  cent.). 

4.  Wassermann  in  fluid. 

(a)  Original  method,  0.2  c.c.  positive  (5  to  10  per  cent.). 
(6)  Larger  quantities    (100   per   cent.). 
III.  Cerebrospinal  syphilis. 

1.  Wassermann  ^-eaction  in  blood  positive  (80  to  90  per  cent.). 

Pressure  frequently  increased. 

2.  Phase  I,  reaction  usually  positive,  exceptionally  negative. 

3.  Lymphocytosis  nearly  always  positive. 

4.  Wassermann  in  fluid. 

(a)  Original  methods  (0.2  c.c.)  positive  in  about  10  per 

cent. 

(6)  Larger  quantities   nearly   always    positive    (of   value 

in    diagnosis    of    multiple    sclerosis,    cerebral    and 

spinal  tumor). 

These  results  of  Nonne's  summarize  fairly  accu^-ately  the  present-day 

attitude  on  the  value  of  the  four  reactions.     The  full  significance 

of  the  findings  can  be  gained  only  by  reference  to  the  originals.    This 

field  of  work  is  rapidly  expanding,  and  that  which  now  appears  on  the 


696  NEUROSYPHILIS 

frontiers  of  our  knowledge  will  undoubtedly  be  much  modified  by  the 
rapidly  advancing  army  of  investigators. 

Additional  Tests. — These  additional  tests  are  valuable  because 
syphilis  may  be  present  with  a  negative  Wassermann.  These  cases 
of  so-called  latent  s,>7)hilis  with  negative  Wassermann  occur  in  about 
35  per  cent,  of  cases  with  no  active  signs  of  the  disease  but  with  a  clear 
history  of  infection. 

The  Liietin  Test. — This  is  a  cutaneous  test  with  a  suspension  of 
killed  spirochete  cultures  as  prepared  by  Noguchi,  known  as  luetin.  A 
positive  reaction  appears  in  the  form  of  a  red  papule  with  indurated 
areola  in  five  or  six  days.  This  test  is  especially  valuable  in  tertiary 
and  latent  syphilis,  conditions  in  which  the  Wassermann  reaction  is 
sometimes  negative.  It  is  valuable  also  in  differentiating  from  other 
conditions  which  might  give  a  positive  Wassermann,  for  this  test 
appears  to  be  a  specific  for  s^q^hilis. 

Provocative  Wassermann. — This  test,  like  the  luetin  test,  is  valuable 
where  s}7)hilis  is  suspected,  but  the  Wassermann  is  negative.  It  is 
dependent  upon  the  fact  that  a  negative  W^assermann  may  be  changed 
to  positive  after  an  injection  of  salvarsan.  This  change  may  occur 
quite  promptly  or  only  after  several  days.  Nichols  recommends 
making  the  Wassermann  twenty-four  and  forty-eight  hours  after  an 
injection  (0.4  gram  salvarsan  or  0.6  gram  neosalvarsan)  and  again 
after  seven  and  fourteen  days. 

Langes  Colloidal  Gold  Test. — This  test  is  dependent  upon  a  color 
reaction  which  makes  it  very  delicate.  It  has  an  added  value  because 
a  single  test,  depending  upon  the  degree  of  discoloration,  tends  to 
show  whether  one  is  dealing  with  a  frank  luetic  or  a  metahietic  proc- 
ess. For  this  reason  it  is  to  some  extent  replacing  the  Wassermann 
test.  A  t^-pical  reaction  in  the  luetic  zone  could  be  3321122200,  a 
t;^^ical  reaction  in  the  paretic  (metaluetic)  zone  would  be  5555432000. 
For  a  description  of  the  technic  the  student  is  referred  to  special  w^orks. 
A  number  of  other  tests  have  recently  appeared,  but  those  mentioned 
are  the  most  important. 

5.  The  Eye  Reflexes. — In  the  diagnosis  of  syphilis  of  the  nervous 
system  the  neurological  examination  of  the  eye  reflexes  is  of  para- 
mount value.  Here  irregularity  in  the  size  of  the  pupils,  irregularity 
in  the  pupillary  margms,  the  impairment  of  the  consensual  light 
reflex,  the  slowing  in  reaction  to  light,  fatigability  of  the  light  reflex, 
alterations  in  response  to  accommodation  efforts,  the  full  develop- 
ment of  the  Argyll-Robertson  sxTidrome,  are  all  to  be  considered. 
These,  one  or  all,  constitute  extremely  delicate  and  valuable  criteria 
for  the  clinical  appraisement  of  syphilis  of  the  central  nervous  system. 

A  fully  developed  Argyll-Robertson  syndrome — loss  of  direct 
pupillary  light  reflex,  with  free  and  ample  response  to  accommodation 
reflexes  in  one  or  both  eyes — represents  for  the  most  part  a  fairly  posi- 
tive criterion  of  syphilis  of  the  nervous  system. 

There  are  many  who  believe  that  this  syndrome  aft'ords  positive 
proof  of  nervous  syphilis.  This  we  do  not  believe  to  be  true,  not 
only  upon  clinical,  but  also  upon  anatomical  grounds.    Clinically  the 


EYE  REFLEXES  697 

Argyll-Robertson  s;yTidrome  has  been  observed  following  direct  injury 
of  the  midbrain  structures  (pistol  shot — Guillain  and  a  group  of  cases 
seen  in  war  practice),  it  has  been  observed  in  poisonings  other  than 
those  of  syphilis,  alcohol  (in  Korsakov,  Wernicke's  polioencephalitis 
superior).  It  may  result  from  pressures  (tumors  of  third  ventricle, 
pineal),  from  poliomyelitis,  from  trspanosomiasis,  from  orbital  trauma 
(Velter,  Ohm),  and  from  other  rare  anomalous  disorders.  Anatomically 
the  s^rTidrome  represents  implication  of  certain  reflex  paths  in  certain 
peculiar  combinations,  and  such  implications  and  combinations  are 
purely  fortuitous  and  accidental,  i.  e.,  so  far  as  nosology  is  concerned. 

As  a  matter  of  fact,  however,  these  combinations  rarely  take  place 
except  as  a  result  of  the  widespread  changes  induced  by  one  particular 
type  of  poisoning — the  sj-philitic  virus^ — so  that  for  clinical  purposes 
the  presence  of  a  permanent,  bilateral,  Argyll-Robertson  s^^ldrome  is 
nearly  enough  positive  for  syphilis  to  permit  one  to  assume  its  presence, 
and  to  therapeutically  guide  one's  self  accordmgly  (Rose.)^ 

Testing  for  the  Argyll-Robertson  sjTidrome,  however,  is  not  as  simple 
as  it  is  usually  supposed.  The  ordinary  devices  of  having  a  patient 
face  the  window,  and  then  cover  and  uncover  the  opened  eyes  with 
the  hand ;  focussing  the  eye  upon  a  distant  object,  and  then  upon  the 
finger  in  close  proximity  to  the  nose;  these  tests  for  the  most  part 
are  entirely  too  crude  to  permit  one  to  judge  with  certainty  concerning 
the  Argyll-Robertson  s^iidrome.  Such  methods  may  suffice  for  the 
majority  of  instances,  but  in  no  field  of  neurology  is  it  more  desirable 
to  utilize  the  most  accurate  methods  than  in  dealing  with  the  vexed 
question  of  sA^jhilis  of  the  nervous  system. 

Few  clinicians  can  follow  out  the  intricacies  of  Weiler's  complicated 
methods,  but  they  may  be  necessary  in  certain  doubtful  cases. 

Repeated  examinations,  under  carefully  regulated  supervision,  in 
the  daylight,  and  in  the  dark-room,  are  therefore  desirable  when  test- 
ing for  anomalies  in  the  pupillary  reflexes.  Careful  checking  of  the 
results  obtained  by  the  small  pocket  electric  lamps  is  imperative,  as 
occasionally  they  give  anomalous  results  and  may  lead  to  serious  error 
(Oppenheim).  The  presence  of  a  fully  developed  Argyll-Robertson 
syndrome  may  be  said  to  be  a  highly  probable  positive  proof  of  s^-philis 
of  the  central  nervous  system,  particularly  of  the  cerebral  and  mid- 
brain neurons.  Its  absence,  however,  by  no  means  negatives  s^-philis 
of  the  nervous  system,  since  cerebral  gummata,  cerebral  syphilis, 
paresis,  tabes,  syphilitic  meningitis,  meningomyelitis,  cord  gummata, 
syphilitic  radiculitis,  syphilitic  neuritis,  all  may  be  present  without 
any  anomalies  in  the  pupillary  reflexes.  It  has  already  been  pointed 
out  that  the  laboratory  findings  of  paresis  and  of  tabes  have  antedated 
the  development  of  neurological  signs  by  at  least  a  couple  of  years, 
and,  moreover,  it  has  been  emphasized  that  pathological  alterations 
in  these  pupillary  reflexes  are  after  all  only  chance  happenings,  when 
certain  reflex  arcs  in  certain  combinations  are  caught  in  the  mesh  of 
the  infiltrative,  syphilitic  alterations.    The  chance  is  a  large  one,  it 

'Mayer:     Jour.  f.  Psych,  u.  Neur.,  1915,  xxi,  221. 


698  NEUROSYPHILIS 

is  true,  but  still  it  is  purely  a  statistical  matter  of  what  has  happened. 
One  word  may  be  added:  in  many  cases  of  cerebrospinal  syphilis 
one  can  gauge  the  progressive  amelioration  of  the  patient's  condition 
by  the  gradual  return  of  the  anomalous  pupillary  reflexes  to  a  more 
normal  condition.  Thus,  an  absolute  Argyll-Robertson  syndrome 
may  become  a  relative  one.  A  unilateral  Argyll-Robertson  may  be 
lost;  slowly  reacting  pupils  may  show  prompt  reactions;  irregularities 
in  size  may  disappear;  rapid  fatigability  may  let  up;  a  consensual 
light  loss,  often  the  first  anomaly  to  appear  in  cerebrospinal  sj^hilis, 
will  clear  up;  irregularities  in  the  pupillary  outlines  will  give  way  to 
regular  outlines,  etc. 

An  inability  to  modify  pathological  pupillary  reactions  by  ample 
syphilitic  therapy  argues  in  part  for  the  chronicity  of  the  process,  or 
the  inefRcacy  of  treatment.  This  is  not  an  absolute  rule,  however. 
It  may  be  possible  for  a  syphilitic  process  to  permanently  destroy  por- 
tions of  the  pupillary  reflex  paths,  and  then  be  completely  and  perma- 
nently arrested.    The  pupillary  reflexes,  however,  remain  impaired. 

In  relation  to  this  question  of  the  pupillary  reflexes  and  anti-syphi- 
litic treatment  the  problem  arises — What  is  the  probable  outcome  of  a 
syphilitic  process  which  comparatively  early  in  its  course  has  destroyed 
the  pupillary  reflex  paths?  Can  it  be  decided,  say  after  two,  five,  or 
ten  years,  during  which  time  there  has  existed  an  Argyll-Robertson 
syndrome  and  little  else,  that  the  disease  has  been  completely  and 
permanently  arrested?  Since  the  rapid  extension  of  knowledge 
concerning  the  cerebrospinal  fluid  this  question  can  be  answered  better 
at  the  present  time  than  ever  before. 

If  the  signs  of  a  meningitis — acute,  subacute,  or  chronic,  i.  e., 
increase  in  cellular  elements,  increase  in  globulin,  and  positive  fluid 
Wassermann,  remain  absent,  then  it  can  be  taken  as  highly  probable 
that  the  disease  process  has  been  arrested.  Judging  by  clinical  methods 
alone  a  longstanding  and  unchangeable  Argyll-Robertson  pupil  may 
be  the  only  lesion  in  a  practically  cured  syphilis  of  the  nervous  system. 
It  is,  however,  to  be  realized  that  such  a  pupillary  anomaly  may 
exist  as  long  as  from  twelve  to  sixteen  years  (alone)  and  then  the 
patient  may  develop  a  paresis  or  tabes,  and  occasionally  it  is  found  as 
an  apparent  personal  peculiarity  without  other  significance.  Of 
eleven  personal  observations,  now  extending  over  twenty-four  years, 
only  two  patients  with  longstanding  Argyll-Robertson  pupil  have  not 
developed  further  signs  of  brain  syphilis.  Certain  deep-seated,  chronic, 
syphilitic  arterial  processes,  which  may  lead  to  focalized  lesions,  hemi- 
plegia, aphasia,  etc.,  may,  however,  go  on  for  some  time  without  distinct 
signs  of  meningeal  irritation  with  the  characteristic  cellular  reactions. 

Clinical  Trends. — It  has  already  been  stated  that  the  so-called  classical 
forms  of  neurosyphilis  are  largely  abstractions.  The  pathological 
processes  are  predominantly  either  meningeal,  arterial  or  infiltrative 
in  character  (syphilis  meningo vascularis  of  Head),  or  parenchymatous 
(syphilis  centralis  of  Head)  alone  or  in  combinations,  and  the  clinical 
manifestations  are  extremely  variable,  complex  and  confusing,  depend- 


SYPHILIS  OF  THE  CRANIAL  BONES  699 

ing  upon  the  interactions  of  the  pathological  trends  and  the  localization 
of  the  anatomical  paths  interfered  with. 

Fortunately  for  the  therapy,  the  clinical  type  is  of  secondary  con- 
sideration, yet  there  are  certain  therapeutic  variables  that  render  it 
desirable  that  a  fuller  analysis  of  clinical  forms  should  be  made  than 
would  at  first  sight  seem  advantageous.  For  instance,  it  may  be 
recalled  that  certain  patients  with  meningeal  infiltrations  of  the  base, 
with  or  without  gummatous  nodules,  either  of  the  base  or  of  the 
convexity,  at  times  may  be  indistinguishable  clinically  from  those 
suft'ering  from  a  parenchymatous  or  central  type  of  neurosyphilis. 
A  pessimistic  therapeutic  attitude  relative  to  the  latter  process  would 
therefore  work  greatly  to  the  disadvantage  of  a  patient  with  the  former. 
This  is  one  of  the  gravest  reflections  brought  against  a  laissez  faire 
handling  of  the  so-called  "hopeless  paretic"  in  institutional  or  in 
private  work.  Many  curable  casss  are  grossly  neglected  because  they 
are  diagnosed  as  "incurable."  Neurosyphilis  meningovascularis  is 
usually  very  amenable  to  treatment.  Often  its  clinical  picture  is  that 
of  a  paresis. 

The  following  clinical  trends  will  be  discussed  here. 

1.  S^-philis  of  the  cranial  bones. 

2.  Syphilis  of  the  basal  meninges. 

3.  Syphilis  of  the  convexity;  epilepsies. 

4.  Cerebral  syphilis — arterial  t\'pes. 

5.  Cerebral  syphilis — parench\Tnatous  or  central  t^pes  (general 
paresis,  taboparesis). 

6.  Syphilitic  psychoses,  acute  and  subacute. 

7.  Tabes. 

8.  Syphilitic  spinal  meningitis;  meningomyelitis;  myelitis;  syphilitic 
radiculoneuritis,  and  related  neurosyphilitic  syndromes. 

9.  Hereditary  neurosyphilis. 

For  a  fuller  consideration  of  the  protean  variations  the  monographs 
of  Riimpf,  Xonne,  ]\Iott,  Plant,  Oppenheim,  Forster,  Schaffer,  Fournier 
and  Southard  and  Solomon,  should  be  consulted. 

1.  Syphilis  of  the  Cranial  Bones  Causing  Nervous  Symptoms. ^ — 
These  were  recognized  as  early  as  the  end  of  the  sixteenth  century. 
Syphilis  of  the  cranial  bones  shows  itself  practically  in  the  form  of 
gummata.  Caries  of  the  cranial  bones  alone,  while  known,  rarely 
gives  rise  to  nervous  symptoms,  headache  excepted.  These  gummata 
may  be  circumscribed,  in  which  case,  if  large  enough,  they  give  rise 
to  the  symptoms  of  a  tumor  of  the  brain,  which  signs  are  largely  deter- 
mined by  the  precise  location  of  the  gummata.  These  circumscribed 
gummata  may  attain  enormous  proportions.  A  personal  observation 
(J.)  recalls  a  gumma  of  the  left  frontal  region  (the  size  of  a  tennis  ball) 
which  originated  in  the  bone  dura  and  protruded  into  the  right  frontal 
lobe.    Similar  gummata  are  not  infrequent. 

Cranial  bone  gummata — usually  involving  the  dura  as  well — are 

'Karpas  and  Gregory:     Jour.  Nerv.  and  Ment.  Dis.,  1914. 


700 


NEUROSYPHILIS 


more  frequently  flattened  and  spreading.  Here  the  symptoms  of  brain 
tumor  are  usually  present.  Headache,  nausea,  vomiting,  sleepless- 
ness, are  among  the  general  symptoms,  while  localizing  signs  in  great 
variety,  depending  upon  the  situation  of  the  gummatous  masses 
are  present.  Epileptiform  convulsions,  lasting  for  years,  may  be 
the  sole  signs  of  such  gummatous  formations,  occupying  or  due  to 
pressure  upon  the  motor  areas.  Such  patients  are  often  mistakenly 
treated  as  epileptics,  and  the  monographs  of  Mott,  Oppenheim,  Rumpf , 
Nonne,  in  recent  years,  are  replete  with  autopsy  records  of  such  cases. 
Monoplegias  of  various  sorts  result  from  such,  as  also  aphasias,  word- 
blindness,  and  various  cranial  nerve  palsies. 


Fig.  379. — Gumma  of  brain. 

In  addition  various  forms  of  periostitis  and  of  osteitis  occur.  This 
latter  may  lead  to  exostoses  or  to  osteoporosis.  In  connection  with 
syphilis  of  the  vertebrae  it  should  be  remembered  that  the  processes 
are  more  often  involved  in  distinction  from  tuberculosis  which  more 
frequently  involves  the  body.  Syphilis,  too,  more  often  affects  the 
cervical,  while  tuberculosis  more  often  the  dorsal  vertebrae. 

Rarer  cases  of  cranial  bone  caries  of  the  base  (sphenoid)  compli- 
cated often  by  caries  of  the  upper  vertebrae,  are  also  known.  Petren 
has  studied  these  in  detail,  and  has  shown  the  value  of  .r-ray  exami- 
nations in  their  diagnosis. 

2.  Syphilitic  Meningitis  of  the  Base. — This  is  perhaps  the  most  com- 
mon trend  in  nem-osyphilis.  A  widespread  syndromy  may  develop 
within  three  months  after  infection,  but  the  majority  of  cases  are  of 
slower  evolution,  one  to  five  years.     In  some  the  symptoms  have 


SYPHILITIC  MENINGITIS  OF   THE  BASE  701 

appeared  as  late  as  twenty-five  years  after  infection.  Its  most  fre- 
quent site,  at  the  beginning,  is  in  and  about  the  interpeduncular  space, 
thus  almost  invariably  involving  the  optic  chiasm.  From  here  it  tends 
to  spread  in  all  directions,  pressing  into  the  sulci,  thickening  the 
meninges,  by  infiltration,  by  arterial  disease,  or  by  gummatous  growth. 
Usually  all  three  types  of  pathological  alteration  are  found.  The 
gummatous  masses  not  infrequently  invade  the  brain  structures  as 
well,  grow  about  the  emerging  or  entering  cranial  nerves,  and  even 
involve  the  bones  of  the  skull,  and  the  upper  cervical  vertebrae.  Thus, 
spinal  meningeal  infiltrations  almost  invariably  accompany  this  basal 
syphilitic  meningitis.  Whereas,  the  diffuse,  conglomerate  types  are 
more  frequent,  isolated  vascular  disease,  circumscribed  gummata,  or 
other  simpler  manifestations  of  the  disease  may  occur,  in  which  latter 
case  the  syndromes  are  apt  to  be  simple. 

The  clinical  course  of  the  more  frequent  types  of  basal  syphilitic 
meningitis  often  resembles  general  paresis,  especially  in  the  beginning, 
but  the  gradual  extension  of  the  infiltrating  or  gummatous  develop- 
ments introduces  variants  which  often  permit  a  differential  diagnosis. 

Headache  is  a  frequent  and  early  sign,  often  preceding  other  symp- 
toms by  weeks,  months,  or  even  years.  It  has  the  frequent  nocturnal 
exacerbations  so  frequently  pictured  as  characteristic  of  syphilitic 
headache.  It  is  described  variously  as  boring,  stabbing,  and  percus- 
sion at  the  base  may  show  tenderness,  though  less  frequently  than  in 
convexity  meningitis.  The  cervical  complications  spoken  of  often 
result  in  stiff ;iess  of  the  neck. 

An  early  implication  of  the  optic  nerve  is  to  be  expected.  It  shows 
itself  (20  per  cent,  to  40  per  cent,  of  the  cases)  either  as  a  pressure 
neuritis  (neurit ic  atrophy)  of  the  nerv'e  in  one  disk,  then  in  the  other, 
later,  if  headache  and  vomiting  or  other  signs  of  intracranial  pressure 
are  prominent,  choked  disk  in  both  eyes  is  apt  to  be  present.  Atrophic 
degeneration  and  optic  neuritis  are  less  often  found.  ]\Iarked  diminu- 
tion in  visual  acuity  may  bepresentwithoutanydiskevidencesof  disease. 

The  third  nerve  is  frequently  and  usually  irregularly  involved.  It 
is  characteristic  of  basal  syphilitic  meningitis  that  successive  branches 
are  implicated.  First  one  eye  may  show  a  ptosis,  then  perhaps  an 
internal  rectus  palsy,  then  the  other  eye  may  show  a  dilated  pupil, 
slow  in  its  reactions  to  light,  then  ptosis  develops  here.  Occasionally 
the  accommodation  reflex  is  lost.  A  series  of  cases  will  show  a  great 
variety  of  oculomotor  palsies.  A  true  Argyll-Robertson  syndrome  is 
not  infrequently  obtained. 

Other  cranial  nerves  are  often  included.  Variations  in  the  corneal 
reflex,  m  the  sensibility,  pain,  anesthesitv  of  the  face,  point  to  a  tri- 
geminal complication.  A  peripheral  facial  palsy  may  be  presented. 
In  some  individuals  the  deeper-lying  cranial  nerves  (IXth,  Xth,  Xlth, 
Xllth)  are  caught  in  the  syphilitic  extension,  with  their  characteristic 
sjTnptcms.  The  cochlear  and  vestibular  nerves  are  frequently 
involved.' 

'  liloyd:  Jour.  Nerv.  and  Mental  Disease,  Aug.,  1919. 


702 


NEUROSYPHILIS 


The  mental  picture  is  usually  very  striking.  It  is  frequently  that  of 
a  slowly  developing  apathy,  or  heaviness  advancing  to  coma,  or  uncon- 
sciousness, with  periods  of  acute  confusion,  possibly  violent  delirium. 
There  is  a  marked  variability  in  these  patients  from  day  to  day  and 
also  considerable  differences  in  different  patients.  Some  patients 
develop  a  sort  of  drunken  delirium;  others  are  heavy  and  stupid  and 
apathetic;  others  are. furiously  violent. 

One  special  feature  is  frequently  met  with.  This  is  a  rapid  altera- 
tion in  the  mental  picture,  when,  after  a  period  of  acute  confusion  or 
deep  coma,  the  patient  becomes  almost  practically  clear  within  a  few 
hours.  This  occurrence  may  even  follow  a  period  of  convulsive 
seizures.    Careful  tests  reveal  an  underlying  series  of  defects  it  is  true. 


Fig.  3b0. — Cerebral  syphilis  ptosis. 
Third  nerve  palsy. 


Fig.    381. — Cerebral   syphilis  with  third 
and  fourth  nerve  palsies.     (Sharp.) 


but  from  a  lay  viewpoint  the  patient  may  appear  to  have  made  a 
complete  recovery  so  far  as  his  psychosis  is  concerned.  Without 
treatment,  however,  the  patient  again  develops  his  apathy,  confusion, 
delirium,  or  coma  and  not  infrequently  dies  in  this  state.  Sometimes 
death  results  by  suicide  as  the  patient  develops,  slowly  or  rapidly, 
a  distinct  depression  with  possibly  persecutory  ideas. 

Thus,  mentally,  the  patients  may  show  the  old-time  rubrics  of  acute 
confusion,  dementia,  mania,  melancholia,  paranoia,  etc.  This  alone 
indicates  the  futility  of  regarding  the  symptom  pictures  which  have 
gone  by  these  names  as  diseases,  some  for  hundreds  of  years.  Happily 
present-day  psychiatry,  largely  under  the  influence  of  Ivraepelin's 
teaching,  recognizes  them  as  only  the  protean  and  kaleidoscopic  pic- 
ture-formation of  not  only  s^^hilis,  but  other  disease  processes  as  well. 


SYPHILITIC  MENINGITIS  OF   THE  CONVEXITY 


703 


Biological  and  cytological  methods  have  permitted  this  definite 
change  in  attitude,  and  have  shown  the  essential  and  close  relationship 
of  many  diverse  neurological  and  psychotic  s^Tld^omes. 

3.  Syphilitic  Meningitis  of  the  Convexity. — This  differs  from  the 
former  only  in  the  trend  of  its  symptoms.  The  pathological  processes 
are  practically  identical.  Many  individuals  show  that  the  process  is 
general,  involving  both  the  base  and  the  convexity;  in  some  instances 
the  pathological  changes  being  more  marked  on  the  convexity  than  on 
the  base. 

Convexity  s\'philis,  like  the  basal  variety,  may  be  a  fairly  localized 
affair,  or  it  may  be  diffuse.  It  may  be  limited  to  the  meninges,  or 
involve  the  bones,  or  the  brain,  or,  as  is  most  usual,  all  three. 


Fig.  382. — Cerebral  syphilis  of  the  coiivexitj-.     Chronic  epileptiform  couvuIsioDs. 

Here,  headache  is  a  prominent  sign.  It  is  paroxysmal,  and  often 
shows  a  nocturnal  increase  in  severity.  Percussion  affords  valuable 
evidence,  as  localized  tenderness  is  very  common. 

Here  the  general  syndrome  of  brain  pressure  is  usually  less  empha- 
sized. Nausea,  vomiting,  giddiness,  may  be  present,  but  are  usually 
late  in  development,  or  more  transitory.  Optic  nerve  changes  are 
less  frequent.  Isolated  symptoms  are  more  prominent.  Epileptiform 
convulsions  indicate  that  the  process  is  in  or  about  the  motor  areas. 
Not  infrequently  the  attacks  are  of  the  Jackson ian  type.  Involve- 
ment of  Broca's  convolutions  produces  temporary  or  more  enduring 
motor  aphasic  attacks.  Sometimes  these  aphasic  attacks  clear  up  in 
a  few  minutes,  an  hour  or  so,  or  a  few  days.    Minor  speech  difficulties 


'04 


NEUROSYPHILIS 


may  only  indicate  the  possibility  that  an  aphasia  might  develop. 
Complete  motor  aphasia  develops  only,  as  a  rule,  with  hemiplegic  or 
monoplegic  accompaniments.    Pseudobulbar  palsy  attacks  indicate  a 


Fig.  383. — Syphilitic  meningitis  with  giant-cell  formation. 


Fig.  384. — Chronic  epileptiform  con\iilsions  due  to  syphilitic  leptomeningitis  and 
syphilitic  vascular  disease, 


VASCULAR  CEREBRAL  SYPHILIS  705 

bilateral  involvement,  probably  both  cortical,  less  frequently  cortical 
on  one  side,  and  subcortical  on  the  opposite  side. 

]\Ionoplegias  of  varying  t^-pes  are  not  infrequent.  Bergmark^  has 
devoted  a  large  monograph  to  their  study.  Sensory  disturbances, 
hemianesthesige,  astereognosis,  haptic  hallucinations  are  met  with. 

With  diffuse  meningo-encephalitic  changes  the  picture  of  general 
paresis  is  assumed,  and  it  is  practically  impossible  to  differentiate  this 
trend  in  the  process.  The  clinical  pictures  may  be  as  various  as  those 
of  paresis.  Possibly  the  only  means  to  distinguish  them  is  that  of  a 
rigid  application  of  the  serological,  cytological  and  chemical  criteria. 
With  these  laboratory  tests  a  practical  insight  may  be  gained  into  the 
more  fundamental  trends  of  the  s^-philitic  process .^ 

4.  Cerebral  Syphilis. — Vascular  Types. — In  considering  the  symp- 
tomatology of  patients  who  are  thought  to  have  cerebral  s^-philis, 
attention  may  again  be  called  to  the  fact  that  there  are  no  dividing 
lines  between  cerebral  syphilis,  basal  meningeal  s\^hilis  or  convexity 
s^'philis.  Rarely  does  one  find  a  pure  basal  or  convexity  meningitis 
without  some  involvement  of  the  cerebral  substance  on  the  one  hand, 
while  conversely  it  is  as  rare  to  find  s^-philitic  processes  strictly  limited 
to  the  cerebral  substance,  and  not  involving  the  meninges.  One  can 
postulate  pure  t^'pes  for  purposes  of  description  only,  but  disease  is 
rarely  a  respector  of  one  cerebral  tissue  more  than  another,  although 
there  may  be  a  tendency  to  greater  meningeal  or  vascular,  or  central 
predominance  in  one  or  another  patient. 

Symptoms. — -It  is  for  this  reason  that  one  is  constantly  reminded 
of  the  multiplicity  of  symptoms  found  in  cerebral  syphilis.  In  this 
connection  it  would  not  be  without  profit  to  glance  for  a  moment  at 
the  diagnosis  of  certain  cases  reported  by  Nonne  in  his  monograph 
several  times  alluded  to.  The  patients  were  illustrations  of  basal, 
or  convexity,  or  encephalic  brain  syphilis,  usually  combmed  forms. 
The  short  descriptive  diagnoses  run  as  follows:  Specific  headache  a 
year  after  infection,  with  secondaries  in  skin  and  mucous  membranes; 
headacl^e  and  pupillary  anomalies;  headache  and  obstinate  vomiting, 
with  tertiary  testicle  signs;  progressive  simple  dementia  cured  by 
treatment;  progressive  dementia  with  defect;  combination  of  con- 
vexity meningitis  and  paresis;  gummatous  meningo-encephalitis  of 
convexity  with  general  symptoms,  choked  disk  and  antisyphilitic 
treatment  unavailing;  surgical  treatment  of  gummata  with  cure; 
Jacksonian  epilepsy;  cortical  epilepsy,  choked  disk,  paresis  of  left 
leg;  cortical  epilepsy,  o])tic  neuritis,  arterial  hemiplegia;  cortical 
hemiepikpsy  and  general  cortical  symptoms;  arterial  hemia])oplexy 
with  hemiepileptic  convulsions;  generalized  epileptic  seizures;  hemi- 
anesthetic  attacks  with  cortical  signs;  uremia,  etc.     Such   illustra- 

»  Brain,  1911. 

*  The  student  should  consult  the  periodical  literature,  for  this  branch  of  study  is 
constantly  evoKdnR  toward  more  and  more  pragmatic  criteria.  See  current  literature 
in  such  neurological  magazines  as  the  Revue  Neurologique,  .Journal  of  Nervous  and 
Mental  Disease,  Ncurologisches  Zentralblatt,  Review  of  Neurology  and  Psychiatry  and 
the  Revista  di  patologia  nervosc  e  mentale. 
45 


706 


NEUROSYPHILIS 


tions  might  be  almost  indefinitely  continued.  They  are  not  the 
exceptions,  they  are  the  rule.  One  is  tempted  to  indulge  in  the  gener- 
alization that  one  hundred  consecutive  patients  with  the  types  of 
cerebral  syphilis  under  consideration  would  show  one  hundred  different 
clinical  syndromes.  Practically  all  of  those  just  enumerated  belong 
to  the  convexity  types  of  cerebral  sj-philis.  Their  enumeration  may 
prove  of  service  in  localizing  the  process. 

A  similar  series  for  the  basal  types  may  be  equally  of  service: 
Gumma  of  right  frontal  lobe;  pressure  neuritis  of  optic  nerve;  choked 
disk  with  general  cerebral  s^Tuptoms;  bilateral  neuritic  optic  atrophy; 


Fig.  385. — Syphilitic  meningitis.     Endarteritis  of  basilar  artery. 

recurrent  optic  atrophy;  hemianopsia;  bitemporal  hemianopsia; 
homonymous  left-sided  hemianopsia  with  right-sided  abducens  palsy; 
temporal  hemianopsia;  cranial  nerve  and  epilepsy;  hemianopsia; 
hemianopic  pupillary  reaction,  oculomotor  palsy ;  epilepsy,  oculomotor 
palsy;  ptosis;  internal  rectus  palsy;  optic  atrophy;  partial  oculo- 
motor palsy,  fifth,  seventh  palsy;  second,  third,  fourth,  fifth,  sixth 
nerve  involvement;  fifth,  sixth,  seventh,  eighth  nerve,  right  arm  and 
leg  palsy;  seventh,  eighth,  psychosis  (paranoia  acuta)  with  manic 
moods;  third,  fourth,  sixth,  tenth,  eleventh  nerve  palsies;  second, 
third,  fourth,  sixth,  seventh  with  epilepsy;  third,  fourth,  seventh, 
polyuria;  isolated  internal  ophthalmoplegia — to  mention  any  more 


VASCULAR  CEREBRAL  SYPHILIS  707 

would  be  to  needlessly  extend  this  chapter.  The  lesson  such  findings 
indicate  is  obvious.  This  multiplicity  of  syndromy  in  neurosyphilis  has 
been  a  commonplace  of  neurology  for  many  decades,  but  the  student  is 
rarely  interested  in  history.  Even  professed  specialists  are  constantly 
rediscovering  it. 

Eliminating,  as  far  as  possible,  the  varied  syndromes  of  cortical 
or  basal  neurosyphilis  meningovascularis,  and  limiting  the  discussion 
of  the  present  section  to  these  trends  of  cerebral  syphilis  due  more 
particularly  to  arterial  disease,  what  is  its  more  frequent  symptom- 
atology ? 

In  the  first  place  it  may  be  mentioned  that  the  vascular  types  of 
brain  syphilis  also  may  be  found  very  shortly  after  infection — as  short 
a  time  as  two  or  three  months.  Naunyn  in  a  thorough  study  found  that 
48  per  cent,  of  335  cases  reported  on  by  him  developed  signs  of  cerebral 
syphilis  within  three  years.  On  the  other  hand,  forty  years  have  been 
known  to  elapse  between  infection  and  the  development  of  a  cerebral 
syphilis. 

Here  the  prodromal  symptoms  are  usually  headache,  dizziness, 
sleeplessness,  irritability,  inability  to  apply  one's  self  continuoush^ 
to  one's  work,  lack  of  interest  in  work,  etc.;  in  general,  the  so-called 
neurasthenic  syndrome. 

Headache. — The  headache  is  usually  very  disagreeable;  it  usually 
has  a  migratory  character — here,  there  and  elsewhere;  usually  dull, 
it  is  at  times  boring.  It  is  inconstant,  intermittent,  often,  not  by  any 
means  always,  worse  at  night  than  in  the  daytime.  It  may  disappear 
for  weeks  or  months,  and  then  suddenly  reappear.  It  may  also  be 
the  only  sign  of  cerebral  syphilis  for  months  or  even  years. 

Dizziness. — Dizziness,  in  shorter  or  longer  attacks,  is  very  signifi- 
cant of  vascular  disease.  It  is  usually  associated  with  the  headache 
but  may  appear  as  the  single  symptom  of  brain  syphilis.  Like  the 
headache  it  is  apt  to  be  increased  by  mental  or  physical  work. 

Insomnia. — Insomnia  is  frequent,  often  obstinate,  quite  variable 
and  not  infrequently  sleep  is  made  irregular  and  non-restful  b}'  the 
sense  of  heaviness  in  the  head  or  actual  headache. 

Psychical  Distinbances. — Psychical  disturbances  are  the  rule  in 
those  patients  with  arterial  disease  of  the  cerebrum.  They  become 
more  or  less  apathetic,  lose  interest  in  their  work;  are  unable  to  work 
because  of  forgetfulness  or  inefficiency.  With  this  there  is  increasing 
irritability,  an  inability  to  size  up  the  situation.  Such  severe  disturb- 
ance is  arrived  at  only  after  some  time  as  a  rule. 

Abnormal  Sleepiness. — Abnormal  sleepiness,  coming  on  in  attacks, 
is  not  infrequent — such  periods  of  torpor  or  apathy  often  intermitting 
with  periods  of  anxiety  or  of  acute  restlessness  or  excitement.  Periods 
of  stupor  or  semicomatose  states  may  occur.  They  often  presage  more 
distinct  neurological  signs,  being  based  as  they  frequently  are,  upon 
sudden  extravasation,  infiltration  or  thrombotic  plugging  oft'  of  the 
blood  from  small  areas  of  brain  tissue. 

Many   patients   with   cerebral   syphilis   of   this   general   character 


708 


NEUROSYPHILIS 


remain  in  this  condition,  it  may  be,  for  some  time;  they  show  a  picture 
precisely  similar  to  certain  patients  with  general  paresis.  It  is  in  this 
general  group  that  the  greatest  difficulties  in  diagnosis  occur.  As 
has  been  previously  stated,  a  complete  serological,  cytological  and 
chemical  laboratory  study  is  the  last  criterion  for  differentiating  the 
trends  in  the  present  state  of  knowledge,  and  even  this  is  not  certain. 

Brain  tumor  is  also  to  be  thought  of  in  diagnosis.  Paresis,  brain 
tumor,  possibly  gumma,  cerebral  syphilis,  at  times  cannot  be  dis- 
tinguished one  from  another  clinically.  With  brain  tumor,  non- 
complicated by  syphilis,  the  absence  of  the  four  reactions  affords  a 
positive  criterion. 

Local  Symptoms. — As  a  rule,  however,  the  greater  number  of  indi- 
viduals with  cerebral  sj'philis  develop  local  symptoms,  and  neurological 

rather  than  psychiatrical  syndromes 
come  into  relief,  or  the  latter  are 
intermingled  with  the  former.  Palsies 
develop.  These  are  transitory,  par- 
tial, not  widespread,  or  maybe  severe, 
complete,  and  permanent,  showing 
various  hemiplegic  syndromes,  ac- 
cording to  the  anatomical  site  of  the 
major  disturbance — usually  throm- 
botic— cortical,  capsular,  midbrain, 
peduncles,  pons,  or  medulla  —  the 
symptomatology  of  the  different  forms 
of  which  are  dealt  with  elsewhere. 

Successive  attacks  of  mild  and 
transitory  palsies  are  very  significant 
of  cerebral  syphilis.  Monoplegias 
are  not  infrequent,  one  arm,  one  leg, 
one  side  of  the  face,  possibly  the 
cortical  speech  areas  with,  in  case  of 
double  lesion,  pseudobulbar  palsy. 
Minor  speech  disturbances  are  ex- 
tremely frequent,  tremors  of  the  facial  muscles  usually  accompanying 
the  stumbling,  stuttering  or  drawling  speech. 

A  list  of  the  usual  clinical  diagnoses  similar  to  those  already  outlined 
for  basal  or  convexity  meningitis  would  show  a  multiplicity  of  phe- 
nomena no  less  complex  in  the  neurological  field  and  certainly  infi- 
nitely more  varied  in  the  mental  symptom  pictures.  As  these  latter 
will  be  discussed  more  in  detail  in  the  section  on  the  psychoses  no 
further  mention  will  be  made  of  them  here. 

Parenchymatous  or  Central  Types. — General  Paresis. — It  is  usual  to 
separate  paresis  as  well  as  tabes  from  other  sx-philitic  disorders  of  the 
nervous  system,  under  the  general  caption  of  para-  or  metasyphilitic 
disorders.  Fournier  is  largely  responsible  for  this,  and  to  paresis  and 
tabes  he  has  added  a  number  of  other  disorders,  in  other  parts  of  the 
body,  to  which  he  applies  the  term  para-  or  metasyphilitic. 


Fig.   386. — Pseudobulbar  palsy  from 
syphilitic  disease.     (Tilney.) 


CENTRAL  PARESIS 


709 


Just  why  para-  or  metas\-philis  is  not  known,  especially  so  far  as  the 
nervous  system  is  concerned.  Many  ingenious  hypotheses  have  been 
formulated  with  the  purpose  of  explaining  the  differences  between 
paresis  and  tabes,  on  the  one  hand,  and  other  types  of  neurosyphilis, 
secondary  or  tertiary,  on  the  other.  It  would  serve  little  purpose  to 
enumerate  them  in  detail,  since  none  has  as  yet  compelled  conviction.^ 


Fig.  387. — Cerebral  sj-philis  (arterial  typej  with  syfteiiinj 
advancing  dementia.     (Lafora.) 


Aphasia,  hemiplegia. 


To  return  to  the  syphilitic  etiology  of  paresis.  It  is  practically 
conceded  "no  syphilis,  no  paresis."  One  is  not  speaking  now  of  those 
few  individuals  who,  either  because  of  the  presence  of  brain  tumor 
or  the  existence  of  arteriosclerosis,  or  of  other  cerebral  disorder,  show 
a  close  clinical  resemblance  to  paresis. 

Finally  the  findings  by  Moore  and  Xoguchi  of  Treponema  palliduvi 
in  twelve  of  seventy  paretic  brains  se-'ve  to  render  more  certain  the 
relationship  of  the  organism  to  the  disease.  These  findings  have  been 
amply  confirmed  by  a  number  of  observers. 


'  Mcintosh,   Fildes,  Head  and  Fearnsides:     Brain,  1913,  vol.  xxxvi. 
Fildes:     Brain,  1914-15,  vol.  xxx^^i. 


Mcintosh  and 


710  NEUROSYPHILIS 

But  s^-philis  does  not  by  any  means  necessarily  lead  to  paresis,  for- 
tunately. The  most  recent  studies  of  Mettler  show  that  about  2  per 
cent,  of  those  infected  with  s\T)hilis  develop  paresis.  A  considerably 
larger  percentage  develop  vascular  cerebrospinal  s\-philis — how  large, 
can  only  be  surmised. 

The  question  then  arises,  How  is  it  that  in  certain  cases  a  disorder 
arises,  usually  more  than  five,  more  frequently  about  ten  years  after 
infection  which,  while  closely  resembling  many  forms  of  cerebral 
s^'philis,  yet  differs  from  it  in  certain  very  noteworthy  particulars, 
and  what  underlies  these  differences?  In  other  words.  Why  para- 
or  metasyphilis? 

Bolton^  believes  that  those  patients  develop  paresis  who  originally 
had  a  defective  t^^pe  of  cortex — a  similar  concept  to  the  concept  of 
organ  inferiority  of  Adler.- 

Naturally  there  are  those  who  say  there  is  no  difference,  either 
anatomically,  biologically,  or  therapeutically.  They  are  in  the  minority 
with  certain  well-developed  arguments,  some  of  which  are  as  yet 
unanswerable.  The  present-day  attitude  is  to  maintain  a  distinction 
between  the  strictly  meningovascular  syphilitic  trends  and  the  paren- 
chymatous or  central  trends,  termed  general  paresis,  taboparesis, 
tabes,  etc.  Chiefly  because  the  histological  pathology  is  unique,  the 
biological  tests  show  slightly  different  quantitative  variations,  and  the 
results  of  therapy  diverse.  The  differences  are  possibly  more  of  degree 
than  of  kind,  but  by  some  observers  these  differences  are  explained 
on  the  basis  of  secondary  phenomena,  subsumed  under  the  general 
conception  of  allergic. 

Von  Pirquet's  conception  of  "allergic"  opened  a  line  of  thought 
which  led  Mcintosh  and  Fildes  to  formulate  the  view  which  many 
observers  now  hold  with  regard  to  the  nature  of  parasyphilis.  Von 
Pirquet  showed  that  all  infections  led  to  a  change  in  the  character  of  the 
reaction  of  the  tissues  to  the  poison.  This  might  take  place  in  two 
directions;  they  might  respond  less  and  less  to  infection  ("hypoallergie") 
until  at  last  they  ceased  to  respond  altogether  and  became  "anergic." 
But,  on  the  other  hand,  a  change  in  the  opposite  direction  might  occur; 
the  reaction  might  appear  both  more  quickly  and  with  greater  violence 
to  a  smaller  dose  of  the  poison.  This  he  speaks  of  as  hyperallergie  and 
brought  into  this  category  the  phenomena,  described  by  Richet,  under 
the  name  of  "  anaphylaxis." 

From  this  point  of  view  tertiary  and  "parasj^jhilitic"  phenomena 
are  manifestations  of  hj'persensitiveness  ("h^'perallergie").  As  the 
tissues  are  hj'persensitive,  they  react  more  strongly  to  a  minute  dose 
of  the  poison,  and  hence  some  of  the  difficulty  of  discovering  the 
spirochete  in  gummatous  and  paras^'philitic  tissues.  The  difference 
between  the  anatomical  consequences,  produced  by  the  impact  of  the 

>  Bolton,  Joseph  Shaw :  The  Brain  in  Health  and  Disease,  London,  Edward  Arnold, 
1914. 

2  AdFer,  Alfred :  Study  on  Organ  Inferiority  and  its  Psychical  Compensation,  Nerv. 
and  Ment.  Dis.,  Monograph  Series,  No.  24. 


CENTRAL   PARESIS  711 

poison  of  the  two  cases,  depends  on  the  nature  of  the  tissue  attacked. 
Thus,  gummatosis  is  the  reaction  of  hypersensitive  vessels  and  con- 
nective tissue,  parts  which  tend  to  show  proliferative  changes.  On  the 
other  hand,  the  pathological  anatomy  of  "paras^-philis"  of  the  central 
nervous  system  is  the  expression  of  the  response  of  hj-persensitized, 
highly  differentiated  nerve-elements  which  have  no  power  of  regener- 
ation. The  profound  difference  between  the  two  forms  of  disease- 
process  depends,  not  on  the  extent  to  which  the  tissues  have  become 
hj-perallergic,  but  on  the  fact  that,  in  the  one  case,  proliferative  repair 
can  occur  at  once,  while,  on  the  other  hand,  the  injury  to  the  nerve- 
elements  results  in  their  death.  This  view  explains  the  fact  that 
"paras^'philitic"  processes  occur  in  those  nerA^e  fibers  in  which  the 
sheath  of  Schwann  is  absent. 

The  conclusions  arrived  at  by  Fildes  and  Mcintosh^  are  as  follows: 
(1)  Parasyphilis  of  the  nervous  system  is  a  purely  clinical  conception. 
It  is  a  diseased  state  which  may  affect  any  part  of  the  brain  or  spinal 
cord;  the  manifestations  of  tabes  and  paresis  can  only  be  erected  into 
two  clinical  divisions  by  an  arbitrary  selection  of  signs  and  symptoms. 
Some  forms  of  progressive  muscular  atrophy,  lateral  and  combined 
sclerosis,  primary  optic  atrophy  and  periodic  epileptiform  attacks  may 
be  equally  definite  manifestations  of  parasA-philis.  (2)  Parasyphilis 
is  slightly,  if  at  all,  amenable  to  antisyphilitic  treatment  with  com- 
pounds of  arsenic  and  mercury,  probably  because  these  bodies  do  not 
enter  the  essential  hmphatic  structures  of  the  central  nervous  system. 
(3)  ParasA-philitic  stages  are  peculiarly  liable  to  arise  after  mild  sj^h- 
ilitic  infection.  (4)  In  paresis,  and  in  active,  untreated  cases  of  tabes, 
and  tabo-paresis,  the  cerebrospinal  fluid  yields  a  positive  Wassermann 
reaction,  often  of  great  strength.  (5)  With  acute  or  chronic  sj^hilis 
of  the  ners^ous  system,  other  than  parasyphilis,  the  behavior  of  the 
cerebrospinal  fluid  depends  upon  the  extent  to  which  the  spinal  cord 
and  its  membranes,  including  those  of  the  brain-stem,  are  affected. 
Thus,  most  cases  of  meningomyelitis  show  a  strong  positive  reaction  in 
the  cerebrospinal  fluid,  while  cerebral  lesions  tend  to  give  a  w^eakly 
positive  or  even  a  negative  reaction .  (6)  AntisA-philitic  treatment  has  a 
profound  effect  on  the  positive  reaction  in  s}-philitic  meningomyelitis, 
and  the  cerebrospinal  fluid  may  give  a  negative  reaction  after  a  few 
months.  But  in  cases  of  parasyphilis  no  positive  change  occurs  in 
consequence  of  such  treatment,  within  any  comparable  period.  (7) 
Whatever  views  may  be  held  of  the  primary  and  secondary  manifes- 
tations in  syphilis,  Fildes  and  Mcintosh  believe  that  all  tertiary  and 
parasy])hilitic  manifestations  are  expressions  of  the  reaction  of  hyper- 
sensitized  tissues  (hyperallergie).  That  is  to  say,  during  the  previous 
stages  of  infection,  the  tissues  have  been  so  altered  that  they  react 
more  violently  to  a  smaller  dose  of  the  spirochete  or  its  toxins.  Gum- 
matosis is  the  reaction  of  hypersensitized  connective  tissues  and  blood- 
vessels, while  parasyphilis  is  a  hyperallergie  reaction  of  the  essential 

•  Mcintosh,  Fildes,  Head  and  Fearnsides:  Brain,  36,  Part  I. 


712 


NEUROSYPHILIS 


nerve  elements  and  neuroglia.  (8)  The  consequence  of  this  hyper- 
allergic  reaction  in  the  tissues  of  the  central  nervous  system  is  death  of 
any  set  of  fibers  or  cells  which  happens  to  be  attacked,  and  proliferative 
reaction  on  the  part  of  the  neuroglia  within  the  same  territory.  Thus, 
the  clinical  manifestations  of  parasyphilis  are  an  expression  of  the 


Fig.  388. — Treponema  pallidum  in  the  brain  of  a  paretic.     (Moore.) 


reaction  and  necrosis  of  hypersensitized  areas  of  the  nervous  system 
evoked  by  reappearance  of  the  Treponema  paUidmn.  (9)  This  hyper- 
sensitive (hyperallergic)  state  of  the  tissues  of  the  central  nervous 
system  is  produced,  in  all  probability,  by  the  passage  of  the  spirochetes 
or  their  toxins  up  the  nerves  from  the  skin  and  mucous  membranes 
during  the  secondary  period.    But  it  is  also  conceivable  that  it  may  be 


CENTRAL  PARESIS 


713 


due  to  a  slight  encephalitis  during  this  stage  of  infection.  The  head- 
ache and  lassitude,  unaccompanied  by  any  gross  nervous  lesions,  which 
so  frequently  occur  during  the  secondary  stage,  possibly  represent 
clinically  the  period  during  which  sensitization  occurs.  (10)  Thus, 
parasyphilis  is  a  clinical  conception  which  comprises  the  manifestations 
of  a  series  of  diseased  states.  From  the  pathological  point  of  view  the 
term  is  inadmissible.  These  states  depend  on  the  reaction  of  hyper- 
sensitized  tissues  to  the  spirochete  or  its  toxins,  and  this  reaction  is  as 


Fig.  389. — Amnion's  horn  in  paresis.     Pyramidal  cell  layer  has  disappeared,  vessels 

infiltrated. 


truly  s}T5hilitic  as  the  production  of  gummata.  The  difference  between 
the  consequences  of  the  tertiary  and  of  the  parasyphilitic  process  lies 
in  the  nature  of  the  tissues  participating  in  the  reaction .  In  the  one 
case  the  connective  tissue  is  capable  of  repair,  and  the  focus  is  readily 
reached  by  the  remedial  agents.  In  the  case  of  parasx-philis,  reaction 
of  the  essential  nerve-elements  leads  to  their  death,  and  the  anti- 
syphilitic  remedies  cannot  readily  reach  the  spirochete. 

We  cannot  here  go  further  into  the  pathology  of  the  parenchymatous 


714  NEUROSYPHILIS 

types  of  neurosyphilis,  but  must  refer  the  student  to  the  studies  par- 
ticularly of  Alzheimer,  Nageotte,  Nissl  {loc.  cit.)  and  Warthin.^ 

Symptoms. — The  syndrome'  is  characterized  by  a  bewildering  mul- 
tiplicity of  forms,  which,  shifting  in  the  individual  patient  from  month 
to  month,  at  times  even  from  day  to  day,  prevent  any  clean-cut  descrip- 
tion that  will  embrace  its  many  vagaries.  Attempts  have  been  made 
to  create  empirical  types.  Thus,  Binswanger,  in  a  notable  study, 
created  three  t\7)es  based  on  certain  anatomical  peculiarities — menin- 
gitic-hydrocephalic,  hemorrhagic,  and  taboparetic  forms.  Such  an 
anatomical  subdivision  has  no  reliable  clinical  counterparts. 

Attempts  at  the  erection  of  clinical  tj-pes  are  perhaps  slightly  more 
encouraging,  for  many  patients  run  a  course  more  or  less  susceptible 
of  literary  descriptive  generalization. 

The  earlier  monographs  of  Simon  and  Krafft-Ebing,  and  the  latter 
ones  of  Jofi'roy,  Obersteiner,  Klippel,  and  Kraepelin^  suggest  the 
following  groups : 

1.  Simple  dementing  t;y^es. 

2.  Simple  depressed  t^pes. 

3.  The  expansive,  or  so-called  classical  type. 

4.  The  agitated  types. 

5.  The  irregular  types  with  localized  symptoms,  Lissauer,  tabo- 
paretic form. 

6.  Juvenile  paresis. 

Before  even  attempting  a  description  of  these  purely  artificial 
delimitations,  pictures  which  are  constantly  shifting  and  showing  com- 
binations of  details,  a  brief  glance  at  the  chief  symptom  components 
is  advisable. 

These  have  frequently  been  divided  into  the  mental  and  physical, 
but  as  this  is  a  purely  arbitrary  distinction  it  will  not  be  emphasized 
here.  As  has  been  noted,  a  diagnosis  of  an  impending  paresis  may  be 
made,  at  times  some  years  before  its  onset,  by  the  findings  in  the 
cerebrospinal  fluid,  but  attention  is  here  first  focussed  upon  the 
mental  picture.  A  peculiar  psychical  weakness  is  one  of  the  early 
phenomena.  This  has  been  badly  termed  neurasthenic  by  some.  A 
difficulty  in  perceiving  external  impressions  shows  this  intellectual 
loss.  In  the  early  stages  it  may  require  special  study  of  reaction  times, 
which  are  usually  lengthened,  but  soon  absent-mindedness,  inattention, 
loss  of  details,  forgetfulness  of  important  facts,  become  apparent. 
There  is  a  gradually  developing  loss  of  ability  for  prolonged  mental 
effort;  in  conversation  finer  shades  of  meanmg  are  lost,  the  patient 
is  no  longer  alert  and  keen,  as  perhaps  has  been  his  normal  habit. 
The  mental  deterioration  going  on  leads  to  many  changes  in  his  usual 
conduct,  until  the  patient  may  be  no  longer  quite  sure  of  himself  in 
his  customary  surroundings. 

Certain  patients  develop  a  state  of  dreamy  consciousness,  as  though 
in  a  mildly  intoxicated  state. 

1  Jour.  Lab.  Clin.  Med.,  1918. 

2  General  Paresis,  Nervous  and  Mental  Disease  Monograph  Series,  No.  15. 


PARESIS 


715 


Increased  fatigability  is  another  early  symptom.  Much  has  been 

written   of   the  preneurasthenic   stages   of  paresis.  This   excessive 

fatigue  may  prevent  him  from  starting  anything  new — sometimes 
he  even  falls  asleep  while  at  work  or  in  conversation. 


;c^:-. 


ENPOTHELIJJM 
'      P^iASMA  CEILS 


ROD 
:,  CELLS 


•A 


%  .-gt>\  LY1.CPH0CYTES 
)     * 


Fig.  390. — Cellular  changes  in  paresis. 


Defects  of  Reientvm  and  Memory. — Retention  and  memorv^  soon 
commence  to  show  defects.  Careful  studies  in  the  early  stages  have 
shown  difficulties  in  association,  lessened  capacity  for  learning,  dis- 
turbance hi  attention,  often  with  good  retention.  The  patients  forget 
recent  happenings  more  readily,  not  knowing  what  has  transpired 
a  week  ago,  yesterday,  sometimes  a  half-hour  ago.  These  grosser 
defects  belong  to  the  later  stages  as  a  rule.    The  memory  of  time  rela- 


716 


NEUROSYPHILIS 


tions  gradually  slips  away,  the  patient  being  unable  to  arrange  suc- 
cessive phases  in  an  orderly  series.  Thus,  many  of  these  patients 
show  the  greatest  defects  in  their  appreciation  of  time  differences, 
when  married,  age  of  oldest  child,  and  related  striking  facts  of  life. 
In  later  stages  all  sense  of  time  may  become  effaced. 


Fig.  391. — Paresis.     Cortical  chaages.     Epileptiform  convulsions.      (Lafora.) 


Fig.  .392. — Paresis  with  Korsakov  syndrome;  Wernicke's  polioencephalitis  superior. 


Impoverishment  of  Ideas. — A  gradual  impoverishment  of  ideas  takes 
place,  varying  in  degree  from  month  to  month  and  showing  also  great 
variability  with  different  patients.  Those  associations  most  in  use 
usually  persist  the  longest,  and  all  tests  should  take  into  consideration 
special  aptitudes.     In  the  later  stages  the  patients  have  lost  prac- 


PARESIS  717 

tically  all  of  their  mental  possessions — the  gap  at  times  being  filled 
in  by  retrospective,  confabulatory  reminiscences.  Certain  of  the 
patients  resemble  patients  with  a  Korsakov  syndrome. 

Loss  of  Judgment. — Loss  of  judgment  naturally  goes  on  pari  passu 
with  the  general  psychical  disintegration.  In  the  early  stages  even 
refined  methods  of  testing,  such  as  those  of  Gregor,  show  that  uncer- 
tainties, contradictions,  logical  lapses  are  not  infrequent.  The  patients 
are  easily  distracted  by  sound  associations,  9  x  9  =  99,  etc.  As  this 
loss  of  judgment  goes  on  the  patients  may  make  the  most  absurd 
plans.  They  do  the  most  unusual  things,  often  involving  their  entire 
fortunes  and  playing  havoc  with  all  of  their  carefully  woven  social 
fabric.  Dream  world  and  real  world  become  hopelessly  confused  in 
this  fundamental  psychic  crumbling. 

Hallucinations,  illusions,  and  changes  in  simple  sensory  perception 
are  found,  but  they  are  not,  as  a  rule,  prominent  features  in  paresis. 
Delusion  formation  is  naturally  present  in  many  instances,  although 
certain  patients  may  go  through  the  disorder  with  but  few  delusional 
developments. 

Delusions. — The  delusional  ideas  vary  immensely — they  are  usually 
senseless  and  fantastic  and  when  combined  with  active  creative  fan- 
tasies, as  they  frequently  are,  especially  in  agitated  or  excited  periods, 
pass  all  bounds.  These  patients  think  in  millions,  billions,  quadrillions, 
etc.  They  are  princes,  kings,  emperors,  potentates,  priests,  Christ, 
God,  supergods.  They  have  rubies,  pearls,  diamonds,  emeralds;  two 
wives,  a  dozen,  a  harem,  thousands  of  beautiful  women,  etc. 

These  delusional  ideas,  simple  or  fantastic,  are  also  liable  to  great 
lability.  They  are  always  changing;  contradictory  as  well  as  uncon- 
scious. New  ones  come,  old  ones  go,  revivals  take  place.  Progressions 
may  go  backward:  Now  they  have  millions,  next  moment  have 
thousands;  now  a  king,  in  ten  minutes  a  fine  soldier.  One  can  at 
times,  by  talking  with  these  patients,  expand  or  contract  their 
delusional  exuberance  almost  at  will. 

The  same  characteristics  may  be  noted  in  regard  to  delusions  of  a 
depressive  or  hypochondriacal  nature.  Nihilistic  delusions  such  as 
believing  they  have  no  stomach,  no  heart,  or  are  dead  are  not  infre- 
quent in  these  types. 

Alteration  of  Emotional  Activity. — ^The  disposition  or  emotional 
reactivity  is  involved,  as  is  the  intelligence.  As  a  rule  the  patients 
in  the  early  stages  are  h3T)erexcitable — others,  however,  are  markedly 
depressed.  They  are  apt  to  be  touchy,  surly,  cross,  even  having  \'iolent 
outbursts  for  the  most  trivial  events.  There  is  often  a  distinct  damper 
in  their  higher  ethical  feeling,  so  that  the  stimulus  of  conversation, 
the  joy  of  music  or  art,  of  various  social  relations  gives  way  to  a 
careless  indifference,  often  at  great  variance  with  the  psychomotor 
activity  of  the  patient. 

As  the  disorder  progresses  the  mood  is  apt  to  be  colored  by  the 
delusional  interpretations.  Anger  and  laughter  may  follow  one  another 
in  quick  succession,  and  a  great  variety  of  fleeting,  changeable,  often 
contradictory,  emotional  states  are  passed  through. 


718  NEUROSYPHILIS 

Character  Alterations. — The  character  alterations  are  predominant. 
Decision  is  progressively  lost;  instability  and  foolhardiness  alter- 
nating with  obstinancy  and  perverseness.  Initiative  is  reduced,  and 
the  patient  may  become  as  clay  in  the  potter's  hand;  such  periods 
often  alternate  irregularly  with  impulsive  heedlessness.  Kraepelin 
relates  the  case  of  a  patient  who  stepped  out  of  a  second-story  window 
to  pick  up  a  cigar  that  he  happened  to  notice  on  the  walk  beneath  him. 
Criminal  actions  may  be  committed  in  just  the  same  manner  as  the 
case  of  the  paretic  who  shot  at  Mayor  Gaynor,  of  New  York.  Suicide 
may  occasionally  take  place  in  the  same  manner.  Stealing  is  by  no 
means  infrequent,  and  sexual  misdemeanors  and  crimes  are  extremely 
prevalent.  This  blunting  of  the  repressions  inculcated  by  the  force 
of  civilization  is  particularly  noticeable,  and  predominantly  in  the 
sexual  sphere.  Hence  results  the  frequent  telling  of  lewd  stories, 
consorting  with  people  of  quite  inferior  social  status,  exhibitionism, 
shameful  and  open  masturbation,  and  even  genital  assaults. 

Osnato  emphasizes  that  it  is  the  abnormal  types  of  personality  that 
are  more  apt  to  develop  paresis  as  a  result  of  luetic  infection  and  that 
the  forms  of  psychological  reaction  will  conform  to  the  types  of  person- 
ality possessed  by  the  individual.^ 

Neurological  Sigjis. — Here  one  finds  not  infrequently  in  the  begin- 
ning phases  a  dull,  heavy  headache.  Hyperesthesia  often  precedes  the 
blunting  of  any  special  sense,  and  various  localized  disturbances,  such 
as  word-blindness,  word-deafness,  auditory  hallucinations,  apraxia, 
asymbolia,  astereognosis,  indicate  a  speciak  localization  for  the  time 
being  in  more  or  less  definite  cortical  areas.  Optic  nerve  atrophy 
occurs,  at  times  early,  in  from  5  to  10  per  cent,  of  the  cases.  Special 
changes  in  the  optic  disk  are  recognizable  in  from  12  to  50  per  cent, 
of  the  cases. 

Changes  in  Cutaneous  Sensibility. — ^Very  frequently  cutaneous  sen- 
sibility is  modified — sharp  pains,  numbness,  itching,  etc.,  occur,  and 
in  those  forms  recognized  as  taboparetic  these  often  show  the  special 
localizations  of  the  tabetic.  Out  of  these  changed  sensations  delu- 
sional interpretations  frequently  arise.  A  general  insensibility  to 
peripheral  stimuli  develops  later,  and  the  patient  may  then  pay  little 
attention  to  any  kind  of  irritant,  heat  or  cold,  full  bladder,  distended 
rectum,  etc.  Occasionally  such  patients  mutilate  themselves,  cutting 
off  a  finger,  or  the  tongue,  or  the  testes  in  order  to  get  rid  of  what 
seems  to  them  a  foreign  body. 

Motor  Incoordinations. — Motor  incoordinations,  from  initial  trem- 
bling to  more  high-grade  ataxias,  apraxias,  adiadokokineses,  Rom- 
berg, asynergise,  are  common.  Intention  tremor  is  not  infrequent 
and  perseveration  is  almost  never  missed  in  the  later  stages. 

Disorders  of  Speech. — In  speech  the  motor  difficulties  have  been 
specially  studied  since  Esquirol  first  laid  stress  upon  such  changes  in 
mental  cases.    Frequently  beginning  with  slight  stumbling,  a  slurring 

*  See  his  interesting  analysis  of  the  content  of  the  paretic  mental  fabric  in  terms  of 
preparetic  character  type  in  Jour.  Nerv.  and  Meut.  Dis.,  May,  June,  1919. 


PARESIS 


719 


over  certain  letters  or  syllables,  r,  I,  etc.,  the  paretic  de\'elops  very 
characteristic  speech  anomalies.  These  come  out  with  marked  promi- 
nence in  the  use  of  test  phrases — electricity,  Methodist  Episcopal, 
organization,  truly  rural,  third  cavalry  brigade,  etc. — when  certain 
letters  are  repeated,  stumbled  over,  or  elided.  Paraphasia,  aphasia, 
ataxia  of  speech,  perseveration  are  among  the  frequent  later  develop- 
ments until  only  a  mumbling  may  be  possible  in  the  last  stages. 
Similar  changes  take  place  in  writing. 

Eye  Syviptoms. — In  the  ocular  movements,  analogous  difficulties 
are  observed  and  in  the  pupils  one  observes  a  variety  of  changes. 
Statistical  studies  show  these  pupillary  anomalies  to  be  extremely 
frequent.  Differences  in  size  from  50  to  80  per  cent.  (Rache);  dis- 
tortion of  the  pupillary  outlines,  74  per  cent.  (Joffroy);  Argyll-Robert- 
son pupil,  50  to  70  per  cent.  (Westphal,  Junius,  Arndt,  etc.  jNIany  of 
these  pupillary  anomalies  undergo  considerable  variation,  changing 
from  time  to  time  even  without  treatment.  Loss  of  consensual  light 
reflex,  as  already  noted,  is  often  one  of  the  earliest,  and  at  the  same 
time  one  of  the  most  persistent  of  the  pupillary  anomalies.  Early 
retinal  changes  of  minute  exudative  types  are  frequent. 


Fig.  393. — Set  of  pictures  of  paretic  convulsion.     (Krapelin.) 


Conmdsive  Phenomena. — Convulsive  phenomena,  epileptiform  or 
apoplectiform  in  character,  are  rarely  missed  in  paresis.  They  are 
usually  of  the  cortical  epileptic  type.  They  often  occur  early  in  the 
disease  or  may  punctuate  any  period  in  its  development.  At  times 
limited,  they  more  often  are  generalized,  and  frequently  have  pro- 
dromata,  such  as  dreamy  states,  motor  incoordination,  thickness  of 
speech,  twitchings,  etc.,  as  a  rule  occurring  early.  As  isolated  phe- 
nomena, typical  status  attacks  may  be  observed,  with  as  many  as  100 
or  more  epileptiform  crises  in  twenty-four  hours.  An  attack  in  the 
very  early  stages  may  last  only  a  few  seconds;  the  patient  suddenly 
sinks  back  on  his  chair,  and  is  all  right  in  a  few  moments — while,  on 
the  other  hand,  status  attacks  may  persist  a  week  or  even  more. 
Unconsciousness  is  usual,  though  it  may  be  very  slight  or  fleeting. 
A  vast  variety  of  focal  residuals  have  been  described. 

Similar  changes  may  be  observed  on  the  sensory  side  of  the  nervous 


720  NEUROSYPHILIS 

system,  and  so-called  psychic  equivalents,  as  in  the  more  classical 
epilepsies,  are  frequent. 

Statistical  studies  show  the  very  great  frequency  of  these  attacks, 
Obersteiner  recording  them  as  often  as  in  90  per  cent,  of  his  patients; 
while  Junius  and  Arndt  in  their  recent  extensive  study  give  them  as 
occurring  in  53  per  cent.  A  personal  study  (J.)  of  two  hundred  cases 
showed  them  in  78  per  cent,  of  the  patients.  Kraepelin  believes 
that  treatment  in  bed  limits  the  number  and  frequency  of  the  attacks; 
his  jMunich  statistics  show  an  incidence  of  about  65  per  cent. 

Alterations  in  Reflexes. — ^The  tendon  reflexes — triceps,  radius, 
knee-jerks  and  Achilles — are  usually  positively  involved,  either 
excessive,  in  the  greater  number  of  cases,  or  diminished,  especially  in 
those  patients  with  posterior  cord  involvement,  which  is  frequent. 

When  the  deep  reflexes  are  found  to  be  increased  other  symptoms  of 
involvement  of  the  pyramidal  tracts  are  not  infrequent.  Babinski 
reflex,  very  frequently  Chaddock's  external  malleolar  sign,  at  times 
the  paradoxical  reflex  of  Gordon  occur.  Possibly  there  is  an  ankle- 
clonus,  and  spasticity  in  gait  is  present.  If,  on  the  other  hand,  the 
deep  reflexes  are  diminished,  other  signs  of  involvement  of  the  posi- 
tion sense  and  deep  sensibility  fibers,  travelling  the  posterior  column 
pathways,  are  usually  found.  Ataxia,  Romberg,  girdle  sensations, 
anesthesise,  etc.,  pains  of  the  radicular  type,  are  also  often  encountered 
in  these  taboparetics. 

In  most  of  the  patients  there  is  great  variability  in  the  two  sides. 
Occasionally-  one  finds  spasticity  of  one  and  hypotonia  and  ataxia  of 
the  other,  and  combined  symptoms  are  to  be  expected  in  the  later 
stages,  especially  in  those  patients  with  prominent  cord  localizations. 

In  the  final  stage  contractures  occur  in  the  bed-ridden  patient. 
They  are  unable  to  do  anything  and  muscular  twitches,  spasms, 
localized  atrophies,  and  a  veritable  museum  of  anomalies  is  to  be 
looked  for. 

Findings  in  Cerebrospinal  Fluid. — ^The  findings  in  the  cerebrospinal 
fluid  have  already  been  discussed.  Suffice  it  to  say  here  that  they  are 
of  paramount  importance  and  a  diagnosis  of  paresis  without  the  signs 
obtainable  in  the  cerebrospinal  fluid  must  always  be  regarded  as 
lacking  in  a  most  important  element. 

Enough  has  been  said  to  show  that  the  clinical  picture  of  paresis 
may  be  closely  counterfeited  by  a  number  of  other  pathological  states 
— notably  brain  tumor,  cerebrospinal  syphilis,  arteriosclerosis,  chronic 
alcoholism,  sleeping  sickness,  etc. 

The  findings  in  the  fluid  are  very  definite.  A  positive  four  reactions, 
the  fluid  used  in  small  quantities— 0.05  to  0.2  c.c. — is  almost  certainly 
diagnostic  of  paresis,  yet  at  times  it  would  appear  that  positive  four 
reactions  are  found  in  other  syphilitic  processes  which  do  not  behave 
like  paresis. 

The  gold-sol  reaction  usually  offers  typical  paretic  curves.^ 

1  Kaplan:  Serology  in  Nervous  and  Mental  Disease,  1.  c;  Rawlings:  Am.  Arch. 
Neur.  and  Psych.,  Aug.,  1919. 


PARESIS  721 

Vasomotor  and  Trophic  Disturbances. — ^\'asomotor  and  trophic  dis- 
turbances may  appear  early  and  come  and  go;  among  them  skin 
eruptions,  such  as  herpes,  and  pemphigus  are  the  commoner  types 
met  with.  The  ready  appearance  of  bed-sores  and  abscesses  indicates 
the  lowered  resistance  of  the  skin  and  subcutaneous  structures. 

The  bodily  temperatiu-e  may  show  considerable  variation,  even  on 
opposite  sides  of  the  body;  it  is  usually  subnormal  in  the  later  stages, 
save  following  convulsive  seizure.  Sleep  is  irregular,  especially  in 
the  excited  stages,  when  the  paretic  may  not  sleep  for  days — whereas 
in  torpid  stages  or  in  those  quiet,  dementing  forms  the  patient  sleeps 
or  is  in  a  doze  much  of  the  time. 

The  appetite  is  capricious,  and  the  bodily  weight  is  apt  to  fall  off 
in  the  early  stages  and  during  excitement,  to  become  much  increased 
in  the  torpid,  quiet  states. 

Disorders  of  the  bladder,  and  incontinence  of  urine  and  feces,  all 
sooner  or  later  come  within  the  outlines  of  the  picture. 

Remissions. — One  clinical  featiu-e  which  is  very  striking  is  the 
tendency  of  this  disorder  to  show  marked  remissions.  In  certain 
respects  this  is  a  general  law  in  disease  processes,  but  in  paresis  it 
appears  most  striking  because  of  the  almost  miraculous  change  that 
takes  place  in  the  patient.  Such  patients  one  could  say  were  about 
to  die;  they  become  absolutely  helpless,  convulsion  follows  convulsion; 
in  the  interim  they  know  nothing,  are  bed-ridden,  have  to  be  fed,  soil 
themselves,  and  are  reduced  simply  to  breathing,  heart-beating 
automata.  They  may  remain  in  this  condition  for  weeks  and  months, 
and  then  pick  up  a  little,  and  then  more  and  more,  and  within  a  space 
of  six  weeks  to  three  months  many  such  patients  appear  to  be  almost 
well  and  like  themselves.  They  have  risen  from  the  dead,  and  strange 
to  say,  although  the  relatives,  friends,  and  business  associates  have 
been  told  over  and  over  again  perhaps,  for  they  should  be,  that  this 
is  not  a  cure,  that  it  is  only  a  remission  of  symptoms,  the  patient  is 
frequently  restored  to  all  his  civil  rights  and  given  full  control  of  his 
affairs.  In  the  majority  of  cases  this  is  disastrous;  he  may  launch 
out  into  new  lines,  involve  his  fortune,  marry  unwisely,  and  then 
after  a  few  months,  perhaps  a  year — the  longer  remissions  on  record 
have  been  five  or  six  years — the  average  is  about  six  months — the 
symptoms  return,  often  in  rapid  progression,  and  usually  lead  to 
death  after  variable  intervals  of  from  six  months  to  a  few  years. 

Forms. — ^To  return  now  to  the  subject  of  the  forms — those  more  or 
less  artificial  groups  which  for  the  purposes  of  description  psychiatrists 
agree  upon. 

The  symptomatology  of  paresis  varies  within  such  wide  limits 
because  of  the  extent  and  distribution  of  the  pathological  changes; 
because  of  the  individual  make-up  of  the  patients,  and  because  the 
disease  process  not  only  affects  the  highest  psychological  levels  but 
strikes  deep  into  the  physicochemical  and  sympathetic  foundations 
upon  which  these  higher  levels  are  built.  The  disease  presents 
therefore,  a  combination  of  psychological  symptoms  more  or  less 
46 


722 


NEUROSYPHILIS 


explainable  at  that  level  coupled  with  disintegrations  of  a  much  more 
material  character  and  stable  organization. 

1.  Dementing  Form. — That  which  characterizes  this  general  group 
is  the  progressive  mental  deterioration  with  motor  paresis.  Excite- 
ments, convulsions,  extravagant  delusion  formations  are  not  prominent 
and  when  present  are  transitory.  In  these  individuals  there  is  the 
early  period  of  nervous  irritable  weakness,  with  loss  of  mental  alert- 
ness, moodiness,  inability  to  work,  forgetfulness,  and  steadily  increasing 
poverty  of  thought.  Naturally,  the  picture  beginning  in  this  way 
may  suddenly  change.  This  is  sufficient  to  throw  the  patient  into 
another  group — but  if  the  development  is  of  the  slow,  progressive 
nature,  gradually  advancing  mental  weakness,  fleeting  delusional 
ideas,  often  with  childish,  weak-minded  features,  these  are  the  general 
symptoms  of  the  dementing  t\^e  of  paresis. 


Fig.  394. — Simple  dementing  form  of 
paresis. 


Fig.  395. — Paresis.     Demented  type 
of  fades. 


2.  Depressed  Forms. — Here  anxious  depression  is  in  the  foreground 
of  the  mental  picture.  Hypochondriacal,  delusional  states  are  promi- 
nent. The  patients  continually  complain  about  bodily  discomfort; 
have  lost  their  intestines,  or  have  destroyed  their  manhood  by  mas- 
turbation or  sexual  excesses.  These  delusional  ideas  become  more 
and  more  nonsensical.  In  many  instances  the  hj'pochondriacal  ideas 
are  dependent  upon  fancied  sinful  actions  or  wrong-doing.  They  are 
great  sinners,  they  must  be  protected  from  the  police,  they  fear  they 
will  be  sent  away.  Such  patients  often  have  persecutory  ideas,  and 
when  such  are  prominent  early  in  the  disease,  before  there  is  marked 
deterioration,  they  are  frequently  regarded  as  "paranoiacs"  especially 


PARESIS  723 

by  those  schools  which  regard  names  as  disease  entities  and  seek  for 
diagnosis  from  a  "pathognomonic"  symptom.  These  paretics  with 
persecutory  ideas  often  have  pronounced  halkicinations  of  hearing. 

Notwithstanding  these  h^T^ochondriacal  or  persecutory,  dehisional 
interpretations,  these  patients  are  markedly  indifferent;  they  are 
apathetic,  talk  and  move  in  a  monotonous,  dull  manner,  and  take 
little  interest  in  their  surroundings. 

Thus  the  loss  of  energy,  the  libido  in  Jung's  sense,  not  being  able  to 
go  forward  in  the  affairs  of  life,  a  marked  pathological  introversion 
takes  place,  and  the  regression  takes  hold  of  all  sorts  of  nonsensical, 
childish,  infantile,  and  archaic  fantasies.  Pathological  projection 
also  is  common,  and  one  has  a  regular  chaos  of  pathological  mental 
mechanisms.  The  sense  of  reality  is  so  markedly  impaired,  and  the 
affective  relativity  so  cut  off,  that  the  nonsensical  beliefs  have  no 
corresponding  or  adequate  emotional  relationships.  The  personality 
is  fragmenting  and  disintegrating. 

Childish  regressions  of  hero  formation  appear.  The  patient  is  a 
god,  a  king,  an  emperor;  like  Jack  and  the  beanstalk,  he  is  miles  high; 
as  m  Gulliver,  he  is  a  great  giant;  as  in  INIidas'  touch,  or  Aladdin's 
lamp,  he  breaks  the  bank  at  Monte  Carlo,  or  is  the  owner  of  immense 
gold  mines,  fabulously  valuable  jewels,  etc. 

Contrasting  states  of  great  inferiority,  weakness,  poverty,  cause  them 
to  be  very  fearful,  easily  confused,  easily  lost;  they  beg  for  protection, 
hide  from  anger,  or  ask  piteously  for  food,  preserve  scraps,  etc. 

They  become  unmanageable  in  bed,  and  finally  in  many  the  agita- 
tion and  fear  develop  great  resistance  and  violence.  Self-destruction 
may  be  attempted,  mutilation  occasionally  occurs.  ]\Iost  of  these 
attempts,  however,  are  fragmentary,  non-sustained  and  bungling. 

Stuporous  states  show  a  contrast  to  this  marked  violence.  They 
may  persist  for  weeks,  months,  or  even  years.  The  patients  lie  stupidly, 
"depressed,"  or  anxious  in  bed,  unclean  and  unmanageable.  Special 
rigidities,  catatonic-like  in  their  nature,  may  develop. 

The  special  statistics  show  that  from  15  per  cent,  to  20  per  cent, 
of  the  material  in  some  of  the  larger  European  hospitals  and  clinics 
may  be  in  general  thrown  into  this  depressed  category, 

3.  Expansive  Types. — This  general  type  has  been  for  years  con- 
sidered "classical,"  yet  they  are  not  as  frequent  as  the  demented  types. 
Thus,  Kraepelin  gives  30  per  cent,  in  his  Heidelberg  series,  Junius 
and  Arndt  27  per  cent,  in  their  Berlin  material.  It  is  to  be  regretted 
that  the  specialist  has  failed  to  emphasize  this  feature,  which  is  of  so 
much  value  to  the  general  practitioner,  and  has  confused  the  issues  by 
speaking  of  a  "  change  in  type.''  It  is  of  more  value  to  insist  upon  the 
comparative  rarity  of  the  megalomaaic  features  of  paresis,  since,  as  the 
average  medical  man  has  been  taught  to  recognize  paresis  by  this  sign, 
it  is  not  to  be  wondered  at  that  so  much  delay  has  occurred  before 
the  recognition  of  paresis.  The  emphasis  should  not  be  laid  upon 
the  comparatively  rare  expansive  cases. 

In  this  megalomanic  type  the  boastful  ego  rises  to  superior  heights. 


724 


NEUROSYPHILIS 


Everything  is  seen  from  the  standpoint  of  a  feeling  of  abundant 
energy.  At  first  the  ideas  are  those  of  great  exaltation,  within  the 
bounds  of  normal  human  experience,  but  soon  the  patient  looses  his 
earthly  bonds  and  soars  to  superhuman  unrealities.  His  strength  is 
appalling;  his  education  superior  to  any  others  in  the  world;  he  speaks 
ten,  nay,  all  languages;  has  all  wealth;  all  power;  figures  mount 
from  thousands  to  millions,  to  pages  of  ciphers.  And  in  kaleidoscopic 
changes,  and  great  individual  variation  one  learns  of  many  marvels 
of  superior  excellence  only  dreamed  of  in  childish  fantasy,  or  seen  in 
the  boasts  of  inferior  peoples. 

One  feature  of  this  frightful  megalomania,  which  has  its  very  great 
ups  and  down,  should  never  be  overlooked;  namely,  the  tendency 
for  such  patients  to  commit  genital  indiscretions,  even  atrocities; 

or  to  engage  in  the  most  foolhardy 
enterprises,  thus  jeopardizing  life  and 
property. 

This  feature  in  paresis  is  of  so  much 
importance  that  special  attention  should 
be  devoted  to  the  legal  measures  which 
should  be  invoked  to  prevent  the  worst 
consequences  of  this  mental  weak- 
ness. 

That  megalomania  has  a  distinct 
deterioration  background  is  seen  in  the 
frequent  combination  of  a  poor  clerk, 
in  a  state  institution,  who  speaks  of 
the  million-dollar  novel  he  is  writing. 
It  consists  of  a  few  miserable  scrawls 
on  toilet  paper,  or  on  the  edges  of  a 
daily  newspaper.  This  is  only  a  type. 
iSuch  inconsistences  may  be  read  of  in 
the  classics  of  psychiatry,  from  the 
work  of  Arnold,  in  1700,  to  the  present 
time. 

These  fantastic,  exalted,  euphoric  states  very  frequently  elaborate 
on  sexual  themes.  Thus  the  patients  have  hundreds,  millions  of 
wives  or  concubines — "'Solomon  was  a  piker  in  this  matter,"  boasted 
a  Belle vue  patient.  The  children  are  more  numerous  and  more 
beautiful  than  any  promised  to  the  ancient  Hebrew  heroes. 

One  patient,  mentioned  by  Kraepelin,  could  lift  ten  elephants,  was 
two  hundred  years  old,  9  feet  tall,  was  a  beautiful  Adonis,  weighed 
four  hundred  pounds,  had  an  iron  chest,  an  arm  of  silver,  a  head  of 
gold,  100  wives,  1000  million  boys  and  girls,  his  urine  was  Rhine  wine, 
and  his  feces  were  gold. 

The  illustrations  might  be  repeated  ad  infinitum.  They  are  to  be 
found  in  richer  or  poorer  elaboration,  in  shorter  or  longer  intervals  of 
excitement,  in  this  exalted  euphoric  type,  but  one  may  see  a  hundred 
paretics,  as  a  general  practitioner  may  see  them  in  the  early  stages, 


F 

_^g^ 

fc^    -k 

^^H^- 

\ 

Bi 

■^j 

i    ] 

^^fc^fi. 

*^ 

B 

k 

Fig.  396. — Paresis,  shomng  gran- 
diose tjTJe. 


PASESIS 


725 


and  never  get  a  ghost  of  an  idea  of  such  experiences.  Of  this  100 
some  time,  sooner  or  later,  25  to  30  of  them  will  be  liable  to  exhibit 
the  exalted,  euphoric,  megalomanic  picture  here  indicated  rather 
than  described. 

Consciousness  is  usually  much  clouded  in  this  form,  especially  while 
the  delusional  projections  are  in  their  full  growth.  Time,  place,  the 
great  world,  is  a  dreamy,  far-off  place  of  little  moment  to  the  mind 
engaged  in  its  ambitious  program.  Continuity  of  thought  is  practi- 
cally impossible,  and  chaos  and  anarchy  exist.  In  such  minds  hallu- 
cinations are  frequent. 


Fig.  397. — Excited  paretic.      (Kraepelin.) 

The  mood  is  happy,  overflowing  with  schemes  for  good  deeds  and 
generosity,  and  all-embracing  in  its  brotherly  love.  But  coherence 
is  not  to  be  expected.  Hypochondriacal  ideas,  such  as  delusions  that 
there  are  worms  in  the  head  may  rest  in  bizarre  connections  with  the 
delusion  of  being  a  great  philosopher,  a  Shakespeare,  etc.,  and  changes 
in  mood  are  of  frequent  occurrence.    Weeping  follows  ecstasy,  and  is 


726  NEUROSYPHILIS 

replaced  by  beatific,  sublime  happiness.  Sudden,  passionate  excite- 
ment leaps  up  under  restraint,  to  subside,  or  to  be  diverted  by  such 
a  trifle  as  a  falling  leaf,  or  a  ring  at  the  door  bell. 

The  great  psychomotor  excitement  is  a  striking  feature,  and  one 
difficult  to  manage.  These  patients  walk  miles,  are  on  the  go,  meeting 
people,  busily  engaged  in  everybody's  business,  making  plans  for  self 
and  others,  and,  when  he  is  confined,  the  paretic's  violence  knows 
no  bounds.    He  is  transformed  into  a  raving  animal. 

Throughout  all  of  the  excitement,  divertibility,  constant  changing 
of  plans,  mixture  of  silly  pleasure  and  superficial  sadness,  there  is  the 
note  of  great  deterioration  of  critique  and  emotional  degradation  and 
degeneration  which  shows  particularly  in  the  conventions  relative  to 
one's  person.-  Carelessness  in  dress,  uncleanliness,  grossness  in  eating, 
loss  of  finer  susceptibilities,  coarse  expressions,  frank  immoralities — 
these  are  but  a  few  of  the  possibilities  in  such  lax  conduct. 

In  watching  such  patients  from  day  to  day,  one  is  struck  by  the 
immense  variability  in  the  picture.  The  ideas  of  grandeur  may  all 
vanish,  the  patient  denies  he  ever  said  any  such  thing,  he  may  get 
angry  in  a  dispute  over  the  matter,  and  then  launch  into  a  magnificent 
grandiloquent  invective  of  collossal  outlines. 

As  the  dementia  increases,  these  large  ideas  may  entirely  disappear, 
or  be  preserved,  and  appear  on  the  surface  only  as  a  few  words,  or 
murmurs,  "good  to  eat,"  "fine  women,"  "millions,"  etc. 

Finally,  in  the  later  stages,  the  patients  all  sink  to  a  more  or  less 
common  level — "sans  everything." 

Among  the  expansive  forms  may  be  found  the  quick,  galloping 
patients  who  die  within  a  short  time.  Increasing  experience  seems  to 
show,  however,  that  these  excited  types  indicate  a  very  severe  reactive 
process,  and  hence,  if  they  do  not  die  in  the  height  of  the  reaction 
(galloping  cases),  they  provide  the  greater  number  of  the  more  sta- 
tionary and  protracted  forms — those  who  make  a  partial  recovery  with 
defect,  and  who  later  disintegrate.  Remissions  seem  to  be  common  in 
this  type  as  well. 

4.  Agitated  Forms. — Those  patients  who  show  a  predominant  motor 
activity  in  the  beginning  may  be  said  to  be  grouped  here.  Great 
restlessness  runs  through  the  entire  picture.  The  mental  content  is 
very  variable — euphoric,  depressed,  hj-pochondriacal,  mood  colora- 
tions flit  in  and  out.  Galloping  cases  are  usually  grouped  here,  in 
which  an  extremely  rapid  and  fatal  course  is  present. 

This  is  really  only  a  subgroup  of  the  preceding  type,  only  artifically 
separated  off  by  reason  of  the  more  consistently  persistent  psychomotor 
restlessness.  Remissions  are  frequent,  as  are  also  the  apoplectiform 
and  epileptiform  attacks.  The  pathological  process  simply  has  a  wider 
extension  in  the  motor  areas. 

The  acute  delirious  cases,  somewhat  resembling  delirium  tremens 
of  alcoholism,  and  independent  of  it,  are  arranged  by  Kraepelin  in  the 
agitated  group. 


TABOPARESIS 


727 


5.  Irreguhr  Types. — Lissauer,  etc.  These  patients,  showing  irregu- 
lar forms  of  development;  neurosjTnptomatic  groupings,  hemiplegias, 
etc.,  are  here  brought  together.  The  hemiplegic  and  taboparetic 
groups  are  the  more  frequent. 

rafcopare^i*.— Taboparesis  is  the  most  striking  of  these  irregular 
forms  and  deserves  a  further  outlining. 

It  has  been  assiuned  by  many,  especially  by  neurologists  (Schaffer, 
for  summary,  1912)  that  tabes  may  be  regarded  as  a  spinal  paresis,  and 
paresis  a  cerebral  tabes;  that  is,  the  disease  varies  only  by  reason  of 
the  greater  severity  of  the  process  in  the  one  or  the  other  locali- 
zation. 

Kraepelin,  on  the  other  hand,  accentuates  the  opposing  psychiatric 
view,  that  whereas  the  two  disorders  are  undoubtedly  fundamentally 
SA-philitic,  yet  they  are  two  different  kinds  of  processes,  and  that  when 


Fig.  398. — Paresis  with  tabetic  changes  in  spinal  cord. 

the  sjTnptoms  of  tabes  are  added  to  paresis  the  changes  in  the  cord  are 
not  exactly  similar  to  those  found  in  tabes  limited  to  the  cord.  The 
different  findings  in  the  cerebrospinal  fluid  in  the  two  disorders  would 
point  to  some  sort  of  a  difference  as  well.  There  are  no  real  differences. 
The  discussion  is  only  worth  while  if  it  will  lead  to  a  solution  of  the  very 
practical  question:  What  makes  the  pathological  lesion  locate  itself 
in  one  or  another  region?  There  are  few  facts  as  yet  available  to 
enable  this  question  to  be  answered.^ 

Clinically,  taboparesis  shows  in  a  combination  of  the  symptoms 
observed  in  the  two  forms.  RajTiiond  and  Nageotte  would  have  it 
that  every  paretic  would  show  tabetic  signs,  if  he  lived  long  enough. 
In  those  patients  with  pronounced  tabetic  onset  one  finds  the  frequent 
pupillary  anomalies,  the  diminution  or  loss  of  the  patellar  reflexes, 

^  See  Jelliffe:  Discussion,  New  York  Neurological  Society,  March,  1919,  Jour.  Nerv. 
and  Ment.  Dis.,  Aug.,  1919. 


728 


NEUROSYPHILIS 


Romberg  sign,  ataxia  of  lower  or  upper  extremities,  or  both,  hypo- 
tonia, lancinating  pains,  crises,  and  arthropathies.  These  are  found 
in  the  more  definite  taboparetics  closely  associated  with  the  psychical 
disturbances  already  outlined.  In  the  more  classical  tabetic  patients 
the  mental  disturbances,  to  which  Cassirer  and  O.  Meyer  have  devoted 
their  attention,  are  very  distinct  from  those  of  paresis. 

6.  Jumnile  Paresis. — ^This  form  is  quite  distinct.  It  was  apparently 
first  recognized  as  late  as  1877  by  Clouston.  It  appears  at  the  present 
time  not  infrequently,  since  the  Wassermann-Plaut  findings  offer  such 


Fig.  399. — ^Juvenile  paresis  in  the  emaciated  stage. 


certain  criteria  for  its  determination.  Such  tests  seem  necessary, 
since  the  clinical  picture  may  be  so  extremely  variable — hence  it  was 
overlooked — many  patients  dying  diagnosed  as  "imbeciles." 

Here  the  patient  may  make  a  comparatively  normal  development 
to  five  or  ten  years  of  age — certain  non-developmental  forms  probably 
belong  here,  but  are  now  disregarded.  Then  the  child's  mentality 
seems  to  drop.  In  older  children,  ten  to  sixteen,  this  drop  is  more 
apparent.  Poor  memory,  bad  motor  adaptation  and  gradual  deteriora- 
tion appear.  Childishness,  fabrication,  excitements,  and  depressions, 
fears,  and  anxieties,  are  frequent.    Epileptiform  convulsions  appear — 


SYPHILITIC  PSYCHOSES  720 

many  juvenile  paretics  are  gathered  into  the  almshouses  and  epileptic 
colonies  as  "epileptics  with  feeble-mindedness" — and  after  a  course  of 
three  or  four  years,  with  gradually  deepening  mental  disintegration, 
the  patient  dies.  The  histopathological  changes  are  identical  with 
those  of  the  adult  form. 

It  is  noteworthy  that  the  age  of  onset,  from  seven  to  twelve  years, 
is  the  same  length  of  time  that  in  the  adult  form  elapses  between 
infection  and  the  outcrop  of  the  parench;^'matous  disease. 

Interpretation  of  the  Paretic  Psychosis. — ^The  interpretation  of  the 
mental  sjniptoms  of  paresis  may  advantageously  be  approached  from 
two  angles :  In  the  first  place,  from  a  consideration  of  the  personality 
make-up  previous  to  the  onset  of  the  disease  process.  The  disorder  of 
mind  must  be  a  disorder  of  that  which  existed  before  and  therefore 
the  character  of  the  mental  symptoms  is  directly  dependent  upon  the 
type  of  individual.  Osnato^  believes  that  it  is  the  abnormal  tjT3es  of 
personality  that  are  more  apt  to  develop  paresis  following  luetic  infec- 
tion. In  the  second  place,  as  an  expression  of  organ  inferiority  (cortex) 
in  harmony  with  the  conception  of  Adler^  and  also  with  the  conclusions 
of  Bolton.^ 

The  grandiose  type  of  paretic  sjTnptom  we  believe  falls  quite  in  line, 
in  its  interpretation,  with  this  concept  of  organ  (cortex)  inferiority. 
The  destructive  luetic  process  produces  as  it  advances  an  ever  mcreas- 
ing  mental  inefficiency  which  is  compensated  for  in  the  only  possible 
way  because  of  its  organic  basis,  namely  by  fantasy.  The  patient 
begins  to  build  up  delusions  of  power  to  compensate  for  the  progressing 
weakness  and  these  delusions  increase  in  improbability  as  the  destruc- 
tive process  results  in  increased  mental  weakness.  This  p\Tamiding 
of  the  delusions  of  grandeur  (the  patient  is  not  only  worth  hundreds 
of  thousands,  but  millions,  trillions,  quadrillions,  etc.,  finally  having 
to  invent  words  to  express  the  size  of  .his  fortune)  goes  on  and  on  in  a 
vain  effort  to  overtake  the  results  of  cortical  (mental)  destruction. 
Real  inefficiency  is  compensated  by  an  effort  to  think  himself  efficient; 
efficienc}'  is  sought  to  be  brought  about  by  thinking  hard  enough  that 
it  is  really  so;  results  are  sought  by  thinking  of  them  as  attained — 
the  "  all-powerf ulness  of  thought." 

5.  Syphilitic  Psychoses.— In  this  section  are  included  the  psychoses 
which  are  associated  with  cerebral  syphilis  and  with  tabes.  In  the 
present  state  of  knowledge  a  clear  distinction  cannot  be  made  either 
on  pathological,  clinical,  or  psychological  grounds  between  the  so-called 
metasyphilitic  and  the  more  clearly  syphilitic  conditions,  and  there 
are  undoubtedly  all  sorts  of  gradations  between  these  two  practical 
divisions. 

Forms. — Neurasthenia. — Kraepelin  speaks  of  a  syphilitic  neuras- 
thenia— a  form  of  mental  disturbance  much  written  upon  by  earlier 

1  Personality  in  Paresis,  Jour.  Am.  Med.  Assn.,  February  16,  1918;  Jour.  Nerv.  and 
Ment.  Disease,  May,  June,  1919. 

'Study  of  Organ  Inferiority  and  its  Psychical  Compensation.  Jour.  Nerv.  and  Ment. 
Dis.  Monograph  Series,  No.  24. 

^The  Brain  in  Health  and  Disease,  London,  Edward  Arnold.  1914. 


730 


NEUROSYPHILIS 


Fig.  400. — Brain  of  a  patient  with  a  syphilitic  psychosis  of  acute  maniacal  type. 
Enlarged  and  engorged  vessels.     Syphilitic  meningitis. 


Fig,  401. — Brain  of  a  patient  with  a  chronic  syphilitic  psychosis  of  maniacal   type. 
SyphiUtic  leptomeningitis,  pachymeningitis. 


SYPHILITIC  PSYCHOSES 


731 


authors.  The  preneurasthenic  phase  of  a  cerebral  sj-phihs  or  of  a 
paresis  is  not  now  under  review.  It  is  apt  to  appear  shortly  after 
infection  and  manifest  itself  in  a  nervous  discomfort,  difficulty  '-in 
thinking,  irritability,  disturbance  of  sleep,  pressure  in  the  head, 
variable  and  changeable  discomfort  and  pain.  To  these  may  be 
added  slight  depression,  dizziness,  confusion,  anxiet}',  slight  difficulty 
in  finding  words,  temperature  variations,  paresthesise,  and  nausea. 

Many  students  prefer  to  interpret  these  symptoms  as  a  direct  result 
of  the  infection,  and  not  as  a  circumscribed  syndrome;  but  slight  vascu- 
lar changes,  minute  pupillary  alterations,  and  particularly  evidence  of 
meningeal  irritation  as  shown  by  the  spinal  fluid  lymphocytosis,  point 
in  the  direction  of  its  being  something  more  than  a  simple,  infectious 
reaction. 


Fig.  402. — Brain  of  juvenile  paretic  showing  marked  atrophy. 

Plant  has  described  the  psychoses  which  are  associated  with  cerebral 
syphilis  and  with  tabes  in  ten  groups.    The  groups  are  as  follows: 

1.  Simple  Luetic  Weakness  of  Mind. — ^This  is  the  weakness  of  mind 
which  usually  goes  with  gross  lesion  of  the  brain,  marked  by  hemiplegia 
or  monoplegia.  It  is  generally  the  result  of  the  blocking  of  a  consider- 
able vessel  by  a  thrombotic  process  and  usually  occurs  in  relatively 
young  people.  There  is  no  well-defined  type  of  mental  defect  resulting, 
as  this  is  dependent,  of  course,  upon  the  location  of  the  lesion  and  upon 
the  make-up  of  the  individual.  There  may  be  depression  or  euphoria 
or  a  simple  forgetfulness  and  indift'erence.  Occasional  cases  of  arterio- 
sclerosis occurring  early  in  life  simulate  this  condition  very  closely. 

2.  Syphilitic  Pseudoparesis. — Here  a  group  of  cases  is  found  which 
seems  to  occupy  all  portions  of  the  territory  between  the  true  syphilitic 
psychoses  and  paresis.  On  the  mental  side  the  distinction  between 
pseudoparesis  and  paresis  is  practically  impossible  to  make.    Persistent 


732 


NEUROSYPHILIS 


auditory  hallucinations,  however,  seem  in  experience  to  have  pointed 
quite  strongly  to  pseudoparesis.    The  most  reliable  diagnostic  criterion 


Fig.  403. — Devastation  of^cortex  in  paresis.     (Kraepelin.) 


is  the  behavior  of  the  cerebrospinal  fluid  toward  the  Wassermann 
reaction,  it  being  often  negative  in  vascular  syphilis  and  positive  in 


SYPHILITIC  PSYCHOSES  733 

paresis.  While  this  is  not  an  absolute  differentiation,  and  larger  doses 
of  the  serum  may  produce  the  positive  reaction,  it  is  still,  however, 
one  of  the  most  important  differentials.  It  has  to  be  remembered,  too, 
that  some  cases  of  paresis  are  found  with  negatively  reacting  fluid, 
and  rarely  cases  of  lues  with  positively  reacting  fluid. 

3.  Paranoid  Forms  Combined  with  Tabes. — In  this  group  are  found 
patients  who  do  not  show  any  considerable  deterioration,  but  present 
ideas  of  persecution  with  numerous  auditory  hallucinations  over  a 
considerable  period  of  time.  There  is  no  self-reproach,  they  remain 
lively  and  affable,  and  what  seems  to  be  peculiar,  present  marked 
hallucinations  of  common  sensibility  accompanied  by  fantastic  ideas. 

4.  Paranoid  Forms  without  Tabetic  Symptoms. — In  this  group  are 
presented  paranoid  ideas  combined  with  auditory  hallucinations,  rather 
resembling  the  alcoholic  hallucinoses.  A  pronounced  delusion  of  jeal- 
ousy was  present  in  one  case  described  by  Plant.  They  have  to  be 
separated  from  manic-depressive  psychosis  and  particularly  from 
dementia  precox.  The  separation  from  the  latter  is  made  from  the 
absence  of  catatonic  signs  and  failure  to  develop  marked  evidences  of 
defect. 

5.  Certain  Epileptic  Forms. — These  appear  to  be  due  to  the  endar- 
teritic  changes  in  the  small  cortical  vessels  and  may  be  combined  with 
paralytic  phenomena  which  develop  as  a  result  of  the  participation  of 
the  larger  vessels  in  the  disease  process.  The  cases  resemble  very 
closely  genuine  epilepsy  and  must  be  differentiated  by  the  neurological 
and  serological  findings.  A  case  described  by  Plaut  showed  transitory 
dream  states. 

6.  Short  HaUicinatory  Confused  States. — These  resemble  the  con- 
fusions associated  with  the  epileptic  forms  and  the  French  have  con- 
sidered them  in  the  same  class  with  the  crises. 

7.  Psychotic  Disturbances  Associated  with  Syphilitic  Cardiac  Disecuse. 
— This  condition  probably  develops  most  frequently  in  connection  with 
syphilitic  aortitis. 

8.  Psychoses  Resembling  Manic-depressive  Psychosis. — Here  con- 
ditions are  grouped  which  superficially  very  closely  resemble  the  manic- 
depressive  psychosis.  As  a  rule,  however,  there  is  something  to  attract 
attention  as  indicating  at  least  an  aberrant  form.  On  the  mental  side 
the  delusions  are  more  grotesque,  more  out  of  harmony  with  the 
personality  of  the  patient,  or  show  an  um-easonableness  which  is  not 
commensurate  with  the  degree  of  excitement.  On  the  physical  side, 
of  course,  inactive  pupils  should  lead  to  a  serological  examination. 
Occasionally  such  episodes  occur  a  long  time  before  the  outcrop  of 
frank  symptoms  of  metasyphilitic  disease. 

9.  Mental  Disorder  Due  to  Syphilis  as  a  Psychic  Trauma. — Here 
a  psychogenic  psychosis  which  is  more  apt  to  take  a  depressive  form 
is  included. 

10.  Hereditary  Luetic  Mental  Disturbances.— Ihis  envisages  psycho- 
pathically  defective  subjects  and  weak-minded  children  with  luetic 
etiology.    The  exact  relation  between  lues  and  various  forms  of  weak- 


734  NEUROSYPHILIS 

mindedness  is  not  accurately  known,  but  it  is  known  that  a  large  number 
of  the  feeble-minded  group  are  luetic.  Syphilitic  brain  disease  may 
occur  in  early  infancy  and  proceed  for  some  time,  producing  only 
transient  symptoms,  perhaps  an  occasional  convulsion,  and  ultimately 
lead  to  serious  defect.^ 

6.  Tabes.^ — History .^ — In  any  historical  presentation  a  sharp  distinc- 
tion must  be  made  between  the  name  tabes  dorsalis,  and  the  disease  as 
now  understood.  So  far  as  is  known,  the  former  had  its  origin  with 
Hippocrates,  the  latter,  if  one  accepts  the  post-Columbian  origin  of 
syphilis,  could  only  have  come  into  existence  among  Europeans  and 
their  descendants  after  the  fifteenth  century. 

The  various  interpretations  given  to  the  Hippocratic  term  through- 
out the  ages  is  a  chapter  of  surprises.  Spermatorrhea,  gonorrhea, 
leucorrhea,  gleet  were  its  initial  meanings,  with  or  without  signs  of 
organic  disease  of  the  cord;  when  combined  with  cord  signs — myelitic 
processes,  usually  tuberculosis — Pott's,  etc. — it  was  called  tabes 
nervosa  or  myelophthisis.  Out  of  this  mass  the  disease  of  the  present 
day  was  separated.  It  was  natural  that  excessive  venery  should  have 
been  attributed  as  the  cause.  As  late  as  the  early  part  of  the  last 
century  (1804)  the  term  persisted  in  the  sense  of  a  "nervous  weak- 
ness," i.  e.,  what  today  one  would  designate  a  "sexual  neurasthenia," 
or  a  "  sexual  phobia."  So  far  as  can  be  made  out  from  Littre's  Hippoc- 
rates this  is  exactly  what  Hippocrates  wrote  about  under  the  term 
tabes  dorsalis.  Even  in  the  theses  of  Harles  and  Brera,  in  the  early  part 
of  the  nineteenth  century,  the  relation  between  the  cord,  the  vessels  of 
the  lower  abdomen,  and  secretions  from  the  genito-urinary  organs 
was  held  to  be  a  close  one. 

As  to  the  earliest  observations  of  modern  tabes,  the  traces  are  very 
indistinct.  To  the  clinicians  of  the  sixteenth  and  seventeenth  centuries, 
the  confused  mass  of  paraplegias  was  practically  insoluble,  and  it  would 
appear  that  it  was  only  in  the  beginning  of  the  nineteenth  century  that 
the  process  of  differentiation  took  place.  E.  Horn  (1816)  called  atten- 
tion to  a  number  of  the  important  features  including  blindness; 
Weidenbach  (1817)  attempted  to  make  a  separate  disease  of  it — he  con- 
tested the  inflammatory  origin — said  it  had  nothing  to  do  with  con- 
sumption, although  still  unable  to  break  away  from  the  belief  in  the 
excessive  venery  etiology  then  rampant.  Schesmer  (1819)  described 
the  peculiar  gait  in  an  unmistakable-  manner,  while  W.  Horn  (1827) 
emphasized  the  real  absence  of  a  true  paralysis,  and  spoke  of  an  ataxia 
wherein  this  affection  was  different  from  other  forms  of  myelitis. 
Decker  (1838)  called  attention  to  the  swaying  and  unsteadiness  with 
closed  eyes,  which  was  taken  up  by  Romberg  three  years  later  and 
rechristened  Romberg's  sign. 

Pathologically,  the  characteristic  cord  signs  were  not  unobserved. 
Hutin  (1828)  describes  them,  Ollivier  of  Anglers  (1837)  gave  the  picture 

1  See  behavior  of  colloidal  gold  tests  in  differentiating  certain  of  these  types  by 
Rawlings,  1.  c. 

2  See  valuable  monograph  by  Maloney:    Locomotor  Ataxia,  New  York,  1917. 


TABES 


735 


reproduced  here  in  part,  while  Cniveilhier  (1832-1845),  in  his  Atlas, 
gives  masterly  clinical  and  pathological  descriptions. 

Romberg  in  the  first  edition  of  his  Lehrbuch  gave  greater  precision 
to  the  description,  and  Steinthal  (1847)  threw  together  the  incomplete 


Fig.  404. — Ganglion-cell  degeneration 
and  rod  cells  in  paresis.     (Lafora.) 


Fig.  405. — Axis-cylinder  degeneration  in 
paresis.     (Kraepelin.) 


paralysis  (or  ataxia),  the  Romberg  sign,  and  the  characteristic  gait, 
but  without  any  real  grasp  of  the  situation  clinically  or  pathologically. 
Later  Romberg,  in  the  second  edition  of  his  text-book  (1851)  gave 
greater  precision  to  the  concept,  and  gave  a  classical  description. 


736 


NEUROSYPHILIS 


Finally,  Duchenne,  in  the  years  1852  to  1858,  elaborated  the  general 
idea,  and  gave  the  first  complete  and  adequate  description  of  the  dis- 
order. It  may  be  said  that  Horn  and  Romberg  had  practically  made 
out  of  the  general  tabes  dorsalis  collection  a  special  tabes  dorsalis 


Fig.  406.^Brain  of  a  paretic,  showing  marked  atrophy.     Flaking  of  the  cortex  due 
to  adhesions  of  the  pia. 


Fig.  407. — Organization  of  Virchow-Robin  lymph  spaces  in  general  paresis.    Tannin 

gilver.     (Achucarro.) 


TABES 


737 


10 


collection  in  the  sense  of  present-day  conceptions,  in  the  physiological 
and  pathological  fashionmg  of  which  Todd  (1847),  who  seemed  to  grasp 
the  fact  that  incoordination  and  posterior 
columns  were  related ,  Rokitansky  (1854) ,  9 

Virchow  (1855),  Tiirck  (1856),  Landry 
(1858),  and  Gull  (1858)  made  lastmg 
contributions.  The  recent  historv  gives 
us  the  names  of  Charcot  (1868),  Vulpian, 
and  Topinard,  and  in  1863  three  mono- 
graphs appear  by  Eisenmann,  Leyden, 
and  Friedreich,  while  the  later  contri- 
butions of  ^Yestphal  (1875),  Argyll- 
Robertson  (1869),  Marie,  Redlich  and 
Obersteiner,  Nageotte,  Oppenheim, 
Dejerine,  Goldscheider,  Erb,  Nissl, 
Schaffer,  and  Alzheimer  record  the 
detailed  studies  m  etiology,  sjToptoma- 
tology,  pathogenesis  and  spinal  cord 
conditions  of  the  last  two  decades.^ 
Finally,  the  even  more  recent  work  of 
the  serologists,  particularly  Wasser- 
mann.  Citron  and  Plaut,^  and  Noguchi 
has  given  the  final  word  regarding  the 
etiological  factor,  syphilis. 

Etiology  and  Occurrence. — S^^hilis  is 
the  real  cause  of  tabes.  The  statistical 
method  had  almost  proved  the  sj-philitic 
etiology  of  tabes,  but  with  the  advent 
of  the  objective  methods  of  Wassermann 
and  his  students  all  doubts  have  van- 
ished. In  the  more  recent  work  of  the 
most  competent  serologists  syphilitic 
substances  are  found  in  the  blood 
serum  of  practically  evers'  case  (Plant, 
loc.  cit.).  Furthermore,  the  cytological 
examination  of  the  cerebrospinal  fluid 
shows  the  presence  of  cellular  exudates 
characteristic  chiefly  of  the  s;y'philitic 
processes,  and  as  will  be  seen  in  the 
consideration  of  the  pathological  feat- 
ures, the  syphilitic  nature  of  many  of  the  findings  is  beyond  con- 
troversy. Finally,  Treponema  'pallidum  has  been  found  in  the  spinal 
cord  areas  in  tabetics. 

1  Jelliffe:  On  Some  of  the  More  Recent  Literature  of  Tabes  Dorsalis,  Pathology  and 
Etiology,  International  Clinics,  1907,  ii,  257.  Later  literature,  see  Schaffer:  Lewandow- 
sky  Handbuch  and  work  by  Massarj-,  1909.  For  early  history  consult  E.  Meyer, 
Kritisch-Historische  Beobachtungen,  Strassburg  Diss.,   1880. 

'  The  Serodiagnosis  of  Syphilis  in  Psychiatry  (Nervous  and  Mental  Disease  Monograph 
Series,  1911). 

47 


12 


Fig.  408. — Illustration  of  the 
cord  of  a  tabetic  given  by  OUivier 
of  Anglers  in  1837. 


738  NEUROSYPHILIS 

Pseudotabetic  s^oidromes  are  known  to  occur  in  multiple  sclerosis, 
in  tumor,  in  caries,  in  poisoning  by  alcohol,  pellagra,  diabetes,  ergot,  etc. 

\^^lat  secondary  factors  are  necessary  to  determine  why  this  or 
that  patient  infected  with  syphilis  should  develop  tabes  cannot  yet  be 
answered.  Less  than  one-half  of  1  per  cent,  of  the  infected  develop 
the  disease,  so  that  other  factors  are  demanded  on  a  priori  grounds  to 
explain  why  one  syphilitic  individual  develops  it  and  ninety-nme  syph- 
ilitics  do  not;  and  this  is  true  only  for  certain  races.  The  many  cases 
of  congenital  tabes  following  a  syphilis  fxom  the  same  source,  suggest 
variations  in  the  virus.  Similarly  the  large  number  of  tabetics  who 
are  known  to  have  followed  the  wake  of  certain  syphilitic  prostitutes 
(Morel  Lavallee,  Erb,  Brosius — glass-blower  cases).  Another  factor 
may  be  due  to  variations  in  resistance.  (See  hyperallergie  discussion 
in  paragraph  on  paresis.) 

Both  of  these  factors  are  impossible  to  measure,  but  it  is  highly 
doubtful  if  any  of  the  many  causes  usually  meationed,  such  as  exposure 
to  cold  and  wet,  trauma,  excessive  venery,  etc.,  have  anything  to  do 
with  the  after-development  of  a  tabes.  The  multiplicity  of  assigned 
causes  makes  it  more  than  probable  that  none  are  concerned.  Ford 
Robertson's  specific  bacillus  is  certainly  not  a  proven  secondary 
factor.  Trauma  is  highly  doubtful.  It  may  cause  a  pseudotabes 
from  lesions  in  the  cauda  equina  chiefly.  Of  heredity  little  is  known. 
Charcot,  Borgherini,  Erb,  and  Gowers  have  laid  much  stress  upon  it. 

Occurrence. — The  majority  of  cases  occur  in  the  fourth  decade,  but 
this  is  largely  due  to  the  fact  that  the  disorder  comes  on  ten  to  twenty 
years  after  infection,  and  sj^Dhilitic  infection  usually  occurs  between 
the  twentieth  and  thirtieth  years.  The  disease  may  appear  at  almost 
any  period  after  infection  (fifteen  to  seventy  years  of  age).  The 
average  runs  from  thirty-five  to  forty,  the  average  interval  after 
infection  about  fifteen  years,  with  extremes  at  four  to  thirty-five. 
Dejerine  and  Raymond  report  cases  forty-five  and  fifty  years  after 
infection.  In  certain  races  with  a  high  syphilis  percentage  (Algiers) 
tabes  is  practically  unknown,  and  there  is  every  reason  to  believe  that 
the  syphilis  has  been  present  there  since  the  fifteenth  century.  In 
other  races  from  three  to  five  of  every  one  hundred  syphilitics  develop 
the  disease.  In  certain  countries  the  ratio  of  men  to  women  is  4  to  1, 
in  others  10  to  1 . 

Occupation  seems  to  play  only  that  role  that  speaks  for  increased 
opportunities  for  sj^^^hilitic  infection. 

Symptoms.^ — The  symptomatology  of  tabes  is  markedly  diverse, 
and  whereas  it  seemed  at  one  time  that  it  presented  a  more  regular 
picture  than  other  nervous  disorders,  accumulating  experience  shows 
that  typical  pictures,  so-called,  are  the  exception  rather  than  the  rule. 
In  other  words,  there  is  no  one  symptom  that  may  not  be  absent,  and 
very  few  symptoms  that  may  not  be  present  in  disorders  other  than 
tabes. 

>  See  Maloney,  loc.  cit. 


TABES  739 

Nevertheless  one  can  rely  fairly  well  upon  the  following  grouping 
of  s\Tnptoms:  Lancinating  neuralgic  pains,  mostly  in  the  lower 
extremities,  usually  preceding  all  of  the  other  ssmptoms;  paresthesise 
and  related  sensory  disturbances,  analgesiae,  hj'poesthesiae,  loss  of 
the  tendon  reflexes  (patellar,  Achilles),  incomplete  or  complete  Argyll- 
Robertson  pupil,  unilateral  or  bilateral  ataxia  in  both  extremities, 
Romberg's  sign,  bladder  disturbances,  hj-potonia,  ocular  palsies,  posi- 
tive Wassermann  in  the  blood  and  hTuphocytosis  in  the  spinal  fluid. 

A  host  of  other  symptoms  may  be  present  in  individual  cases,  either 
early  or  late,  but  those  just  mentioned  belong  more  particularly  to 
the  majority  of  the  cases,  and  are  usually  sufficient  to  make  an  early 
diagnosis.  A  description  of  the  individual  sjinptoms  will  show  some- 
what of  the  proportionate  occurrence,  both  in  point  of  time  and 
frequenc}\ 

Pains. — Severe  lancinating  pains  occur  in  about  90  per  cent,  of  the 
cases,  and  usually  as  an  initial  sign  (60  per  cent.).  The  patients 
complain  in  an  almost  stereotyped  manner  of  having  had  sharp, 
severe,  fugacious  pains  usually  in  the  sciatic  and  crural  distributions, 
which  they  speak  of,  and  unfortunately  are  regarded  even  by  physi- 
cians, as  rheumatic.  These  pains  come  on  in  attacks,  last  a  few  minutes 
or  more,  several  hours,  a  few  days  and  then  disappear  to  again  recur. 
They  maj-  precede  the  development  of  other  symptoms  by  a  few 
months  or  even  many  years  (twenty-two  years — Erb).  The  average 
varies  widely.  They  are  an  indication  of  the  leptomeningitis  or 
radiculitis  which  is  one  of  the  fundamental  results  of  the  s\T3hilitic 
virus  or  products  induced  by  it.  Maloney  (I.e.)  contends  they  are 
mainly  due  to  implication  of  the  vegetative  nervous  system  and 
groups  the  pain  syndromes  with  gastric  and  other  crises.  The  pains 
are  usually  of  extreme  severity  and  are  much  dreaded  by  the  patient. 

Whereas  the  distribution  is  predominantly  sciatic  or  crural  and 
radicular  at  first,  the  pains  may  be  widely  distributed,  and  may  in  fact 
start  in  any  sensory  root,  cranial  or  spinal.  Thus  trigeminal  neuralgia 
may  be  an  initial  sign,  or  the  pains  may  affect  the  larynx,  or  the 
stomach,  or  the  heart,  the  bladder,  the  testicles,  the  intestmes,  and 
give  rise  to  various  forms  of  crises,  so  characteristic  and  so  much 
feared.    The  pains  may  be  felt  in  the  skin,  or  deeper  lying  structures. 

Deep,  boring  pains  are  also  present,  usually  later.  Not  infrequently 
the  larger  nerve  trunks  are  somewhat  painful  to  pressure.  This  fact 
may  lead  to  confusion  in  separating  an  alcoholic  or  other  neuritic 
pseudotabes. 

Crises. — ^These  have  some  relation  to  the  pains  of  tabes,  and  are 
probably  due  to  similar  pathological  alterations,  but  located  in  other 
vegetative  reflex  arc  segments.  The  best  known  are  the  gastric 
crises,  noted  as  early  as  1856  by  Gull  and  recognized  by  Charcot 
(1868)  as  belonging  to  the  general  picture  of  tabes.  The  patients  have 
sudden,  violent  gastric  pain,  radiating  in  all  directions,  and  in  the 
severe  attacks  accompanied  by  nausea,  vomiting  and  great  prostration. 
Like  the  pain  attacks,  these  crises  may  last  for  hours,  or  a  few  days  and 


740 


NEUROSYPHILIS 


then  disappear  for  weeks  or  months  to  recur  at  irregular  intervals. 
They  disappear  as  rapidly  as  they  come,  and  quite  analogous  to  the 
lancinating  pains,  may  be  early  or  late  symptoms.  Certain  cases  of 
tabes  begin  with  such  crises.  Such  a  beginning  is  frequently  not 
recognized  and  has  often  led  to  laparotomy. 

Similar  crises  affecting  other  internal  organs  have  the  same  etiology 
and  course.  Thus  there  are  intestinal  colics  with  diarrhea,  rectal 
pains  with  tenesmus  and  diarrhea,  vesical  crises  with  strangury, 
urethral  crises,  renal  colic-like  attacks,  testicular  crises,  vulvovaginal 


Fig.  409. — Tabes  involving  the  Cauda  equina,  showing  the  radicular  distribution  of 
the  sensory  disturbances.  Tactile  pain  and  thermal  sensibility  were  involved.  The 
patient  first  had  pains  and  sphincter  disturbances.  The  second  dorsal  root  was  also 
slightly  involved,  hence  the  sensory  changes  in  the  arms.     (Dejerine.) 


crises,  laryngeal  and  diaphragmatic  crises  with  cyanosis  and  dyspnea. 
pharjTigeal  crises  with  obstinate  hiccough,  bronchial  crises  with  cough, 
cardiac  with  angina-like  attacks.  Sneezing  attacks  have  been  described 
as  an  initial  tabetic  sign. 

Sensory  Involvement. — As  a  result  of  the  implication  of  the  meninges 
of  the  sensory  roots,  alterations  in  the  sensory  functions  take  place. 
The  pathological  process  in  some  is  so  gradual  or  mild  as  not  to  give 
rise  to  pain,  and  in  many,  initial  paresthesise  may  precede  the  pains, 
but  more  often  the  same  pathological  process  gives  rise  to  both. 


TABES 


741 


Tingling,  numbness,  crawling  sensations,  flashes  of  hot  and  cold, 
slight  benumbing  of  the  tactile  sensibility,  causing  the  sensation  of 
wearing  a  glove,  or  walking  upon  a  textile  are  the  usual  forms.  They 
may  be  expected  almost  an\^-here  from  the  region  of  the  trigeminus 
through  any  sensory  cervical  nerve  branch  to  the  tips  of  the  toes, 
although  the  ulnar  region  seems  a  site  of  special  predilection.  Occasion- 
ally they  cause  the  peculiar  girdle-band  sensation,  at  one  time  con- 
sidered so  characteristic  of  tabes.  A  whole  limb  may  be  involved, 
but  under  any  condition  the  tendency  for  the  sensory  disturbances  is 
to  show  a  radicular  distribution  (Dejerine)  (Figs.  409-413). 


Fig.  410  Fig.  411  Fig.  412  Fig.  413 

Figs.  410,  411,  412  and  413. — Radicular  distribution  of  sensory  disturbances  in 
tabes;  Figs.  410  and  411  represent  the  distribution  of  tactile  anesthesia;  Figs.  412  and 
413  that  of  loss  of  pain  and  temperature  sense.     (Dejerine.) 

Increasing  sensitiveness  is  also  frequent,  so  that  the  patient  dreads 
the  cold,  or  draughts,  or  sudden  shocks,  or  the  clothing,  not  only  on 
account  of  the  abnormal  sensitiveness,  but  because  these  may  bring 
on  the  severer  pains.  Tactile  anesthesia  follows  this  and  is  a  direct 
resultant  of  the  degenerative  process  in  the  root  area.  These  are  also 
irregularly  distributed. 

Other  sensory  functions  also  become  involved.  The  pain  sense 
may  become  lost  in  irregular  areas.  Retardation  in  the  carrying  of 
pain  impulses  is  present  at  times.  Complete  loss  is  a  sign  of  a  com- 
plete lesion.  ]\Ialoney  gives  three  signs  of  a  partial  lesion:  1,  a  raised 
sensory  threshold;  2,  delayed  perception;  and  3,  a  sensation  of  imper- 
fect contact.  There  may  also  be  numbing  or  loss  of  heat  and  cold 
sensibility.  Bony  sensibility  as  tested  by  the  tuning-fork  also  may 
be  absent  and  shows  irregular  distribution.  In  general,  deep  sensibility 
is  more  profoundly  affected  than  epicritic  sensibility.  ^Nlaloney  states 
that  this  is  due  to  the  fact  that  the  vegetative  nervous  system  is 


742  NEUROSYPHILIS 

largely  concerned  in  deep  sensibility  and  is  the  sensory  system  mainly 
affected  in  tabes. ^ 

Since,  as  has  been  pointed  out,  Ranson-  identifies  the  cutaneous 
"  protopathic"  system  of  Head  with  certain  unmyelinated  fibers  of 
the  posterior  spinal  roots,  this  conception,  as  maintained  by  Maloney, 
is  probably  the  correct  one.  In  1912  Ranson  published  his  discovery 
of  unmyelinated  fibers  in  the  dorsal  roots.  These  are  mostly  cutaneous 
nerves,  and  they  practically  all  enter  the  cord  in  bundles  which  lie 
laterally  to  the  myelinated  fibers  of  the  same  root.  The  former  are 
called  by  Ranson  the  "lateral  division"  of  the  dorsal  root.  A  much 
smaller  number  of  myelinated  fibers  is  found  also  in  this  lateral  divi- 
sion. All  the  neurons  of  the  lateral  division  run  up  or  down,  in 
Lissauer's  tract,  a  very  short  distance — usually  less  than  a  segment. 
That  is  to  say,  these  fibers  run  into  the  gray  matter  at  or  near  the 
level  at  which  they  enter  the  cord.  Their  intraspinal  course  suggests 
at  once  that  they  are  the  fibers  of  pain  and  temperature  sensations, 
since  it  is  known  that  the  afferent  impulses  underlying  these  sensations 
pass  through  the  gray  matter  as  soon  as  they  reach  the  cord. 

Ataxia. — ^The  most  prominent  sign  of  the  sensory  involvement  just 
noted  is  seen  in  the  gradually  (sometimes  suddenly)  developing  ataxia. 
The  fibers  conducting  the  impulses  from  the  joints  and  the  muscles 
to  the  chief  organ  for  their  coordination,  the  cerebellum,  are  degener- 
ating, and  there  results  an  imperfect  knowledge  of  the  position  of  the 
joints  and  of  the  states  of  muscular  tension  necessary  to  the  proper 
performance  of  motor  functions.  There  results  a  hypotonus  and  sway- 
ing of  the  arms  in  the  finger-nose  test  and  finger-finger  test,  and  of 
the  legs  in  the  knee-heel  test,  and  in  walking.  The  patients  are  par- 
tially or  completely  unaware  of  where  their  limbs  may  be,  and  are 
unable  to  control  the  same,  save  to  a  certain  degree  through  other 
avenues,  namely,  the  eyes.  iVccording  to  Maloney  this  unawareness 
is  due  to  the  suppression  by  the  pysche  of  the  feeble,  imperfect  and 
delayed  postural  images  which  arise  from  the  musculature  in  the  area 
of  the  affected  nerve  roots  and  which  owing  to  the  misleading  nature 
of  their  information  are  suppressed  in  favor  of  vision.  Hence  with 
closed  eyes  all  of  these  signs  of  ataxia  are  markedly  increased.  With 
this  great  uncertainty  of  movement  there  is  no  muscular  paralysis,  and 
little  loss  of  muscular  strength,  save  as  the  patient  generally  becomes 
weaker. 

Gait  disturbances  are  accompaniments  of  the  ataxia,  and  are 
extremely  characteristic.  They  were  described  by  Schermer  as  early  as 
1819.  In  the  early  stages  the  patient  notes  a  difficulty  in  going  up  and 
down  stairs,  or  finds  himself  insecure  on  uneven  surfaces.  He  stumbles 
and  at  times  falls.  At  night  he  finds  it  more  difficult  to  get  about,  and 
he  soon  notices  that  he  must  keep  his  eyes  glued  to  his  legs  or  the 
surface  on  which  he  is  walking  if  he  is  to  be  able  to  control  them. 
Later  he  must  walk  with  a  cane,  and  his  legs  are  thrust  somewhat 

>  Maloney:     Locomotor  Ataxia,  New  York,  1917. 
2  Am.  Jour.  Phys.,  1916,  xl,  571, 


TABES  743 

wider  apart,  are  thrown  somewhat  irregularly  forward,  and  are  then 
brought  to  the  ground  with  a  sharp  stamp,  the  knee  being  stiffened 
or  even  bent  slightly  backward — overextended — at  times  so  much  so 
as  to  cause  him  to  fall. 

His  step  becomes  quicker  and  more  stamping,  until  he  reaches  a 
point  where,  unless  he  has  trained  himself  to  walk  anew,  he  becomes 
bed-ridden.  Maloney  has  demonstrated  that  the  preataxic,  ataxic, 
and  bed-ridden  stages  are  not  so  much  stages  of  structural  demolition 
as  stages  of  mental  deterioration,  stages  of  loss  of  cerebral  control  over 
voluntary  movement.  The  manner  of  rising  from  a  chair  or  sitting 
down  soon  becomes  very  characteristic.  The  ataxia  in  the  upper 
extremities,  which  is  usually  less  than  that  in  the  lower,  affects  the 
patient's  WTiting,  the  buttoning  of  the  clothes,  his  dressing,  etc.  His 
hands  are  constantly  moving — one  finger  and  then  another  is  raised  or 
lowered,  or  the  WTist  turned.  Oppenheim  has  called  particular  atten- 
tion to  these  spontaneous  movements,  which  closely  resemble  a  static 
ataxia,  and  are  found  throughout  the  body. 

Other  muscles  naturally  may  be  involved  in  the  ataxia,  particularly 
those  of  the  face,  mouth,  tongue,  larynx,  pharynx  in  which  case 
speech,  singing,  swallowing,  etc.,  are  affected.  Many  patients  die  of 
aspiration  pneumonia  through  ataxia  in  the  swallowing  apparatus. 

Romberg's  sign  is  another  result  of  the  loss  of  position  sense.  It 
may  be  an  early  sign,  but  is  more  apt  to  develop  later  in  the  dis- 
ease. Many  patients  without  well-developed  Romberg  are  unable  to 
balance  themselves  on  one  foot,  and  further,  a  mild  Romberg  may 
be  more  readily  demonstrated  by  having  the  patient  bend  slightly 
forward. 

Tendon  i?e/?e.T^5.— Westphal  first  emphasized  the  importance  of  the 
diminution  or  loss  of  the  tendon  reflexes — notably  of  the  knee-jerk 
and  the  Achilles  reflex.  These  belong  among  the  initial  symptoms  in 
the  larger  number  of  cases.  The  knee-jerk  may  be  first  diminished 
on  one  side,  best  demonstrable  bv'  the  Jendrassik  method,  or  lost,  and 
this  for  years,  perhaps,  before  the  development  of  a  complete  Westphal 
phenomenon.  The  Achilles-jerk  is  lost  in  a  similar  manner  and  not 
infrequently  even  before  the  loss  of  the  knee-jerk  (Babinski  method). 

These  tendon-reflex  changes  are  all  referable  to  the  degenerations 
in  the  root  zones  and  sensory  columns. 

Cranial  Nerve  Involvement. — Any  one  or  all  of  the  cranial  nerves  may 
be  implicated.    Loss  of  smell  is  rare. 

Puinllary  Reflexes. — Here  a  striking  phenomenon  is  observed. 
Patients  with  tabes — as  with  many  other  s\philitic  affections  of  the 
medullary  or  midbrain  region — show  a  diminution  or  loss  of  the  pupil- 
lary light  reflexes,  without  any  loss  of  the  reflex  of  convergence  or  of 
accommodation.  This  is  the  Argyll- Robertson  phenomenon.  It  is 
present  in  over  60  per  cent,  of  the  cases,  and  may  be  present  for  many 
years  without  other  symptoms.  The  pupils  are  apt  to  be  at  first 
irregular  in  size,  and  also  not  infrequently,  in  shape.  The  light  reaction 
is  at  first  less  prompt — usually  in  one  eye  before  the  other — later 


744 


NEUROSYPHILIS 


both  eyes  are  involved.  Myosis  in  marked  degree  is  then  apt  to 
develop.  Loss  of  the  consensual  light  reflex  is  one  of  the  earliest 
signs  of  this  pupillary  change  ( Weiler) .  The  sympathetic  dilatation  of 
the  pupils  is  also  soon  diminished  or  lost. 

Optic  nerve  changes  are  frequent  and  may  occur  early.  There  is  an 
irregular  gray  atrophy,  with  narrowing  of  the  macular  vessels  and 
gradual  narrowing  of  the  visual  field,  first  for  colors  and  then  blind- 
ness, partial  or  complete,  with  advanced  optic  atrophy. 

Ocular  Palsies.- — These  very  frequently  belong  to  the  early  symptom- 
atology and  are  often  fleeting.  Persisting  ocular  palsies  are  more 
often  encountered  in  the  later  stages  of  the  disease.  These  palsies  in 
tabes  occur  quite  irregularly  and  show  themselves  as  atonic  ptoses, 
irregular  diplopias — from  fourth  or  sixth  or  third  nerve  affections — 
and  rarely  show  signs  of  a  complete  ophthalmoplegia,  internal  or 
external. 


Fig.  414. — Tabes.     Third  and  sixth  nerve  palsy. 

The  trigeminus  involvement  may  show  not  only  neuritic  pains  as 
already  noted,  but  also  trophic  disturbances  of  the  cornea,  herpes, 
irregular  development  of  the  bony  parts,  etc.  The  motor  root  may 
also  be  partly  paralyzed. 

Facial  palsy  occurs,  but  not  often. 

The  acoustic  is  occasionally  involved  in  both  cochlear  and  ves- 
tibular branches,  producing  unilateral  or  bilateral  deafness,  or  vestib- 
ular nystagmus,  Meniere-like  crises,  etc.    (Bonnier's  syndrome.) 

The  vagus  and  accessory  nerves  are  frequently  implicated,  causing 
crises  already  noted,  and  also  slowing  of  the  pulse,  dyspnea,  tachyp- 
nea, etc.    Taste  is  not  infrequently  lost.    Bulbar  palsies  are  frequent. 


TABES 


745 


Visceral  Symptoms. — The  bladder  is  usually  in\'olved  at  some  stage 
of  the  disorder.  The  crises  have  been  referred  to.  Weakness  of  the 
bladder  is  the  most  usual  occiurence.  Cystitis  usually  develops  and 
is  a  cause  of  general  weakness.  Incontinence  is  not  infrequent.  Con- 
stipation is  also  frequent,  but  fecal  incontinence  not  common.  Loss 
or  increase  of  erotic  desire  is  an  early  sign;  impotence  is  common; 
continuous    priapism    unusual. 

Trophic  Symptoms. — These  may  involve  any  of  the  tissues  of  the 
body,  but  particularly  skin  and  bones. ^  Arthropathies  are  very  frequent, 
usually  occurring  after  the  disorder  is  well  advanced,  not  infrequently 
as  an  early  sjniptom.  The  knee-joint  is  the  joint  of  special  predilec- 
tion.    The  arthropathies  usually  develop  with  surprising  rapidity — with 

edema  and  swelling  but  none  of  the 
usual  signs  of  a  rheumatic  joint ;  then 
new  bone  formations  take  place,  with 
or  without  subluxation.  Almost  any 
joint  ma}'  be  affected,  even  the  jaw. 


Fig.  415. — Tabes.     Third  nerve  palsy. 


Fig.  416. 


-Tabetic  arthropathy  of 
the  rib. 


Fragility  of  the  bones  is  a  further  complication.  Perforating  ulcers 
of  the  feet  constitute  another  trophic  disorder.  These  trophic  changes 
constitute  further  evidence  to  the  fact  of  the  implication  of  the  vege- 
tative (protopathic)  fibers  in  tabetic  pathology. 

Muscular  Atrophies. — An  initial  neuritis  may  give  rise  to  an  early 
muscular  atrophy,  but  atrophies  are  not  common.^ 

Blood  Serum  and  Cerebrospinal  Fluid. — The  Wassermann  reaction 
of  the  blood  serum  is  positive  in  most  of  the  cases  of  tabes.  The  cere- 
brospinal fluid  is  positi^'e  in  from  60  to  80  per  cent.,  and  Plant  is  of 
the  opinion  that  it  will  be  found  to  be  more  often  so  with  improved 


*Flatow:     Die  trophischen  Storungen  bei  der  Tabes  DorsaUs,  Berlin  Thfesis,  1888. 
'See  Lapinsky:     Arch.  f.  Psych.,  40-42. 


746 


NEUROSYPHILIS 


technic.    Nonne  and  Hauptmann,  by  using  larger  quantities  of  serum, 
have  established  this.    It  seems  to  be  less  often  positive  than  in  paresis 


Fig.  417. — Tabetic  arthropathy  of  ribs. 


Fig.  418. — Tabetic  arthropathy  in  the  wrist. 

— which,  in  view  of  the  opinion  held  concerning  the  identity  of  the 
two  disorders  is  food  for  reflection  regarding  the  local  reaction  of  the 
brain  and  cord  proper. 


TABES 


747 


There  is  a  marked  lymphocytosis  of  the  fluid,  which  is  usually  found 
in  the  early  stages  of  the  disorder,  and  is  an  index  of  the  meningitic 
sjTDhilosis  usually  found. 


Fig.  419. — Tabetic  arthropathy  -with  destruction  of  the  joint  and  hjT)otonus. 


Fig.  420.— Charcot  joint. 

The  colloidal  gold  reaction  may  give  curves  resembling  paresis, 
especially  Avhen  there  is  some  mental  extension,  but  as  a  rule  the  curve 
is  not  classical. 


748  NEUROSYPHILIS 

Course  and  Variation. — Tabes  is  essentially  a  chronic  disorder,  usually 
mildly  progressive,  developing  fully  after  many  years.  In  a  small 
number  of  cases  it  runs  an  acute  course,  causing  death  within  a  few 
years.     In  others  it  is  stationary  and  in  this  sense  gets  well. 

Duchenne  (1858-1859),  in  his  memorable  description,  made  a  divi- 
sion into  three  stages,  which  have  been  somewhat  modified.  The 
periods  usually  considered  at  the  present  time  are  as  follows:  (1) 
Prodromal  or  preataxic  stage;  (2)  ataxic  period;  (3)  paralytic  period. 

Such  a  division  is  of  arbitrary  value  only;  no  two  cases  are  exactly 
alike.  The  preataxic  stage  may  last  many  years,  or  there  may  be  none, 
ataxia  and  paralysis  developing  with  extreme  rapidity.  The  symptoms 
are  so  many,  and  the  times  of  their  appearance  so  variable,  that  a 
so-called  typical  course  is  the  exception  rather  than  the  rule,  yet  a 
not  unusual  course  is  one  extending  over  about  ten  years,  with  two 
or  three  years  of  pains,  with  or  without  crises,  then  the  gradual  develop- 
ment of  the  loss  of  knee-jerks;  Argyll-Robertson  pupils,  gradually 
increasing  difficulty  in  walking,  worse  in  the  dark,  Romberg — then 
the  patients  are  confined  to  bed,  and  then  the  stage  of  paralysis  and 
atrophies.  Maloney  correctly  denies  the  existence  of  the  paralytic 
stage  and  rightly  calls  it  the  surrender  stage,  the  stage  in  which  the 
ataxic  will  not  pay  the  tax  in  effort  which  movement  demands. 

Death  results  from  the  disease  itself,  or  from  complicating  disorder 
— bulbar  accidents  causing  pneumonia,  laryngeal  choking,  cardiac 
syncopes,  kidney  complications,  very  often;  intercurrent  disease  such 
as  tuberculosis,  in  large  part,  pneumonia,  t}^hoid,  and  erj^sipelas  in 
smaller  percentages. 

Prognosis  is  always  most  sinister.  Stationary  cases  are  known,  but 
a  question  concerning  diagnosis  may  be  raised  respecting  the  cases 
in  the  older  literature.  With  the  newer  objective  Wassermann  and 
cytological  symptoms  it  is  to  be  seen  whether  such  stationary  cases 
exist.  Since  such  are  found  as  paretics,  it  is  not  improbable  that  the 
same  will  hold  true  for  tabes. 

The  patients  with  severe  bladder  complications  usually  do  badly. 

The  duration  of  life  has  varied  from  six  months  to  thirty  and  more 
years.    The  general  average  runs  between  ten  to  fifteen  years. 

Forms. — Certain  types  are  worthy  of  special  mention  as  forms, 
these  are: 

1.  Juvenile  tabes,  like  juvenile  paresis,  occurs  in  children  from 
five  to  ten  years  of  age,  or  in  young  adults  apparently  up  to  about 
twenty-five  years.  The  higher  age  incidence  is  rare.  The  pupillary 
signs  are  early,  and  the  bladder  is  soon  involved.  Ataxia  and  paralysis 
then  develop.  The  objective  serological  and  cytological  changes  are 
usually  positive — the  .number  of  cases  examined  is  as  yet  too  small  to 
permit  wide  generalizations,  but  s>;philis  of  the  parents  is  an  essential. 

2.  Late  Tabes. — The  appearance  of  the  disorder  after  the  age  of 
fifty  is  rare,  yet  cases  are  recorded  in  which  the  initial  signs  have 
come  on  as  late  as  seventy  years.  One  case  is  on  record  of  infection 
at  twenty,  tabes  at  fifty-nine.    After  all  it  is  mostly  a  questioa  of  when 


TABES  749 

infection  takes  place — since  cases  of  infection  at  eighty  or  over  are 
known,  it  is  practically  impossible  to  determine  the  tabes  age  limit. 
No  noteworthy  symptomatic  variations  are  knowTi  in  late  tabes. 

3.  Rapid  Tabes. — Here  the  disease  advances  very  rapidly,  rendering 
the  patient  incapacitated  in  a  few  months,  with  death  as  a  result  of 
of  the  paralyses.  The  paralyses  and  atrophies  occupy  the  foreground 
in  the  picture,  the  ataxias  being  less  prominent.  The  pupillary  signs 
are  present.    Death  may  take  place  within  six  months. 

4.  Sloio  Cases. — ^These  are  the  more  usual  cases  already  described. 

5.  Stationary  or  Benign  Cases. — A  particular  type  in  which  blind- 
ness comes  on  early  and  which  follows  a  benign  course  was  fc'rst 
described  in  1881  by  Benedikt.  Dejerine  and  Martin  {These  de  Berne, 
1890)  called  attention  to  the  fact  that  it  is  rare  to  find  a  case  of  tabes 
beginning  with  blindness  that  advances  to  the  second  stage. 

Furthermore,  amaurotic  tabetic  cases  seem  to  have  fewer  pains. 
The  pathological  features  are  the  same  as  in  other  cases,  save  as  to 
extension,  and  the  explanation  of  this  variation  is  difficult  to  find. 
A  certain  diminution  in  the  general  symptoms  in  amaurotic  tabes  has 
been  observed.^ 

Diagnosis. — ^Little  difficulty  exists  after  the  development  of  the 
xALrgyll-Robertson  pupil,  lost  knee-jerks,  ataxias  and  Romberg.  Prac- 
tically the  only  differential  at  this  stage  is  a  polyneuritic  process, 
principally  of  alcoholic  origin.  Here  the  pupillarv'  disturbances  are 
less  in  evidence,  but  can  occur,  and  if  present  there  are  usually  more 
grave  cerebral  symptoms  and  a  diagnosis  of  taboparesis  is  more  in 
question.  The  serological  and  cytological  results  determine  a  diag- 
nosis almost  at  once.  Still  the  most  difficult  cases  to  differentiate 
are  those  of  alcoholism  complicated  with  syphilis.  Where  the  alco- 
holism causes  a  pseudotabetic  picture  and  the  syphilis  gives  its 
serological  and  cytological  findings  with  slight  meningitis  affection 
as  its  only  spinal  or  cerebral  concomitant,  the  cases  are  diagnosed 
only  with  the  greatest  of  difficulty. 

Other  toxic  pseudotabes  ofi^er  few  difficulties — such  as  those  due  to 
ergot,  diabetes,  lead,  pernicious  anemia  and  the  infectious  toxemias. 
Here  the  pupillary  signs  are  absent  for  the  most  part  and  the  serum 
and  spinal  fluid  normal. 

Certain  cerebellar  atrophies  and  new  growths  cause  symptoms 
closely  resembling  those  of  tabes,  but  the  gait  is  more  widely  sway- 
ing, the  individual  movements  have  less  ataxia,  the  absent  knee- 
jerks,  and  Argyll-Robertson  are  wanting,  and  there  are,  moreover, 
to  be  found  the  definite  signs  of  cerebellar  involvement  in  the  nys- 
tagmus and  its  alterations  to  the  Barany  vestibular  tests.  Normal 
serum  or  cord  findings  are  to  be  expected. 

INIultiple  sclerosis  can  occasionally  cause  a  tabetic  syndrome  if  a 
patch  should  involve  the  sensory  neurons  but  here  the  other  symptoms, 
the  nystagmus,  the  signs  of  spasticity,  masked  by  the  h^-potonus, 

'  Moloney;     Jour,  of  Nerv.  and  Ment.  Dis.,  1914,  vol.  xli, 


750  NEUROSYPHILIS 

Babinski  phenomena,  etc.,  should  afford  the  clue.  Rare  sclerotic 
patches  in  the  pons  and  midbrain  regions  have  caused  unilateral 
Argyll-Robertson  pupillary  pictures. 

Syringomyelia  occasionally  develops  with  an  initial  tabetic  picture, 
but  soon  the  classical  dissociation  shows  itself,  and  permits  a  diagnosis. 
The  blood  and  spinal  fluid  findings  are  also  to  be  reckoned  with. 

Hysteria  as  an  astasia  abasia  occasionally  causes  difficulty,  but 
here  careful  examination  can  exclude  the  entire  tabetic  symptom- 
atology. 

Pseudotabetic  neuritides  and  eye  signs,  i.  e.,  irregular  pupils,  gastric 
crises,  may  occur  in  masked  myxedema  (hypothyroidism)  of  later 
years.     Cauda  equina  lesions  may  cause  tabes-like  gaits. 

Pathology  and  Pathogenesis. — Both  Ollivier  d'Angiers  and  Cruveilhier 
gave  descriptions  of  the  general  gross  anatomy  and  both  recognized 
the  sclerosis  of  the  posterior  columns.  Todd  associated  the  sclerosis 
with  the  ataxia.  Burdon  and  Luys  (1861)  called  attention  to  the  rela- 
tion of  the  sclerosis  of  the  posterior  roots  and  the  posterior  column 
sclerosis,  since  which  time  the  development  of  knowledge  concerning 
the  pathological  processes  in  tabes  have  been  most  actively  studied, 
although  be  it  said,  without  yet  arriving  at  general  uniformity. 

Seen  with  the  naked  eye,  the  cord  is  usually  markedly  atrophied, 
hence  the  origin  of  the  old  term  consumption  (tabes);  the  posterior 
roots  are  atrophied,  sometimes  more  marked  in  one  region  than 
another.  The  diminution  in  volume  of  the  cord  also  varies  in  places, 
being  more  pronounced,  as  a  rule,  in  the  dorsal  and  sacrolumbar  regions 
than  the  cervical,  and,  as  Cruveilhier  noted,  the  atrophy  preponderates 
markedly  in  the  posterior  columns.  The  pia  and  arachnoid  are  some- 
what swollen  but  translucent  with  slight  opalescences  or  cloudiness. 
Meningeal  involvement  is  very  frequent;  a  fact  brought  out  more  in 
recent  years,  and  in  strict  conformity  with  the  findings  of  the  cellular 
contents  in  the  cerebrospinal  fluid. 

Throughout  the  entire  length  of  the  cord  one  finds  a  graymg  dis- 
coloration, the  margins  are  slightly  sunken  below  the  normal  level, 
and  the  discolored  areas  are  harder  to  the  touch.  The  atrophy  seems 
to  cease  with  the  sensory  medullary  nuclei — save  in  those  cases  where 
cranial  nerves  are  markedly  affected  when  irregular  atrophies  are 
encountered  in  the  upper  sensory  neurons. 

Histologically  the  picture  is  fairly  uniform.  There  is  a  mild  inflam- 
matory thickening  of  the  pia  and  arachnoid  with  lymphocyte  and 
plasma-cell  infiltrates.  This  leptomeningitis  varies  considerably  in  its 
localization,  and  in  its  intensity,  and  the  vessels  are  not  infrequently 
involved.  The  whole  process  closely  approaches  that  of  a  syphilitic 
meningitis,  but  is  less  intense,  and  is  rarely  accompanied  by  the 
presence  of  spirochetes. 

The  posterior  roots  are  irregularly  atrophied ;  the  sacral  and  lumbar 
roots  may  not  show  a  single  unchanged  fiber,  whereas  the  cervical 
roots  ar  •  less  involved,  save  in  the  case  of  cervical  tabes,  where  the 
reverse  holds  true. 


TABES 


751 


The  posterior  columns  show  the  most  uniform  lesions.  These  are 
greatly  diminished  in  volume.  There  is  a  gradual  degeneration,  often, 
however,  more  intense  on  one  side  than  the  other.  The  atrophy  is 
not  universal,  for  many  fibers  are  intact.  These  are  usually  collaterals, 
originating  within  the  cord  itself.  The  direct  fibers  in  the  columns 
of  Goll  and  Burdach,  from  the  degenerated  posterior  roots,  are  those 
chiefly  involved.  A  great  deal  of  variability  exists  as  to  the  respective 
distribution  of  the  atrophic  degenerated  fibers,  but  such  bear  a  direct 
proportion  to  the  distribution  of  the  degenerated  posterior  roots. 
Certain  portions  of  the  cord  escape,  due  to  anatomical  reasons;  these 
are  more  particularly  the  commissural  zones,  Flechsig's  centrum  ovale, 
Gombault  and  Phillippe's  triangle  in  the  lumbosacral  region,  the 
comucommissural  zones,  Schultze's  comma  tract  and  the  fascicles  of 
Hoche.     (See  Plate  X.) 


Fig.  421. — Tabetic  changes  in  cord  in  paresis. 

As  a  result  of  the  disappearance  of  the  fibers  a  secondary  neuroglia 
infiltration  takes  place.  This  consists  of  glia  with  fine  prolongations, 
and  also  spider  cells.  In  the  cases  of  taboparesis  one  finds  lympho- 
cytes and  plasma  cells  within  the  cord  substance.  With  a  limited 
tabetic  process  they  are  not  usually  encountered.^  Many  ganglion 
cells  of  the  posterior  horns  show  degenerative  changes.  They  are 
diminished  in  size,  there  is  definite  chromatophilia,  or  vasculization 
and  other  signs  of  degeneration.  The  cells  of  the  column  of  Clarke 
are  diseased  only  to  a  slight  extent. 

In  tabes  with  cranial  nerv^e  signs  the  degeneration  can  be  traced  in 
the  medulla  and  pons  with  secondars-  atrophy  of  the  sensorj-  nuclei. 
Atrophy  of  the  optic  nerve  is  not  infrequent. 

Jendrassik,  SchaftVr,  Epstein  and  Krauss  have  also  shown  that 
the  brain  cortex  is  not  uninvolved  even  in  t\^)ical  tabes.  There  is 
a  diminution  in  the  nerve  fibers,  and  alterations  in  the  vessels  and  pia 
— there  may  be  lymphocyte  and  plasma-cell  infiltration  even  iti  the 

•Alzheimer:     Nissl's:  Arb.,  i,  14. 


752 


NEUROSYPHILIS 


absence  of  pronounced  mental  symptoms.  Cerebellar  degenerations 
are  also  kno^\^l.  The  anterior  horn  cells  do  not  always  escape.  In 
the  patients  with  pronounced  atrophy  many  motor  cells  of  the  anterior 
horns  are  found  degenerated.  Similar  changes  are  knoA\ai  where  there 
are  bulbar,  lar;yTigeal  or  other  cranial  nerve  palsies.  The  cerebrospiral 
fluid  also  shows  the  efTects  of  the  mild  inflammatory  process  by  its 
increased  lymphocytosis;  a  count  of  over  10  cells  to  the  cubic  milli- 
meter (Fuchs  and  Rosenthal  chamber)  is  to  be  regarded  as  positive, 
50  to  100  are  not  unusual.    Plasma  cells  are  also  to  be  found. 


Spinal  Cord 


Dura 


AnttriorRoot 


4rac7nioid. 


Posterior  Root. 


Anterior  Hoot 

Muj-iiis 


Gan^h'o?/ 


JiootZo7ie 
iffVolvedinJaOes. 

I  ^^--\ -Posterior Foot 

JVeuritis. 


Fig.   422. — Nageotte's  scheme  representing  the  location  of  the  chief  lesion  of  tabes 
in  the  posterior  root  zone. 

Serologically  a  positive  Wassermann  is  to  be  expected,  although 
here  the  number  of  positive  results  would  seem  to  fall  below  that 
obtained  in  paresis.     Possibly  paresis  indicates  a  more  acute  process. 

Nonne  phase  I  reaction  is  also  frequently  positive. 

The  posterior  ganglia  are  also  aft'ected  in  many  cases,  although 
not  uniformly.  There  is  atrophy  and  destruction  of  the  cells,  and 
proliferative  inflammatory  exudates  of  the  capsule.  The  changes  are 
not  sufficiently  constant  to  permit  one  to  assume  that  the  primary 
part  of  the  disorder  is  located  in  the  posterior  ganglia. 

The   peripheral   nerves    are    also    frequently    found    degenerated; 


SYPHILITIC  MEN  IN  GO  MYELITIS  753 

Xonne  says  constantly;  thus  showing  the  complete  degeneration  of 
the  sensory  neuron,  central  as  well  as  peripheral. 

Vegetative  System. — ^This  shares  in  the  general  destruction  and 
shows  particularly  in  the  regions  involved  by  gastric  or  other  visceral 
crises. 

Pathogenesis. — Unanimity  of  opinion  has  not  yet  been  reached. 
The  various  h^-potheses  evoked  have  upheld  vascular  (meningeal). 
medullar}',  radicular  and  neuritic  theories,  not  to  mention  the  idea 
of  a  prlmars'  system  intoxication.  The  general  tendency  is  to  regard 
as  fundamental  a  primary-  s\'philis  involving  particularly  the  posterior 
roots — a  modified  s^-philitic  radiculitis  as  the  most  constant  feature  in 
the  pathogenesis.  The  process  is  one  of  chronic  specific  h\i)erallergic 
reaction  in  which  there  is  (1)  an  involvement  of  the  posterior  radicular 
fibers  and  of  the  peripheral  nerves;  (2)  an  extension  to  the  vegetative 
nervous  system  fibers  both  s^-mpathetic  and  autonomic;  (3)  to  the 
motor  nerve  system. 

Concerning  the  nature  of  this  hyperallergie,  if  present,  little  is  kno^^•n.^ 

7.  Syphilitic  Meningomyelitis. — Xonne  has  devoted  a  large  portion 
of  his  noted  monograph  to  a  consideration  of  the  lesions  of  s^-philis 
of  the  spinal  cord  and  its  membranes.  This  is  a  general  indication 
of  their  extreme  frequency,  yet  most  patients  showing  s^-philitic  lesions 
of  the  cord  also  show^  signs  in  the  brain  or  its  meninges.  They  are 
nearly  all  examples  of  cerebrospinal  s}-philis.  For  practical  purposes, 
however,  it  has  been  found  of  value  to  arbitrarily  divide  this  large 
conglomeration  and  discuss  it  under  two  captions:  cerebral  s^■philis 
and  spinal  s^-philis  or  meningomyelitis.  This  means  simply  that  one 
is  dealing  with  cerebrospinal  s^-philis  with  predominant  cerebral  and 
minor  spinal  symptoms  on  the  one  hand,  or  with  predominant  spinal 
and  nerve  root,  with  less  prominent  cerebral  signs  on  the  other.  It 
again  seems  advisable  to  accentuate  the  purely  pragmatic  character 
of  all  such  classifications. 

Sjrmptoms. — In  considering  meningomyelitis  as  a  unit,  further 
emphasis  may  be  put  upon  separable  s\Tnptom  groups.  ^Yithin  this 
conglomeration  again  clinical  neurology  shows  four  fairly  clear 
tendencies: 

1.  Syndromes  due  to  pronounced  meningeal  implication. 

2.  SjTidromes  due  to  root  and  cauda  equina  disease.  Radiculitis 
and  neuritis.  Syphilitic  osteoarthritis  of  the  spine  is  a  frequent  factor 
in  these. 

3.  Myelitic  syndromes  due  to  indiscriminate  transverse  disease. 

4.  Syndromes  of  less  extensive  transverse  lesions  and  fiber-tract 
isolation.     System  sjTidromes. 

A  combination  of  all  would  make  a  complete  meningomyelic  syn- 
drome. This  is  a  not  unusual  picture  in  a  rapidly  developing  case;  in 
its  more  chronic  course  the  emphasis  seems  to  be  laid  upon  one  or 
another  of  the  just  mentioned  groupings. 

>  See  Head:  Brain,  1913.     Dind:  Rev.  d'Urologie,  1914. 
48 


754 


NEUROSYPHILIS 


These  are  characteristic  SAiidromes  of  the  early  and  secondary 
stages  of  s;s^hilis.  The  syndromes  may  develop  within  a  few  months 
after  infection,  or  only  come  on  after  many  years.  In  the  former 
case  the  acute  myelitic  changes  are  frequent,  also  root  lesions  (many 
neuralgias,  sciatica,  etc.).  The  later  developing  cases  show  more 
the  systemic  lesions  and  gradually  advancing  meningopathies  (later 
secondary  meningitis)  with  compression  (spastic)  phenomena. 

In  all  one  expects  to  obtain  a  positive  Wassermann;  cerebrospinal 
fluid  Wassermann  may  be  negative  in  early  cases,  but  usually  becomes 
positive;  pleocytosis  is  frequent — often  the  cell  count  being  very  high, 
always  indicating  the  grade  of  meningeal  involvement.  The  lympho- 
cytes are  sometimes  found  before  the  stage  of  roseola;  are  abundant 
in  the  active  secondary  stages  and  less  frequent  in  the  tertiary  stages 
of  a  meningomyelitis.    The  protein  content  varies  considerably. 


•Fig.  423. — Meningomyelitis.     Radiculitis.     Degeneration  of  Burbach's  columns. 


1.  Meningeal  Syndromes. — Severe  pains  are  signs  of  meningeal 
involvement.  They  shoot  across  the  shoulder-blade,  in  the  neck, 
across  the  hips,  dart  down  the  arms  or  legs  and  cause  a  stiffness  of  the 
neck,  the  shoulders  and  the  thighs.  The  spinal  column  is  usually 
sensitive  to  pressure,  and  to  percussion,  and  local  intensities  may 
show  both  these  signs,  and  also  the  peripheral  signs  of  a  definite  zone 
localization. 

Pain  is  frequently  preceded  by  paresthesise,  like  the  crawling  of 
ants,  numbness  and  coldness.  There  is  a  gradual  increase  in  the 
reflex  excitability  of  the  cord,  due  to  pressure  and  evidenced  by 
increased,  knee-jerks,  possible  Babinski  sign,  Oppejiheim  or  Chaddock 


SYPHILITIC  MENINGOMYELITIS 


755 


signs.  When  pressure  is  exerted  in  the  sacral  segments  bladder  and 
rectal  disturbances  are  frequent. 

With  chronic  meningeal  thickening  these  pressure  symptoms 
increase  markedly  and  spastic  paretic,  phenomena  augment,  especially 
when  gummata  add  their  special  pressures.  Gummata  may  give  rise 
to  a  "cord  tumor"  syndrome. 

2.  Root  Syndromes. — Here  pain  is  frequent  and  neuritic  atrophies 
appear.  Sensory  losses  of  a  root  distribution  are  in  evidence.  Many 
show  the  characteristic  reversal  of  epicritic  touch  loss  being  less  exten- 
sive than  protopathic  (vegetative  fibers)  pain  loss  as  pointed  out  by 
Head  as  characteristic  of  radicular  localizations. 

The  atrophy  of  the  muscles  also  follows  the  radicular  distribution. 


Fig.  424. 


-Radicular  distribution  of  sensory  loss  in  tabes,  particularly  to  tactile  pain 
and  thermal  sensibility.     (Dejerine.) 


Many  obstinate  neiu-algias  are  due  to  syphilitic  radicular  disease. 
Possibly  one-half  of  the  sciaticas  are  of  this  nature.  Dejerine  has 
put  them  as  high  as  80  per  cent,  in  Paris.  A  ver}'  large  proportion  of 
the  brachial  neuralgias,  so  long  looked  upon  as  rheumatic  or  gouty, 
or  what  not,  are  due  to  a  syphilitic  root  meningitis. 

Neuritic  muscular  atrophy  from  pressure  on  the  anterior  roots  is 
further  complicated  by  pressure  on  the  anterior  horns  by  the  thick- 
ened meninges.  Thus  very  anomalous  atrophies  result.  When 
occurring  in  the  eighth  cervical  and  first  dorsal  region  one  obtains 
classical  Klumpke  paralysis  with  dilatation  of  the  pupil,  and  narrow- 
ing of  the  palpebral  fissure  of  the  aft'ected  side.  Lower  localizations 
result  in  intercostal  palsies,  back  muscle  atrophies,  hip  girdle,  thigh  or 
Cauda  lesions.  In  this  latter  situation  striking  dissociations  are  obtained, 
as  in  the  upper  arm  region,  and  radicular  sensory  disturbances  and 
lost  reflexes  are  the  rule  with  atrophies. 


756  NEUROSYPHILIS 

3.  Myelitic  Syndromes. — These  indicate  the  complete  involvement 
of  the  cord  and  also  point  to  intraspinal  vascular  disease,  rather  than 
to  a  meningeal  lesion.  Complete  flaccid  palsy  is  the  usual  result. 
This  is  combined  with  sensory  loss  as  well.  The  completeness  of  the 
sensory  loss  varies  considerably  and  indicates  the  severity  of  the 
lesion.  Absence  of  a  lymphocytosis  points  to  a  purely  vascular  and 
usually  focal  lesion  within  the  cord.  The  bladder  and  rectal  functions 
are  implicated  as  well. 


Fig.  425. — Pjiiliilitic  meningomyelitis  with  radicular  sj^mptoms,  atrophy,  etc. 

In  the  regressive  stage  an  increase  in  spasticity  marks  the  subsid- 
ence of  the  inflammatory  reaction  and  many  anomalous  syndrome 
mixtures  result.  This  phase  of  meningomyelitis  offers  abundant 
opportunity  for  very  heterogeneous  syndromes.  A  Brown-Sequard 
complex,  poliomyelitis,  tabetic  syndrome  with  atrophy,  amyotrophic 
lateral  sclerosis  syndrome — these  are  but  a  few  of  the  possible  com- 
binations. 

4.  System  Syndrcmes.— These  occur  not  so  much  as  residuals  of  the 
previous  myelitic  changes,  nor  as  due  to  meningeal  compressions,  but 
represent  disease  in  or  about  the  long  motor  tracts,  tractus  cortico- 


CONGENITAL  OR  HEREDITARY  SYPHILIS 


757 


spinalis,  especially.  They  give  rise  to  the  forms  of  primary  lateral 
sclerosis  (Erb),  some  combined  scleroses  and  particularly  to  clinical 
pictures  closely  resembling  multiple  sclerosis.  Combined  disease  of 
the  posterior  and  lateral  columns  is  quite  apt  to  be  syphilitic. 

Special  localization  of  one  or  more  of  these  forms  of  meningeal 
syphilis  gives  rise  to  the  special  forms  of  hypertrophic  cervical  pachy- 
meningitis, which  have  been  described  by  Joffroy  and  Charcot,  and 
to  Kahler's  disease. 

The  anterior  horns  may  be  predominantly  involved  usually,  however, 
as  a  result  of  a  transverse  myelitis  (Nonne)  giving  the  picture  of  a 
chronic  anterior  poliomyelitis. 


Fig.  426. — Pachymeningitis  hypertrophica  cervicalis. 

In  hypertrophic  cervical  pachymeningitis  an  enormous  thickening  of 
the  meninges  is  found  with  or  without  gummata  and  located  in  the 
cervical  region.  Here  root  and  compression  symptoms  are  present. 
Pains  in  the  neck  and  shoulder,  stiffness  of  the  cervical  spine,  shooting 
pains  down  the  arms.  Sensory  loss  ma\'  then  show,  particularly  to 
pin  prick  with  relatively  intact  sensibility  to  cotton-wool.  The  ulnar 
and  median  are  particularly  implicated.  Fibrillary  contraction  of 
the  muscles,  atrophy  and  loss  of  electrical  excitability  occiu*.  The 
special  type  of  deformity  known  as  preacher-hand  is  one  of  the  fre- 
quent expressions  of  the  involvement  of  the  brachial  plexus  in  the 
cervical  meningitis. 

8.  Congenital  or  Hereditary  Syphilis.— Effect  of  Hereditary  Syphilis. 
— Serological  studies  have  thrown  much  light  on  the  question  of  the 


758  NEUROSYPHILIS 

mode  of  transmission.  This  cannot  be  entered  upon  here.  These 
studies  as  particularly  carried  out  by  Plaut,  Mott  and  others  have 
shown  the  enormous  importance  of  transmitted  syphilis  in  the  per- 
nicious effects  upon  the  nervous  system.  Linser,  moreover,  has 
shown  that  two-thirds  of  the  children  of  syphilitic  parents  show  a 
positive  Wassermann  reaction,  although  much  fewer  show  signs  of 
congenital  syphilis. 

It  may  be  recalled  that  Fournier  stated  the  proportion  as  high  as 
98  per  cent.,  and  that  68.5  per  cent,  of  the  children  died.  This  does 
not  include  the  aborted  offspring.  Should  these  be  reckoned,  one 
could  obtain  a  true  idea  of  the  morbidity  of  syphilis  in  the  young. 
Hochsinger  reports  an  interesting  group  of  cases  in  this  connection. 
In  72  families  there  was  paternal  syphilis.  The  mothers  were  not 
syphilitic.  Seventy  mothers  gave  birth  to  307  children — 110  still- 
born, 166  syphilitic  and  31  healthy.  The  healthy  were  all  the  last 
born  save  in  four  instances. 

Of  the  children  of  tabetics,  one  obtains  the  same  story  from  Mott, 
Mendel  and  others.  Either  no  children,  many  abortions,  many  dead 
children,  few  living  and  no  one  knows  as  yet  the  fate  of  these.  Cer- 
tainly one-half  are  doomed  to  disease  and  disorder  of  the  nervous 
system. 

It  would  appear  that  the  common  effect  of  such  syphilitic  infection 
is  to  reduce  the  resistances  of  the  body  and  its  powers  for  full  develop- 
ment both  in  the  general  body  and  nervous  tissues.  Syphilis  dimin- 
ishes the  vital  energy  of  the  germ  plasm  prior  to  conjugation  and  can 
cause  pathological  variations  in  nervous  structures  just  as  it  can 
transmit  the  disease  through  the  germ  cells.  The  abundant  studies 
on  alcohol  and  its  influence  on  the  germ  cell  affords  an  analogy  in 
understanding  how  this  takes  place  with  another  type  of  toxemia. 

The  classical  formula  of  Fournier  seems  to  hold— abortion,  dead 
child,  early  death,  living,  healthy  child.  This  is  in  need  of  amend- 
ment; it  is  worse.  The  formula  reads:  Complete  sterility,  miscar- 
riage, abortion,  stillbirths,  children  dying  in  infancy  or  convulsions, 
marasmus,  meningitis,  hydrocephalus.  Then  follow  children  who  are 
comparatively  healthy,  but  who  in  later  life  develop  late  hereditary 
syphilis. 

A  study  by  Hochsinger  (1911)  says  that  of  208  children  of  syphilitic 
parents  who  had  been  under  observation  over  four  years,  89,  or  43 
per  cent,,  had  some  disease  of  the  nervous  system.  Of  these  there 
were  9  cases  of  hydrocephalus,  2  of  Little's  syndrome,  6  epileptics,  2 
paresis,  1  tabes,  6  Argyll-Robertson  pupil,  36  extremely  neurotic,  5 
hysterical,  14  chronic  headaches,  10  imbeciles.  This  is  in  strange 
contrast  to  the  statements  of  Jonathan  Hutchinson,  who,  but  a  few 
years  ago,  taught  that  nervous  syphilis  was  negligible. 

It  has  been  abundantly  shown  that  nearly  every  form  of  adult 
syphilis  of  the  nervous  system  can  be  encountered  in  hereditary 
syphilis  and,  as  Mott  well  says,  if  congenital  syphilis  were  not  so 
fatal  to  infant  life  the  number  of  people  suffering  from  syphilitic 


CONGENITAL  OR  HEREDITARY  SYPHILIS 


759 


disease  of  the  brain  would  be  appalling.  It  would,  therefore,  be  of 
little  service  to  repeat  what  has  aheady  been  written  regarding  ner- 
vous syphilis  of  adults  as  it  appears  in  children,  and  the  present 
discussion  will  be  limited  to  a  consideration  of  such  forms  of  juvenile 
nervous  syphilis  as  are  present  only  in  children.  These  are,  particu- 
larly, certain  forms  of  feeble-mindedness,  of    hydrocephalus,  ependy- 


*  i^ 


Fig.  427. — Congenital  syphilis: 


juveaile  paresis,  at  first  considered  as  "imbecile. 
(Mott.) 


mitis,  Friedreich's  ataxia,  piimary  optic  atrophy  and  encephalitides 
or  encephalomalacias,  leading  to  various  hemiplegic  syndromes,  often 
loosely  grouped  together  as  Little's  disease.  Juvenile  paresis  and 
juvenile  tabes  are  among  the  commonest  congenital  disorders  of  later 
infancy  or  adolescence. 

Congenital  syphilis  of  the  nervous  system  may  show  itself  before 
or  at  birth;  it  may  develop  in  earliest  infancy  or  in  adolescence;  it 
may  develop  as  late  as  twenty,  or  even  forty-two  to  forty-three  years 


Fig.  428. — Congenital  syphilis.  Three  miscarriages;  then  five  children  born  alive 
and  well.  Last  child  snuffles,  Hutchinson  teeth.  Did  well  in  school,  then  deteriorated, 
noisy,  maniacal;  thought  to  be  paretic.  Autopsy  showed  generalized  cerebrospinal 
gummatous  meningitis,  perivascularitis  and  endarteritis.     (Mott.) 


(Miiller).  How  long  may  the  virus  remain  latent,  finally  to  develop 
a  definite  s\-philitic  reaction?  As  yet  the  answer  will  depend  upon 
the  general  bias  of  the  answerer,  rather  than  upon  empirical  data. 
It  would  appear  from  the  evidence  at  hand  that  no  definite  age  limit 
can  be  put  upon  the  time  when  such  a  latent  factor  becomes  activated 
by  causes  as  yet  unknown.    That  an  activator  of  some  sort  plays 


760 


NEUROSYPHILIS 


a  role  in  such  disorders  as  tabes  and  paresis  seems  at  present  a  justi- 
fiable hypothesis.  When  a  newborn  child,  known  to  have  been 
syphilitic  biologically,  can  be  followed  throughout  life,  his  serum 
reactions  being  tested  from  year  to  year,  as  is  now  being  done,  then 
the  question  can  be  finally  decided. 

In  this  connection  a  line  may  be  devoted  to  the  subjects  of  con- 
genital syphilis  "unto  the  third  generation."  While  of  late  years 
doubts  have  been  accumulating  relative  to  this  matter,  certain  posi- 
tive cases  are  being  reported.  The  rationale  of  this  seems  plain  in 
view  of  the  observations  of  recent  years  made  by  Levaditi,  Bab  and 
others,  that  Treponema  pallidum  may  be  found  in  the  ovum,  and  in 
an  apparently  resting  stage  similar  to  the  resting  stage  known  for 
other  flagellate  protozoa  closely  allied  to  the  organism  causing  syphilis. 


Fig.  429. — Brain  of  a  congenital  syphilitic  idiotic  child. 


The  fact  of  the  whole  matter  is  that  opinions  and  statistics  relative 
to  congenital  syphilis  of  the  nervous  system,  supported  by  clinical 
observation  alone  and  uncontrolled  by  the  available  biological  tests, 
are  insufficient  approximations  and  very  insecure.  When  relied  upon 
for  negative  conclusions  they  are  harmful  to  the  advance  of  thought, 
and  detrimental  to  the  relief  of  sick  humanity. 

Notwithstanding  the  importance  and  often  the  strikingly  gruesome 
character  of  these  late-appearing  congenital  cases  the  attention  of  the 
practitioner  should  be  riveted  upon  the  numerically  preponderant  mis- 
carriages, stillbirths  and  early  syphilitic  deaths,  if  he  would  get  in  the 


CONGENITAL  OR  HEREDITARY  SYPHILIS  761 

right  attitude  toward  the  therapeutics  of  this  disease,  as  it  affects  the 
nervous  system. 

In  congenital  syphilis,  as  has  been  stated,  a  replica  of  what  has  been 
found  in  adult  syphilis  may  be  expected.  Pathologically  speaking, 
the  lesions  are  nearly  always  combined.  There  is  a  variable  compos- 
ite of  endarteritis,  of  leptomeningitis,  of  pachymeningitis,  gummata, 
large  and  small,  localized  or  infiltrating  gummatous  neuritis,  diffuse 
degenerative  changes  in  the  cells  of  the  spinal  cord,  in  the  basal 
ganglia  or  of  the  cortex.  Thus  the  clinical  pictures  are  apt  to  be 
conglomerate  and  almost  unanalyzable.  Those  more  accentuated 
trends  which  permit  a  nosological  term  will  be  considered  here. 

Hydrocephalvs. — As  a  result  of  congenital  syphilis  this  condition  has 
been  suspected  for  two  hundred  years.  Hasse,  in  1828,  Cruveilhier 
in  his  Atlas,  von  Rosen,  in  1862,  and  Yirchow  reported  definite 
examples  of  it.  It  arises  in  these  congenital  forms  largely  from 
syphilitic  disease  of  the  cerebrospinal  fluid-producing  structures — 
choroid,  ependyma,  or  from  definite  obstructive  factors  in  the  cere- 
bral foramina,  gummata,  vascular  swelling  obstructing  the  iter, 
etc. 

It  is  a  not  uncommon  sequel  of  congenital  svphilis  and  is  undoubt- 
edly more  frequent  than  is  realized.  In  Hochsinger's  series  of  362 
cases  of  congenital  syphilis  34,  or  nearly  10  per  cent.,  showed  hydro- 
cephalus. In  his  series,  which  affords  a  fairly  average  review  of  the 
situation,  the  hydrocephalus  began  three  to  eleven  months  after 
birth;  sometimes  it  was  fetal.  In  11  cases  there  were  no  nervous 
symptoms,  i.  e.,  up  to  the  time  of  reporting.  In  the  others  restless- 
ness, sleeplessness,  chronic  vomiting,  convulsions,  contractures, 
nystagmus  and  feeble-mindedness  were  the  objective  phenomena. 

The  more  usual  clinical  picture  is  that  of  a  child,  boy  or  girl,  from 
three  to  six  months  of  age,  who  following,  or  not,  an  insignificant 
blow  on  the  head  or  some  gastro-intestinal  or  bronchial  disturbance, 
develops  within  a  few  days  grave  cerebral  disturbances.  There  is 
great  irritability  and  sleeplessness,  screaming  and  kicking".  The  head 
is  usually  drawn  back,  the  eyes  and  the  fontanelles  are  apt  to  bulge 
somewhat.  Vomiting  is  frequent  and  there  are  signs  of  oculomotor 
involvement.  Internal  strabismus  from  paresis  of  the  external 
rectus  is  not  unusual.  It  is  frequently  preceded  by,  or  accompanied 
by,  nystagmus  and  irregular  pupils— often  not  responding  to  light. 

Pain  is  present,  as  the  child  cries  and  struggles,  and  not  infre- 
quently the  active  movements  of  the  arms — often  highly  spasmodic 
or  convulsive — seem  to  try,  in  a  blind  reflex  sort  of  way,  to  get  at 
and  brush  away  the  source  of  it,  i.  e.,  the  head,  pulling  the  hair, 
grasping  and  rolling  the  head.  Spasticity,  rigidity  and  other  signs 
of  intracranial  pressure  may  at  times  be  demonstrated.  As  a  rule 
the  temperature  is  only  slightly,  or  not  at  all  raised,  and  the  minor 
signs  of  an  epidemic  cerebrospinal  meningitis,  ?'.  e.,  herpes,  tempera- 
ture, flushed  and  spotted  skin  are  absent.  The  diagnosis  of  all  of 
these  infantile  meningeal  disturbances  is  fraught  with  much  difficulty. 


762  NEUROSYPHILIS 

Feeble-mindedness. — Syphilis  undoubtedly  plays  a  much  larger  role 
in  producing  mental  defectives  than  is  suspected.  The  early  statistics 
are  comparatively  worthless.  They  are  quoted  at  great  length  even 
in  modern  works  on  idiocy,  imbecility  and  the  like. 

The  more  correct  appreciation  of  this  chapter  on  syphilis  and 
feeble-mindedness  began  with  the  studies  of  Fournier  on  parasyphilis. 
Those  truths,  somewhat  uncontrolled,  were  forced  upon  him  by  his 
clinical  observations.  The  early  English,  German  and  American 
figures  varied  from  0.1  (Shuttleworth)  to  17  per  cent.  (Ziehen). 
Whereas  the  results  following  serological  investigations  start  with  the 
higher  figures  and  mount  upward,  in  some  cases  as  high  as  60  per 
cent.  The  American  figures  available  (Atwood  and  Clark)  showed 
that  20  per  cent,  of  the  idiots,  imbeciles  and  morons  at  Randall's 
Island,  New  York,  were  syphilitic. 

Intra-uterine  feeble-mindedness  is  more  or  less  a  contradiction. 
Those  children  whose  mental  defects  date  to  disease  going  on  in  the 
uterus  rarely  live.  Plant  expresses  the  opinion  that  feeble-minded- 
ness may  be  regarded,  so  far  as  syphilis  is  concerned,  as  the  result 
of  an  extra-uterine  syphilitic  disease  undergone  in  infancy.  In  some 
there  are  signs  of  an  acute  brain  disease.  Some  instances  of  recovered 
hydrocephalus  show  the  signs  of  havoc  in  their  inability  to  develop 
normally.  Many  others  show  no  acute  stage,  but  fail  to  develop. 
Many  recorded  observations  are  available  to  show  the  very  gradual 
development  of  mental  defect,  without  convulsions  or  fever,  alto- 
gether without  signs  of  organic  disease,  which  arrived  at  a  definite 
termination,  and  which  left  behind  entirely  stationary,  perhaps  even 
improvable,  idiots  or  imbeciles. 

That  type  of  hereditary  syphilitic  child  without  any  tangible 
disease,  formulated  by  Fournier — ^his  "enfants  arrieres" — ^which  is 
described  as  unintelligent,  simple,  silly,  limited  children,  always 
behind,  not  infrequently  shows  the  Wassermann  reaction. 

Again,  one  is  convinced  by  the  researches  of  others  that  mental 
defect  in  less-marked  grade,  or  more  properly  speaking,  along  more 
restricted  or  special  lines,  is  allied  with  this  broad  group  on  the  basis 
of  congenital  syphilis.  Thus  Nonne  reports  cases  of  general  irritable 
weakness  of  the  nervous  system.  The  patients  are  highly  excitable, 
are  extremely  nervous,  they  are  very  moody,  suffer  from  headaches, 
irregularities  of  appetite,  sudden  fits  of  passion — not  associated  with 
other  forms  of  epileptiform  analogies — and  for  whom  mercury  and 
the  iodides  work  wonders. 

Still  another  chapter  has  been  opened  in  this  hereditary  syphilis 
problem  in  its  relation  to  mental  defect.  It  concerns  many  so-called 
psychopathic  children.  These  children  are  bright,  but  they  show 
marked  ethical  defects.  Here  one  can  conceive  of  the  mental  defect 
in  terms  of  limited  cortical  control  to  the  affective  response  of  the 
sexuality  and  to  the  nutritional  instincts.  These  children  want  and 
take  without  going  around  by  the  circuitous  routes  devised  by  cultural 
standards.  Anatomically  one  can  posit  a  defect  of  certain  cortico- 
cortical  association  areas  on  the  basis  of  the  syphilitic  poisoning. 


TREATMENT  763 

Taking  the  whole  group  of  feeble-mindedness,  it  is  evident  that 
dinicall}'  one  cannot  pick  out  the  hereditary  syphilitic  child  in  all 
instances.  Indeed,  it  should  be  emphasized  that  too  much  weight  is 
given  to  the  anomalies  in  physical  structure — Hutchinson  teeth, 
saddle  nose,  strife  about  the  mouth,  prominent  veins,  scaphoid  scapula, 
etc. — if  one  rejects  those  who  from  the  heredosyphilitic  class  fail  to 
show  such  anomalies.  Xor  can  we  recognize  any  certainly  pathog- 
nomonic psychical  anomalies.  A  careful  neurological  examination 
frequently  aids  in  enlarging  the  group — particularly  in  the  study  of 
pupillary  anomalies.  The  cytological  tests  are  of  the  highest  impor- 
tance and  every  child  born  of  syphilitic  father  or  mother  should  be 
systematically  examined  by  these  cytobiological  methods. 

Treatment. — The  treatment  of  syphilis  of  the  nervous  system  appear- 
ing in  any  of  the  forms  previously  enumerated  is  often  extremely  sat- 
isfactory. In  fact,  at  times  one  might  say  the  results  are  often  too 
good,  as  the  speedy  relief  not  infrequently  leads  the  patient  to  forego 
further  treatment  or  causes  him  to  pursue  his  course  half-heartedly.^ 

Cerebral  syphilis  for  the  most  part  is  accompanied  by  active  spiro- 
chetes; hence  the  therapy  is  to  be  directed  against  this  organism. 
Mercury,  arsenic  and  iodine  are  the  best  available  spirocheticidal 
drugs.  Mercury  and  arsenic  are  active,  iodine  is  very  weakly  toxic, 
but  Neisser's  most  recent  studies  tend  to  show  that  it  has  actions 
other  than  that  usually  ascribed  to  it,  namely,  to  promote  the  taking 
away  of  breaking-down  syphilitic  tissue  or  its  product^ — its  so-called 
resorption  action,  probably  a  result  of  increased  sympathetic  stimuli 
to  the  bloodvessels  (see  h\'perth\Toidisms).  Xeisser  shows  that 
iodides  in  large  doses  are  toxic  to  spirochetes  as  well. 

The  treatment  of  nervous  syphilis  then  should  be  an  attempt  to 
follow  out  a  general,  antisj^hilitic  treatment  with  special  attention 
to  certain  structural  peculiarities  of  the  nervous  system.  These 
peculiarities  are  of  much  importance.  In  the  first  place,  small  lesions 
in  the  central  nervous  system,  by  impinging  on  important  centers, 
bring  about  disastrous  results,  optic  atrophy,  pontine,  medullary 
hemorrhages,  etc.  The  enormous  importance  of  correct  nervous  and 
mental  functioning  in  the  struggle  for  existence  is  self-evident.  The 
difficulties  in  the  way  of  repair  in  nervous  tissues  are  enormous — at 
times  insuperable.  Hence  one's  attack  upon  nervous  syphilis  should 
be  prompt  and  complete.  A  complete  sterilization  is  desirable — and 
this  is  often  extremely  difficult  to  bring  about  in  nervous  tissues. 

It  must  always  be  borne  in  mind  that  cerebral  sj'philis  is  often 
present  with  a  negative  Wassermann  reaction — this  finding  then 
should  not  deter  one  if  there  are  clinical  signs  of  diagnostic  import. 
Not  infrequently  active  antisyphilitic  treatment  (arsenical  compounds) 
causes  the  appearance  of  a  positive  blood  reaction — how  often  this 
occurs  is  not  yet  knoAMi. 

Study  of  the  cerebrospinal  fluid  is  often  a  better  guide  to  the  correct 

^  White  and  Jelliffe :  For  more  complete  discussion  consult  Modern  Treatment  of 
Nervous  and  Mental  Diseases,  vol.  ii. 


764  NEUROSYPHILIS 

appreciation  of  the  situation  as  well  as  a  reflection  of  the  activity  of 
the  process.  Lymphocytosis  is  often  present  months  or  years  before 
any  definite  nervous  signs.  Lumbar  puncture  is  too  often  neglected 
in  cerebral  syphilis.  Many  authors  state  that  if  the  blood  is  negative 
to  the  Wassermann  test  there  is  little  occasion  for  studying  the  cerebro- 
spinal fluid.  This  leads  to  bad  results.  Vascular  and  meningovascular 
processes  may  progress  for  years  in  nervous  tissues  without  giving 
rise  to  a  positive  Wassermann  blood  reaction.  Here  is  an  occasion  in 
which  this  symptom  fails  to  be  present. 

An  energetic  treatment  should  therefore  be  carried  out  if  there  are 
suggestive  signs  of  nervous  syphilis,  even  should  there  be  a  negative 
Wassermann  test. 

In  certain  patients  rapid  action  seems  imperative;  in  others  the 
need  for  this  rapidity  is  not  so  much  in  evidence.  Granted  a  knowl- 
edge of  these  requirements,  the  choice  of  remedies  is  not  as  simple  as 
it  might  seem.  Iodides  given  by  the  mouth  in  doses  of  30  grains,  2 
grams  a  day,  show  evidences  of  activity  in  about  one  week.  Mercury 
by  inunction  shows  results  in  about  five  days,  while  the  newer  arsenical 
preparations  show  reactive  capacities  in  about  forty-eight  to  seventy- 
two  hours.  None  of  these  figures  should  be  accepted  as  final,  so  far 
as  curative  action  is  concerned,  nevertheless  they  are  worth  something. 
It  is  not  apparent  that  iodides  have  a  rapid  toxic  action;  hence  in 
lesions  which  are  characteristic  of  the  more  florid  aspects  of  spiro- 
chetal growth — basal  meningeal  types  of  acute  onset  particularly — 
they  should  not  be  chosen  in  the  initial  attack. 

Inunction  Method. — For  years  neurologists  have  taught — chiefly 
under  the  influence  of  Erb — that  nervous  syphilis  is  best  attacked  by 
the  inunction  method — combined  with  iodides.  In  those  situations 
in  which  the  time  element  is  of  less  moment  this  attitude  seems  justi- 
fiable, especially  for  gummatous  types  of  the  disease. 

Oleate  of  Mercury. — ^The  oleate  of  mercury  is  of  value  in  that  it  is 
comparatively  cleanly  and  produces  results  as  rapidly  as  other  mer- 
curial preparations  applied  to  the  skin.  A  dram  of  the  10  per  cent, 
oleate  is  to  be  used  night  and  morning  for  four  days.  The  patient 
then  takes  a  vapor  bath  and  the  same  dose  is  used  once  a  day  for  four 
days  more.  If  sponginess  and  soreness  of  the  gums  do  not  appear — 
with  cleaned  teeth  and  gums — the  double  dose  may  be  continued; 
otherwise  a  single  dose  should  be  utilized.  In  using  the  oleate  one 
usually  employs  a  small  piece  of  flannel  in  the  rubbing — the  first  dose 
should  be  larger,  as  the  flannel  absorbs  it,  and  the  same  piece  of 
flannel  should  be  used  continuously. 

The  oleate  may  irritate  the  skin,  but  as  it  is  absorbed  fairly  well 
from  all  parts  of  the  body  one  can  shift  around  more  readily  with  it 
than  with  other  mercurial  ointments.  Another  object  of  using  the 
oleate  is  on  the  ground  of  secrecy. 

This  line  of  treatment  should  continue  at  least  six  weeks;  after  the 
first  week  10  grains  (0.6  gram)  of  potassium  iodide  t.  i.  d.  should 
be  administered  during  the  course  of  treatment.  There  is  very  little 
advantage  in  raising  the  amount  of  iodide  above  60  grains  (2  grams) 


TREATMENT  765 

a  day.  After  six  or  eight  weeks  the  treatment  should  be  discontinued 
absolutely — to  be  renewed  not  later  than  three  months  after  the  ter- 
mination of  the  last  treatment.  A  third  and  fourth  course  is  advisable, 
even  imperative  if  a  positive  Wassermann  test  is  present  in  the  blood 
or  hTuphocytes  above  10  to  the  cubic  millimeter  are  obtained  from 
the  cerebrospinal  fluid. 

Ungiientum  Hydrargyri. — Unguentum  hydrargyri  is  much  used  and 
widely  recommended.  It  has  the  disadvantage  of  being  dirty  and  of 
attracting  attention.  The  latter  may  be  partly  obviated  by  adding 
some  non-stainable  coloring  matter  or  some  smelling  compound 
such  as  balsam  of  Peru.  Attention  can  thus  be  diverted  from  its 
characteristic  color.  The  ointment  is  used  in  daily  doses  of  from  5j 
(4  grams)  to  3ij  (^  grams)  best  rubbed  in  in  the  evening  in  a  fairly 
definite  manner  and  in  places  where  the  skin  is  more  permeable.  One 
uses  the  inner  surfaces  of  the  arm  and  forearm  for  the  first  rubbings, 
covering  them  with  bandages,  then  the  inside  of  the  groin,  then  the 
popliteal  space,  then  the  abdomen  and  back.  The  fifth  or  sixth  day 
the  patient  omits  his  rubbing — takes  a  Turkish  bath  and  then  starts 
over  the  same  course.     This  course  is  kept  up  for  thirty  doses. 

Other  Details. — ^The  care  of  the  skin  and  of  the  mouth  is  naturally 
to  be  kept  in  mind.  The  blood  Wassermann  reaction  should  be  tested 
at  the  end  of  the  period,  and  if  strongly  positive,  or  if  spinal  puncture 
shows  active  lymphocytosis,  or  if  clinical  signs  seem  slow  in  respond- 
ing, the  inunctions  should  be  continued  at  least  two  or  three  weeks 
longer. 

Checking  up  by  the  Wassermann  test  and  lumbar  puncture  three  or 
four  months  later,  or  any  increase  in  clinical  signs  should  determine  a 
repetition  of  the  treatment  along  identical  or  more  strenuous  lines. 

Iodides,  30  grains  daily,  are  to  be  given  throughout  the  course  of 
the  inunctions.  All  medication  should  cease  at  the  end  of  the  cure, 
unless  there  are  definite  indications  for  its  continuance. 

Other  mercurial  inunction  masses  may  be  used.  Those  of  value 
are  the  hydrargyri  vasenol,  vasogen,  mitin,  resorbin,  which  have 
special  indications  which  may  render  them  particularly  valuable. 

Mercury  Injection  Treatvient. — Injection  treatment  attempts  an 
even  more  rapid  and  energetic  attack  upon  the  spirochete.  ]\Iany 
battles  have  been  fought  among  s^-philographers  as  to  the  compara- 
tive merits  of  the  insoluble  or  soluble  salts  of  mercury.  When  so 
much  diversity  of  opinion  can  be  found,  it  usually  indicates  that  the 
real  difl"erences  are  usually  minimal.  Hence  ease  of  administration, 
safety,  painlessness,  etc.,  determine  the  choiceof  the  remedy  in  each  case. 

Calomel,  mercury  salicylate  and  acetate  of  mercury  are  among  the 
more  favored  insoluble  salts.  Calomel  has  occupied  a  high  rank 
and  can  be  utilized  in  the  following  forms: 

I^ — Hydrargjri  chloridi  mitis 5.0  gm. 

Sodii  chloridi         5.0" 

Aq.  dest 50.0     " 

Mucilagio  arabici 2.5" 

It — Hydrargjii  chloridi  mitis. 
01.  sesami,  10  per  cent. 


766  NEUROSYPHILIS 

Pravaz  s^Tingefiil  every  second  or  third  day,  preferably  into  the 
muscles  of  the  thigh  or  back,  for  12  to  15  doses. 

The  hypodermic  use  of  calomel  is  often  accompanied  by  much  pain. 
Abscess  and  necrosis  is  not  uncommon,  and  lung  emboli  may  occur — 
with  care,  however,  calomel  given  by  hypodermic  is  free  from  danger. 

Creams  of  calomel,  devised  by  Lambkin,  have  been  extensively 
used,  as  they  cause  less  pain  and  give  rise  to  no  complications  if 
bloodvessels  are  avoided.  These  creams  should  be  sterile.  The 
formulae  of  some  in  use  are  as  follows: 

H — Calomel 5  gm. 

Creosote 20     " 

Camphoric  acid 20     " 

Palmitin 100     " 

Inject  lOlU  of  this  cream  once  a  week  for  one  month,  to  be  replaced  by  the  foUowjng: 

I^ — Hydrargyri  (metallic) • 10  gm. 

Creosote 20     " 

Camphoric  acid 20     " 

Palmitin 100     " 

This  is  injected  in  doses  of  lOTfl  twice  a  week  for  three  weeks. 

After  six  doses  have  been  given,  stop  for  two  months.  Four  injec- 
tions of  the  metallic  cream  are  then  given  at  fortnightly  intervals. 
Then  a  rest  for  four  months.  Then  four  injections  as  before  and  a 
rest  for  six  months.  Then  a  repetition  of  four  fortnightly  doses — 
an  interval  of  one  month  and  a  final  series  of  four  metallic  cream  doses. 

English  syphilographers  have  found  these  creams  admirable  in 
army  and  navy  work.  They  are  adapted  for  early  stages  better  than 
for  nervous  syphilis,  but  are  worthy  of  more  extended  trial  in  nerve 
syphilis. 

The  use  of  the  insoluble  salts  has  the  advantage  of  a  much  more 
prolonged  action  of  the  mercury.  They  also  have  the  disadvantage — ■ 
all  mercurial  salts  share  in  this,  however — of  irritation  of  the  kidneys. 
If  albumin  is  found  before  the  use  of  mercury  one  should  look  for 
a  syphilitic  nephritis.  Tuberculosis,  diabetes,  alcoholism,  marked 
cachexia  are  additional  factors  to  be  carefully  dealt  with.  Gastro- 
intestinal disturbances  are  frequent,  but  it  is  extremely  rare  that 
mercury  causes  a  neuritis. 

Thorough  cleansing  of  the  mouth  and  the  use  of  a  chlorate  of  potash 
mouth  wash  is  imperative. 

The  soluble  salts  in  use  are  very  numerous.  They  include  the 
sozoiodolate,  bichloride,  lactate,  succinamide,  biniodide,  benzonate  and 
cyanide.  Fournier  lists  about  30.  They  may  be  injected  within 
the  muscles  or  into  the  skin,  superficially.  All  are  somewhat  painful 
and  accidents  are  possible.  In  general  the  dosage  is  from  j  to  |  of  a 
grain.     The  injections  are  given  twice  or  three  times  a  week. 

General  Scheme  of  Injection. — ^The  following  general  scheme  is 
suggested : 

1.  The  site  usually  chosen  is  the  posterior  third  of  the  buttock,  to 
avoid  the  sciatic  nerve  and  vessels. 

2.  The  skin  should  be  scrubbed  with  alcohol  or  ether. 


TREATMENT 


767 


3.  The  injection  should  be  made  deeply  into  the  muscle,  using  each 
buttock  alternately. 

4.  The  syringe  and  piston  should  preferably  be  of  glass,  easily 
sterilized,  and  the  needle  of  platinum  iridium,  about  1^  inches  in 
length  and  sterilized. 

5.  After  insertion  of  the  needle,  the  piston  should  be  slightly  with- 
drawn, and  if  any  blood  appears  the  needle  should  be  reinserted  in 
order  to  avoid  injection  into  a  bloodvessel.  The  injection  of  the  solu- 
tion free  from  air  bubbles  can  then  take  place. 

Solutions. — Various  solutions  are  in  use.  Only  a  few  can  be  men- 
tioned here. 


I^ — Hj'drargyri  sozoiodolate gm.  0.2 

Sodii  iodidi gm.  0.3 

Aq.  dest ; gm.  10. C 

Dose — 10  to  20  minims  constitute  the  dosage. 

IJ — HydrargjTi  lactatis gm.  0 . 2 

Aq.  dest c.c.  18.0 

Dose — 10  to  25  minims. 

I^ — Hydrargyri  succinamidi gm.  0.2 

Aq.  dest c.c.  10.0 

Dose — 10  to  25  minims. 

^ — Hydrargyri  chloridi  corrosi\Tim gm.  0.5 

Sodii  cbloridi gm.  3 . 0 

Aq.  dest gm.  100.0 

Dose — 1  to  2  c.c.  daily  or  alternate  days. 


gr.  uj 
gr.  V 
3iiss 

gr.  iij 
3iv 


gr.  nj 
3iiss 


gr.  \nij 
gr.  xlv 
Siij 


The  use  of  corrosive  sublimate — following  Lewin — is  usually  very 
painful. 

I^ — Hydrargyri  cyanidi gm.       1.0  gr.  xv 

Cocain  hydrochloridi gm.      0.3  gr.  v 

Aq.  dest .         ad  gm.  100.0  Siij 

Dose — 1  to  2  c.c. 

A  useful  variant  of  this  combines  the  cyanide  with  arsenic  and 
strychnine,  as  follows: 

I^ — Hydrargyri  cyanidi, 

Strych.  arsenatis aa     gm.      0.6     aa     gr.  ix 

Cocain  muriat gm.      0.3  gr.  v 

Aq.  dest gm.    60.0  5ij 

Dose — 5  to  10  minims  every  other  day  for  20  to  25  doses. 


Cocain  may  be  added  to  any  of  the  soluble  salts.  Its  addition 
lessens  the  pain. 

Fournier  has  always  advocated  the  use  of  the  biniodide  dissolved 
either  in  sterilized  oil  or  in  water.  It  is,  he  claims,  painless,  sure  and 
free  from  dangers.  In  2457  injections  only  9  produced  pain.  Such 
results,  however,  obtain  only  when  the  physician  is  very  careful. 
Careless  use  with  the  biniodides  will  produce  all  of  the  accidents,  pain, 
abscess,  etc. 


768  NEUROSYPHILIS 

^ — Hydrargyri  biniodide gm.       0.4  gr.  vj 

Olive  oil  (sterilized) gm.     10.0  Siiss 

Dose — One  Pravaz  syringeful  every  other  day. 

I^ — Hydrargyri  biniodide gm.       0.2  gr.  iii 

Sodii  iodide gm.       0.2  gr.  iij 

Aq.  dest gm.     10.0  3iiss 

Dose — 1  to  2  c.c.  daily  or  on  alternate  days,  with  gradual  elevation  of  the  dose  if 
stomatitis  or  gastro-intestinal  signs  are  not  in  evidence.  Twenty  to  twenty-five  injec- 
tions constitute  a  course  of  treatment. 

IJ — Hydrargyri  benzoatis gm.       1.0  gr.  xv 

Sodii  chloridi gm.      2.5  gr.  xlv 

Aq.  dest gm.  120.0  5iv 

Dose — 1  to  2  c.c.  daily  or  on  alternate  days. 

Combined  arsenical  and  mercurial  injections  were  very  much  in 
vogue  before  the  introduction  of  the  salvarsan  preparations.  One 
of  the  most  popular  of  these  has  been  the  arsenical  salicylate  or  enesol. 
This  remedy  has  been  used  widely  in  nervous  syphilis  and  often  with 
surprisingly  good  results.     Schaffer  speaks  very  highly  of  it. 

The  combined  use  of  the  cacodylates  and  of  mercury  has  been 
observed  to  give  good  results.  The  early  reports  of  optic  nerve  disease 
apparently  following  the  use  of  the  cacodylates  served  to  force  these 
salts  into  the  background.  Inasmuch  as  such  optic  nerve  changes 
apparently  occurred  in  other  than  syphilitic  patients,  it  would  not 
appear  that  they  are  to  be  interpreted  as  instances  of  those  neuro- 
recidives  which  have  been  so  actively  discussed  since  salvarsan  has 
been  introduced. 

Newer  Arsenic  Products. — ^Any  attempt  at  an  exhaustive  sum- 
mary of  the  various  reports  upon  these  remedies  in  the  treatment  of 
nervous  syphilis  would  require  a  special  volume.  A  simple  enumera- 
tion of  the  bibliography  alone — best  obtained  in  brief  in  Lewandowsky's 
Handbuch  der  Neurologic,  articles  by  Forster  and  Schaffer  and  others 
— in  Nonne's  discussion,  already  referred  to  would  require  dozeas  of 
pages.  Only  the  present  (1919)  drift  of  opinion  will  here  be  expressed. 
Since  the  great  disturbance  in  the  chemical  industries  due  to  the  Great 
War  there  have  been  many  new  arsenical  compounds  made,  either  as 
replicas  or  imitations  or  as  substitutes  for  those  originally  introduced 
by  Ehrlich  and  the  German  chemists.  Not  enough  time  has  elapsed 
to  permit  an  adequate  appraisal  of  these  remedies.  The  reader  is 
warned  that  many  pirate  combinations  are  on  the  market  and  that 
fatal  results  have  been  more  frequent  in  recent  war  times  from  the 
use  of  arsphenamine  products.  Great  care  must  be  taken  in  the 
selection  of  the  product  used. 

In  the  exudative,  hyperplastic,  gummatous  and  arterial  forms 
arsenical  compounds,  arsphenamine,  related  to  salvarsan  and  neo- 
salvarsan,  are  by  far  the  most  efficient  remedies  possessed  at  the 
present  time.  One  form  needs  to  be  excepted,  that  of  the  large 
gummata,  for  which  surgery  alone  is  adequate.  It  would  also  appear 
that  much  larger  doses  of  salvarsan  are  required  for  nervous  syphilis 
than  were  used  in  the  earlier  stages  of  its  administration.     Since  the 


TREATMENT  769 

use  of  adequate  dosage  the  so-called  neurorecidives  have  almost  entirely 
disappeared.  It  is  the  present  trend  of  opinion  that  mercury  and 
arsphenamine  combined  gives  the  best  results.  Whether  or  not 
arsenic  and  mercury,  both  active  spirochetal  drugs,  supplement  each 
other  in  this  combined  use  is  not  certain,  but  the  results  obtained  have 
in  many  instances  been  very  satisfactory. 

At  the  same  time  it  needs  to  be  observed  that  a  number  of  patients 
have  relapsed  and  the  final  results  of  therapy  in  nervous  sj^philis 
have  been  far  from  being  as  hopeful  as  had  first  appeared.  Because 
this  has  been  so  is  no  reason  why  it  should  remain  so.  The  most 
obvious  reason  that  stands  out  in  many  of  the  recent  discussions 
that  have  taken  place  relative  to  this  point  is  that  the  patients  have 
been  insufficiently  treated.  Finally,  arsphenamine  has  not  been  long 
enough,  as  compared  with  mercury,  in  use  to  warrant  anything  but 
as  yet  tentative  conclusions. 

With  this  short  summary  of  conclusions,  a  few  words  may  be  said 
as  to  its  application  and  dosage.  Whether  neo-arsphenamine  is  to 
replace  arsphenamine  or  not  cannot  yet  be  determined.  The  intra- 
venous administration  of  arsphenamine  is  the  best  method  of  giving 
it.  It  should  not  be  given  unless  the  patient  is  under  some  sort  of 
supervision — in  a  hospital  or  remaining  in  bed — and  minute  attention 
to  the  technic  is  absolutely  necessary  to  avoid  certain  dangers.  It 
is  highly  important  that  fresh,  distilled  water  be  employed  if  arsphena- 
mine is  to  be  used  intravenously. 

To  kill  spirochetes  in  the  nervous  system,  however,  is  one  thing, 
and  to  overcome  the  results  of  tissue  changes  is  quite  a  different  one, 
and  this  above  all  is  the  stumbling-block  in  the  treatment  of  nervous 
tissue  s^'philis.  Nevertheless,  if  nerve  tissues  have  not  been  extensively 
destroyed,  one  can  hope  for  excellent  results  by  a  proper  combination 
of  arsphenamine  therapy  with  mercury. 

Arsphenamine  must  be  used  in  much  larger  quantities,  however, 
than  was  at  first  practised.  At  the  end  of  this  section  the  outline  of 
an  energetic  combined  therapy  is  given  and  reference  may  be  made 
to  those  pages  for  the  general  indications  of  such  a  course  of  treatment. 
Modifications  to  a  less  active  mercurial  salt  than  calomel  may  have  to 
be  made.     Every  patient  needs  individual  treatment. 

Notwithstanding  the  very  evident  fact  that  saivarsan  and  neosal- 
varsan  are  active  spirocheticidal  drugs,  it  is  still  an  important  problem 
how  to  reach  them  in  the  nervous  system.  Careful  chemical  inves- 
tigation of  the  cerebrospinal  fluid  has  heretofore  failed  to  obtain  any 
trace  of  arsenic  when  saivarsan  has  been  given  in  the  usual  manner. 
One  may  infer  that  the  arsenic  has  become  fixed  in  some  chemical 
combination  which  fails  to  react  to  the  usual  chemical  tests.  It  is  not 
yet  fully  comprehensible  why  nervous  syphilis  is  so  resistant  to  treat- 
ment and  why  the  hopes  aroused  by  the  striking  results  of  saivarsan 
therapy  in  general  syphilis  seem  not  to  have  been  borne  out  in  nervous 
syphilis. 

It  has  already  been  pointed  out  that  the  lymph  spaces  being  blocked 
49 


770  '  NEUROSYPHILIS 

by  the  sj^hilitic  process  constitutes  a  great  barrier  to  the  introduction 
of  drugs.  \Mien  this  difficulty  is  overcome  and  better  penetration 
obtained  then  the  treatment  of  the  parenchymatous  or  central  types 
maj'  give  better  results. 

Swift  and  Ellis  have  attempted  to  place  a  spirocheticidal  solution 
directly  into  the  cerebrospinal  fluid.  Salvarsan  and  neosalvarsan 
were  employed  by  direct  injection  into  the  spinal  canal  through  the 
Quincke  lumbar  puncture.  This  method  they  found  was  to  be  con- 
demned. It  failed  to  give  any  beneficial  results,  and,  moreover, 
caused  marked  pains.  It  is  probably  a  dangerous  procedure,  as 
animal  experimentation  has  sho^vn. 

An  attempt  was  then  made  to  introduce  into  the  patient's  cerebro- 
spinal fluid  some  of  his  o"^ti  blood  serum  which  had  previously  been 
mixed  with  the  arsphenamine  by  intravenous  infusion.  This  pro- 
cedure is  carried  out  in  the  usual  manner.  After  a  certain  length  of 
time — one  hour  was  found  to  give  the  most  active  serum— blood  was 
withdrawn,  separated  from  its  corpuscles,  after  twenty-four  bom's, 
diluted  with  40  per  cent,  of  normal  saline  and  then  heated  to  56°  C. 
for  thirty  minutes.  A  lumbar  puncture  is  then  made  and  from  5  to 
15  c.c.  of  cerebrospinal  fluid  is  withdrawn,  i.  e.,  until  the  pressure  falls 
to  30  mm.  of  mercury.  Thirty  cubic  millimeters  of  the  warmed  serum 
is  then  injected  into  the  subarachnoid  spaces.  The  patient  must  lie 
quiet,  the  foot  of  the  bed  usually  being  raised.  After  ten  days  to  two 
weeks  the  injections,  which  are  usually  well  borne  are  to  be  repeated. 

They  thus  obtained  very  striking  results  in  the  action  upon  the  sero- 
biological  factors  known  to  accompany  cerebral  sj'philis.  Then  a  more 
crucial  experiment  was  planned.  This  consists  in  the  introduction 
into  the  subarachnoid  spaces  of  serum  taken  from  another  individual, 
usually  a  secondary  sjqjhilitic  under  treatment,  the  technic  being 
that  just  outlined.  In  the  treatment  of  tabes  by  a  heterologous  serum 
most  excellent  results  have  been  obtained  in  some  cases.  In  others 
they  have  been  nil  or  positively  dangerous.  Why  the  striking  char- 
acter of  the  results?  for  it  is  at  once  evident  that  the  amount  of 
spirocheticidal  substance  in  a  few  cubic  centimeters  of  serum  taken 
from  the  body  of  another  patient  who  had  received  the  usual  intra- 
venous salvarsan  therapy  must  be  very  small  indeed,  i.  e.,  reckoned 
as  arsenic.  If  other  factors  than  the  salvarsan  itself  enter  into  the 
situation  these  are  as  yet  unknown.  One  significant  fact,  however, 
would  tend  to  indicate  that  other  forces  are  operative.  Extensive 
experiments  carried  on  by  Swift  and  Ellis  with  the  heated  and  unheated 
serums  show  that  the  heated  sera  are  three  times  as  spirocheticidal 
to  Spirocheta  duttonii  in  mice. 

In  the  treatment  of  tabes  the  intraspinal  method  is  often  very 
efficacious.  Again  it  is  disadvantageous.  The  advantages  outweigh 
the  disadvantages.     Excessive  pain  is  sometimes  the  first  reaction. 

In  the  treatment  of  paresis  intracranial  injections  of  salvarsanized 
serum  ofi'er  increasing  evidences  of  amelioration.  These  injections 
may  be  intraventricular  or  intradural.     The  indications  are  crystalliz- 


TREATMENT  771 

ing.  The  general  trend  is  to  make  a  rapid  attack  by  intravenous 
methods,  which  if  unavaihng  can  be  followed  up  by  the  intracranial 
route. 

The  most  striking,  suggestive  result  is  an  almost  immediate  diminu- 
tion in  the  number  of  pathological  cells  in  the  cerebrospinal  fluid.  The 
globulin  reaction  diminishes,  positive  Wassermanns  of  the  cerebro- 
spinal fluid  with  small  quantities  of  fluid  require  larger  quantities  to 
show  positive  or  become  negative  and  the  amelioration  of  the  symp- 
toms has  in  a  few  cases  been  rapid. 

Mercury  by  the  Mouth. — IMercury  by  the  mouth  will  always  remain 
one  of  the  simplest,  and  yet  at  the  same  time,  least  efl^cient  methods 
of  treating  syphilis  of  the  nervous  system.  Here  again  one  has  a  rich 
choice  of  remedies.  Those  most  in  use  are:  protoiodide  grain  |  to  f, 
sublimate  grain  -^q,  calomel  2  to  5  grains.     Various  vehicles  are  used. 

The  disadvantages  of  treatment  by  means  of  the  intestinal  canal 
are  many — chief  of  which  is  the  slow  and  weak  action  of  the  remedies 
employed.     IMoreover,  the  gastro-intestinal  tract  sufi"ers. 

The  chief  advantage  is  that  mercury  may  be  combined  with  the 
iodides.  Furthermore,  conveniences  of  medication  must  often  con- 
strain one  to  use  this  mode  of  giving  antisyphilitic  remedies,  but  only 
as  a  necessary  choice. 

In  nervous  syphilis  it  would  appear  that  mercury  medication  by 
mouth  is  not  radical  enough.  One  may  use  it  after  an  energetic 
treatment,  by  the  methods  outlined,  has  been  employed,  but  oral 
administration  is  rarely  a  method  of  certain  value  and  hence  is  not 
advisable,  save  under  particular  circumstances. 

Among  the  newer  mercury  preparations  which  future  experience 
may  prove  to  be  of  value  are:  Mercury  dicarboxylate,  two  forms  of 
which  are  on  the  market,  with  pronounced  toxic  action  on  spirochetes 
in  rabbits.  It  is  claimed  to  be  twenty  times  as  toxic  to  spirochetes  as 
corrosive  sublimate  and  yet  shows  no  action  on  the  body.  Its  dosage 
has  not  yet  been  worked  out. 

Iodides. — Sodium  and  potassium  iodide  have  been  used  in  the  treat- 
ment of  syphilis  of  the  nervous  system  for  years  and  often  with  good 
results.  According  to  Xeisser  the  iodides  are  weak  spirochetal  poisons. 
Our  belief  in  its  resorptive  powers  is  justified  on  empirical,  if  not  on 
pharmacological  grounds.  Personal  experience  does  not  confirm  the 
belief  in  the  efficiency  of  specially  large  doses,  although  that  is  the 
American  preference. 

The  use  of  the  iodides  in  doses  of  from  10  to  30  grains  t.  i.  d.  com- 
bined with  mercury  is  particularly  ^•aluable  in  the  gummatous  type 
of  cerebral  syphilis.  It  is  folly,  however,  to  try  to  do  away  with  large 
gummata  by  means  of  massive  doses  of  iodides. 

The  dosage  of  the  iodides  will  depend  upon  the  individual.  There 
are  many  idiosyncrasies  to  be  borne  in  mind.  At  times  small  doses 
cause  marked  disturbances  and  cannot  be  borne.  Here  one  may 
employ  other  combinations  than  those  of  sodium  or  i)otassium.  Hence 
strontium,  rubidium  and  organic  iodine  preparations  have  come  into 


772  NEUROSYPHILIS 

use.  lodopin,  sajodin,  iodoglidin,  iodoval,  iodocitin,  iodostarin  are 
among  the  newer  of  these  combinations. 

lodopin  may  be  injected  as  well  as  administered  by  mouth.  In 
the  former  case  it  is  used  in  quantities  of  10  c.c.  on  alternate  days, 
or  smaller  doses  1  to  3  c.c.  at  more  frequent  intervals.  In  giving  it 
by  hypodermic  both  the  syringe  and  the  remedy  should  be  slightly 
warmed,  the  needle  should  have  an  ample  bore  and  the  drug  be  intro- 
duced slowly.  It  is  also  given  by  the  mouth  in  5  j  doses.  In  the  form 
of  lodopin,  large  quantities  of  iodine  may  be  introduced  without  toxic 
effect.     Its  action  on  nervous  syphilis  has  not  been  extensively  studied. 

Iodoval  and  iodocitin,  the  latter  a  lecithin-albumin  compound,  have 
been  found  to  be  borne  well  in  the  course  of  salvarsan-mercurial  treat- 
ment. The  former  is  given  in  doses  of  about  5  grains  t.  i.  d.  through- 
out an  energetic  salvarsan-mercury  treatment — the  latter  in  about 
the  same  doses. 

Plan  of  Intensive  Treatment. — Nervous  syphilis  is  treated  too  gin- 
gerly by  most  practitioners.  It  is  difficult  to  kill  the  syphilis  organism, 
hence  an  energetic  course  of  treatment  is  here  outlined: 

First  day 0.03  calomel  (or  other  mercurial)  hypodermically. 

Third  day   .  .      .  0.05  calomel  (or  other  mercurial)  hypodermically. 

Fifth  day 0.4    salvarsan  intravenously. 

Seventh  day      .      .      .      .  0.5    salvarsan  intravenously. 

Ninth  day 0.05  calomel  hypodermically. 

Eleventh  day    ....  0.05  calomel  hypodermically. 

Thirteenth  day       .      .      .  0.4    salvarsan  intravenously. 

Fifteenth  day   .      .      .      .  0.5    salvarsan  intravenously. 

Seventeenth  day    .      .      .  0 .  05  calomel  hypodermically. 

This  should  be  continued  for  six  weeks,  or  until  at  least  5  grams  of 
salvarsan  are  administered.  The  whole  course  can  be  compressed 
into  three  weeks  if  a  soluble  mercury  salt  is  given  and  at  least  5  grams 
of  salvarsan  can  be  administered  in  that  time.  The  patient  should 
be  w^atched  very  carefully,  especially  with  reference  to  the  kidneys. 
Furthermore,  there  are  patients  who  do  not  bear  calomel  well.  Vago- 
tonic individuals  react  excessively  to  mercury,  especially  to  minute 
doses.  (Compare  article  on  Syphilis  of  the  Nervous  System  in  Modern 
Treatment  of  Nervous  and  Mental  Diseases,  White  and  Jelliffe,  Vol.  II.) 


PART  III. 

PSYCHICAL  OR    SYMBOLIC   SYSTEMS. 

NEUKOSES,  PSYCHONEUROSES,  PSYCHOSES. 


Introduction. — Present-day  psychiatry  is  almost  wholly  a  product 
of  this  century.  Nineteenth  century  psychiatry,  even  well  along  in  its 
latter  half,  had  only  a  few  relatively  simple  concepts  with  which  to 
approach  the  problems  of  mental  disease.  It  was  altogether  in  the 
descriptive  stage  endeavoring  to  define  the  material  with  which  it  had 
to  deal.  The  whole  group  of  the  psychoses  was  classified,  for  the  most 
part,  as  states  of  mania,  melancholia,  and  dementia,  plus  the  congenital 
states  of  the  various  degrees  of  idiocy  and  imbecility.  Paresis  was  the 
only  disease  that  had  a  well  recognized  course  and  outcome  and  even 
its  specific  etiology  was  only  suspected.  Paranoia  was  a  later  concept 
and  was  largely  conceived  to  be  secondary  to  chronic  states  of  mania 
and  melancholia.  The  adolescent  psychoses  were  only  being  slowly 
dismembered  by  the  description  of  catatonia  and  hebephrenia  which 
Kraepelin  later  used  in  formulating  his  dementia  precox  concept. 
This  together  with  the  manic-depressive  concept,  also  formulated  by 
Kraepelin,  were  the  notable  contributions  to  the  psychiatry  of  the  latter 
half  of  the  century. 

Kraepelin's  method,  however,  was  a  distinct  advance  upon  what 
had  gone  before.  It  approached  the  problem  of  mental  disease  by  the 
natural  history  method  of  studying  its  life  history,  but  in  so  doing  still 
remained  at  the  descriptive  level.  Psychiatry  in  this  period  of  its 
development  was  occupied  largely  with  questions  of  classification  and 
dominated  by  a  psychology  which  was  also  in  its  descriptive  stage  of 
development  and  handicapped  by  metaphysical  speculations  on  the 
nature  of  the  psyche  and  its  relations  to  the  body. 

The  present-day  trends  and  concepts  of  psychiatry  had  their  begin- 
nings in  a  larger  knowledge  of  the  nervous  system,  particularly  of  its 
development  and  of  the  functions  of  the  vegetative  neuromusculo- 
glandular  apparatus ;  in  the  growth  of  a  genetic  psychology  (psychology 
of  primitive  man  and  child  psychology);  in  the  results  of  the  studies 
of  borderland  states  (particularly  the  neuroses  and  psychoneuroses) ; 
in  the  growth  of  a  behavioristic  psychology  largely  founded  upon 
animal  psychology;  and  finally  in  the  rise  of  the  psychoanalytic  school. 

The  older  psychology,  both  normal  and  morbid,  was  not  only  descrip- 

(773) 


774  NEUROSES,  PSYCHONEUROSES,  PSYCHOSES 

tive  but  largely  a  refined  physiology  of  the  sense  organs.  In  this  psychol- 
ogy the  sensation  was  considered  the  unit  of  psychological  experience 
while  in  the  new  approach  to  psychological  problems  the  icish  is  coming 
to  replace  the  sensation  as  the  unit.  The  wish  is  the  form  in  which  the 
psyche  registers  the  result  of  the  somatic  trends  as  finally  integrated 
and  expressed  in  the  psyche.  Psychology  thus  becomes  the  study  of 
the  tendencies  of  the  whole  individual  as  expressed  in  the  psyche, 
the  functions  of  the  individual,  in  distinction  from  physiology  which 
deals  only  with  the  functions  of  the  several  parts,  the  organs. 

Inasmuch  as  each  organism  is  so  integrated  as  to  have  tendencies 
which  express  it  as  a  whole  to  which  its  several  parts  minister  as  subor- 
dinate to  the  larger  ends  the  psyche  has  a  history  which  is  equal  in 
length,  contemporaneous,  to  the  history  of  the  soma.  To  speak  only 
of  man,  this  history  is  the  history  of  his  cultural  development  and 
is  comprised  of  a  past  of  five  hundred  thousand  (according  to  the  best 
recent  calculations)  years.  Just  as  the  history  of  the  body  has  been  laid 
down  in  structures,  the  organs,  which  represent  its  final  answer  to  the 
several  problems  of  adjustment  in  terms  of  physiological  function,  the 
history  of  nutrition,  for  example,  is  laid  down  in  the  structure  and 
functions  of  the  gastro- intestinal  tract,  etc.,  so  the  history  of  the  psyche 
is  laid  down  in  ways  of  thinking  of  which  ideas  and  feelings  are  the  mani- 
fest evidence,  and  as  the  meanings  of  the  various  organs  can  only  be 
understood  by  an  analysis  of  their  past  (embryology  and  compara- 
tive anatomy),  so  the  meanings  of  given  ideas  and  feelings  can  only 
be  understood  by  tracing  their  growth  and  development.  The  his- 
torical past  of  the  psyche  is  the  region  of  the  unconscious,  and  this 
region  can  be  reconstructed  only  by  an  analysis,  an  uncovering  of  its 
contents  much  as  the  history  of  the  earth's  crust  can  only  be  known 
through  excavation  and  the  discovery  of  the  fossil  remains  in  the 
several  strata.  The  uncovering  of  the  contents  of  the  past  in  the 
unconscious  may,  by  analogy,  be  termed  paleopsychology.  This 
concept  is  important  because  m  mental  disease  we  constantly  see  ideas, 
feelings,  w^ays  of  thinking  outcropping  which  can  only  be  understood 
if  we  relate  them  to  like  ideas,  feelings,  and  ways  of  thinking  in  the 
developmental  history  of  the  psyche.  Patients  constantly  display 
psychological  symptoms  which  can  thus  be  at  once  related  to  the 
psychological  characteristics  of  primitive  man  and  of  the  child. 

In  dealing  with  the  individual  then  at  the  psychological  level  it 
becomes  necessars^,  for  the  first  time,  to  use  terms  which  refer  to  the 
individual  as  a  whole.  In  fact  that  is  only  another  way  of  designating 
what  psychology  is.  At  the  physicochemical  and  the  sensorimotor 
levels  it  was  possible  to  deal  with  isolated  phenomena  so  far  only  as 
related  to  the  particular  and  immediate  end  of  the  function  under 
consideration.  For  example:  tapping  the  patella  tendon  produces  an 
extension  of  the  leg  on  the  thigh,  which,  if  average  in  extent,  indicates 
a  normal  motor  pathway  to  the  quadriceps.  At  the  psychological 
level,  however,  it  is  no  longer  possible  to  deal  with  parts  of  the  indi- 
vidual in  this  way,  but  the  whole  individual  comes  at  once  under 


tNfRODUCTlON  775 

consideration.  Thus  it  may  be  a  question  of  desire,  of  failure,  of  regret, 
of  inefficiencies  of  all  sorts,  but  it  is  always  a  desire,  failure,  regret, 
or  what  no.t,  of  the  individual.  The  individual  as  such  has  failed  in 
effectmg  an  adequate  adjustment.  The  failure,  it  is  true,  may  have 
been  conditioned  by  bad  vessels,  by  visceral  disease  of  all  sorts,  but 
the  consideration  of  these  factors  is  a  consideration  at  the  physiological 
level.  As  soon,  however,  as  the  problem  is  expressed  at  the  psycho- 
logical level  it  is  of  necessity  expressed  in  terms  that  refer  to  the  indi- 
vidual as  a  whole,  as  a  social  unit. 

A  few  paragraphs  devoted  to  an  account  of  the  development  of  these 
tendencies  of  the  whole  individual  before  taking  up  a  consideration 
of  the  disorders  at  this  level  will  be  useful. 

The  baby  in  its  mother's  uterus  has  no  desires;  it  has  to  do  nothing 
for  itself,  not  even  to  breathe;  it  rests  quietly,  far  removed  from  sources 
of  outside  stimulation  and  irritation,  every  function  being  performed 
for  it  by  the  mother.  After  the  baby  is  bom  this  condition  of  affairs 
still  continues,  or  at  least  an  effort  is  made  for  it  still  to  continue.  The 
baby,  to  be  sure,  has  to  begin  to  breathe  for  itself,  to  eat  for  itself, 
to  perform  the  functions  of  digestion  and  elimination  for  itself,  but 
on  the  other  hand,  there  stand  about  the  army  of  the  household,  not 
satisfied  to  wait  upon  desire,  but  with  every  heartstring  of  emotion 
tense  to  forestall  it.  He  is  waited  upon  hand  and  foot  by  all;  he  is, 
in  the  sense  that  every  desire  is  satisfied,  truly  omnipotent. 

As  the  days  go  by  and  development  proceeds  apace,  as  the  sense 
organs  become  more  acute,  the  muscular  adjustments  more  refined, 
the  baby's  contact  with  the  world  becomes  progressively  and  increas- 
ingly complex,  and  try  as  they  will  the  loving  attendants  cannot  fore- 
stall all  of  his  desires,  and  so  there  come  times  when  food  is  not  offered 
at  the  instant  it  is  needed,  when  sleepiness  overtakes  the  baby  but  he 
cannot  woo  it  if  he  is  in  a  bright  and  noisy  street  or  on  a  clattering 
car  far  from  his  soft  bed.  And  so  there  arises  insidiously  but  neces- 
sarily the  mental  state  of  desire,  things  wished  for  because  they  are 
not  had. 

Still  even  in  this  stage  of  development  the  discrepancy  between 
desire  and  attainment  is  not  great.  Attainment,  in  fact,  is  usually 
very  near  at  hand,  the  hunger  is  not  permitted  to  last  long,  the  baby 
does  not  have  to  be  kept  awake  by  noises,  except  for  a  brief  period, 
while  in  the  matter  of  other  desires,  such,  for  instance,  as  the  desire 
to  empty  the  bladder,  that  is  indulged  in  forthwith  without  any 
further  consideration  of  the  matter.  As  development  progresses,  how- 
ever, desires  become  more  and  more  numerous,  because  the  baby 
touches  reality  at  more  numerous  points,  and  each  one  of  these  points 
offers  a  new  possibility  for  a  frustrated  or  delayed  desire,  while  with 
such  matters  as  emptying  the  bladder  there  soon  steps  into  the  situa- 
tion the  social  repressions  represented  by  the  prohibitions  of  the 
mother. 

Thus  growing  up  in  the  life  of  the  baby,  beginning  even  in  the  earliest 
days,  an  ever-increasing  discrepancy  between  desire  and  attainment 


776  NEUROSES,  PSYCHONEUROSES,  PSYCHOSES 

takes  place,  and  as  the  years  go  on  it  will  be  seen,  without  the  necessity 
for  further  illustrations,  that  the  amoral,  egocentric  baby  must  grad- 
ually take  into  consideration  the  world  about  him.  He  is  forced  to 
lay  his  conduct  along  certain  lines  which  imply  a  putting  off  of  the 
satisfaction  of  desire  into  an  ever-receding  future.  Later  in  life,  when 
he  is  hungry  and  wishes  to  eat,  he  can  only  satisfy  this  desire  provided 
he  has  worked  and  earned  the  wherewithal  to  buy  food,  and  if  he 
endeavors  to  satisfy  it  othei'wise  by  taking  any  food  that  may  be  at 
hand  he  offends  the  social  usages  and  becomes  a  thief.  If  he  wishes  to 
empty  his  bladder  he  has  to  wait  until  he  gets  to  an  appropriate  place; 
it  cannot  be  done  anj^where  and  at  any  time.  He  has  to  adjust  himself 
to  the  requirements  of  society  or  run  serious  risks  if  he  fails.  As  he 
becomes  progressively  more  complex,  as  his  desires  become  more 
and  more  difficult  of  fulfilnient,  as  he  demands  more  and  more  of  the 
world,  the  individvial  finds  that  he  has  to  put  off  fulfilment  further 
and  further  into  the  future  and  be  satisfied  to  struggle  perhaps  for 
years  to  attain  some  specific  end. 

Conflict  is  therefore  at  the  very  basis,  the  very  root  of  mental  life; 
the  adjustment  of  the  individual  to  the  world  of  reality  is  by  no  means 
the  passive  molding  by  external  forces,  but  the  individual  is  constantly 
and  actively,  in  his  mind  at  least,  reaching  out  and  trying  to  mold  the 
world  to  suit  himself.  Conflict  is  even  more  universal  than  this 
statement  would  indicate.  It  is  not  only  at  the  basis  of  mental  life 
but  of  all  life.  The  change  from  unicellularity  to  mult>cellularity  is  a 
change  brought  about,  so  to  speak,  by  the  necessity  for  a  finer  adjust- 
ment to  functional  problems  effected  by  the  specialization  of  the 
functions  of  groups  of  cells — organs.  These  groups  of  cells  have,  there- 
fore, to  relinquish  certain  of  their  tendencies  in  favor  of  a  greater 
specificity  of  response  which  is  calculated  better  to  serve  the  individual 
— biological  unit — as  a  whole.  In  the  mental  sphere  the  analogy  is  as 
between  the  wishes  of  the  individual  as  such  and  the  welfare  of  the 
social  group.  These  two  groups  of  impulses  constantly  cross  and 
as  a  result  the  individual  has  constantly  to  renounce  his  personal, 
selfish  ends  to  the  welfare  of  the  group  and  to  sublimate  his  per- 
sonal satisfaction  in  the  reward  of  social  esteem.  Just  as  groups  of 
cells  get  out  of  hand  when  they  are  not  adequately  integrated  in  the 
general  scheme  of  the  whole  individual  and  produce  tumor  formations 
so  the  individual  who  is  not  adequately  integrated  as  a  member  of  the 
social  group  becomes  asocial  and  a  group  of  such  individuals  becomes 
the  nucleus  of  a  distinctive  social  movement. 

i\n  important  corollary  to  this  concept  is  that  the  conflict  is  always 
between  diametrically  opposed  tendencies.  In  general  the  great 
tendencies  are  self -preservative  and  race-preservative  (creative), 
symbolized  at  the  symbolic  (psychological)  level  in  various  ways  as 
between  death  and  life,  bad  and  good,  left  and  right,  down  and  up, 
etc.    This  is  the  basis  of  the  ambivalency  of  the  symbol. 

It  is  from  this  basal  fact  of  conflict  that  there  take  origin  two 
forms  of  thinking,  an  understanding  of  which  is  of  great  importance 


INTRODUCTION  777 

for  the  comprehension  of  the  psychoneuroses,  in  fact  of  all  behavior, 
sick  or  well.  Thinking  which  is  dominated  by  the  reality  motive,  the 
thinkmg  which  is  a  conscious  intentional  effort  at  efficient  relation 
with  reality,  is  the  thinking  to  which  the  word  thinking  is  usually 
applied.  But  there  is  another  kind  of  thinking,  the  thinking  by 
fantasy  formation,  which  is  of  great  importance.  In  this  form  of 
thinking  it  is  not  the  reality  motive  that  dominates,  but  the  pleasure- 
pam  motive.  The  other  hom  of  the  conflict  is  here  represented,  and  in 
moments  of  quiescence  when  the  real  world  slips  away  from  our  vision 
and  we  settle  back  within  ourselves,  our  thoughts  flow  without  refer- 
ence to  this  outside  world,  they  come  and  go  without  critique  on  our 
part.  We  are  dreammg,  perhaps  in  sleep  or  perhaps  in  waking,  and 
the  fancies  which  come  at  these  moments  of  rumination  are  all  wish- 
fulfilling  fancies  controlled  by  the  pleasure  motive  and  represent  the 
satisfaction  of  desires  which  are  either  put  off  or  rendered  incapable 
of  fulfilment  in  the  real  world.  These  thoughts  are  not  only  the 
thoughts  that  dreams  are  made  of,  but  the  thoughts  which  the  psy- 
choneuroses are  made  of,  and  are  therefore  of  immense  importance 
for  their  understanding. 

From  the  very  first  the  immediate  satisfaction  of  desire  is  frus- 
trated, to  be  technical,  it  is  repressed  and  some  other  form  of  activity 
has  to  be  substituted,  for  example  in  later  life,  to  use  our  same  illus- 
tration, mstead  of  maintaining  the  immediate  relationship  between 
hunger  and  food,  there  is  introduced  another  series  of  factors,  repre- 
sented by  work  and  compensation  for  work  in  the  shape  of  money, 
which  money  may  be  exchanged  for  food.  And  so,  instead  of  the 
immediate  relationship  that  obtains  in  infancy  a  more  remote 
relationship  is  maintamed,  and  the  activities  instead  of  going  straight 
to  their  goal  take  a  more  or  less  circuitous  and  involved  path.  The 
original  relationship  therefore  tends  to  be  lost  sight  of,  and  the  more 
involved  and  complicated  one  takes  its  place.  There  are,  therefore, 
gradually  throughout  the  period  of  development,  all  sorts  of  desires 
being  repressed  which,  thus  put  out  of  consciousness,  are  replaced  by 
other  forms  of  activity.  The  desires  which  belong  to  infancy  and  which 
thus  are  early  repressed  and  superseded  by  other  forms  of  activity, 
constitute  the  material  out  of  which  the  unconscious  is  formed  and  the 
material  from  which  come  the  activating  moments  for  fantasy  for- 
mation. The  discrepancy  between  desire  and  fulfilment,  then,  is 
compensated  in  later  life  by  the  wish-fulfilling  fantasies  that  have 
their  origin  in  the  repressed  material  of  infancy  and  occupy  the  realm 
of  the  unconscious. 

Between  this  realm  of  the  unconscious,  which  contains  relatively 
infantile  mateiial  only,  and  the  realm  of  the  clearly  conscious,  there  lies 
the  realm  of  the  so-called  foreconscious,  which  contains  the  material  of 
recent  experience,  material  which  is  quite  easily  made  conscious.  In 
other  words,  it  is  just  out  of  mind  and  it  is  not  difficult  to  bring  back 
into  the  focus  ofiittention  when  the  individual  so  desires. 

The  region  of  the  unconscious  is  of  very  great  importance  for  an 


778  NEUROSES,  PSYCHONEUROSES,  PSYCHOSES 

understanding  of  the  pyscboneuroses,  because  it  represents  the  region 
of  the  deepest  repressions,  of  the  thoughts  that  are  least  Hke  the  present 
conscious  thoughts,  and  which,  therefore,  when  they  break  through 
into  consciousness,  produce  symptoms  that  are  so  grotesque  and 
strange  appearing  upon  the  surface  and  non-understandable,  not  only 
to  the  onlooker,  but  to  the  patient  himself.  It  is  therefore  desirable 
to  know  something  of  the  nature  of  the  unconscious  and  of  its  content. 
To  do  this  certain  features  in  the  development  of  the  child,  particularly 
those,  of  course,  that  are  at  a  later  date  repressed  and  superseded  by 
other  activities,  will  have  to  be  described. 

Before  doing  this,  however,  it  is  necessary  to  point  out  that  all  the 
activities  of  the  individual  lead  in  one  of  two  directions,  viz.,  in  the 
direction  of  self-preservation,  the  nutritive  activities,  or  in  the  direction 
of  race-preservation,  the  sexual  activities.  The  energy  which  drives 
toward  these  goals  may  be  called  the  libido  and  so  it  may  be  spoken 
of  as  the  nutritive  or  the  sexual  libido,  not  as  different  kinds  of  energy 
but  as  different  directions,  different  pathways  along  which  the  energy 
is  being  used.  Now  each  organ  in  the  body  tends  to  preserve  itself  but, 
on  the  other  hand  must  give  some  of  its  energies  to  the  preservation 
of  the  whole  individual  just  in  the  same  way  that  an  individual  member 
of  society  while  he  strives  to  take  care  of  himself  must  give  something, 
in  the  way  of  taxes,  for  the  general  weal. 

Self-preservation  and  race-preservation  are  seen  thus  to  be  funda- 
mentally opposed  to  one  another,  the  former  implying  gettmg  and 
keeping,  the  latter  giving.  One  who  keeps  that  which  he  should  give 
is  called  selfish. 

From  the  few  words  already  devoted  to  the  evolution  of  the  indi- 
vidual from  the  early  condition  of  relative  omnipotence  it  will  be  seen 
how  the  change  has  to  be  one  of  progressive  socialization.  For  example, 
from  the  infantile  immediate  relationship  hunger — food  there  is  devel- 
oped the  more  remote  relationship  hunger — work — money — food  in 
conformity  with  the  social  requirements.  The  libido  has  to  be  social- 
ized or  to  use  the  more  frequent  term,  sublimated.  The  instincts  of  the 
individual  remain  the  same  but  cultural  advance  depends  upon  a 
projection  of  these  instinctive  tendencies  upon  ends  that  yield  less 
immediate  concrete  satisfactions  and  are,  with  the  progress  of  social 
evolution,  progressively  more  socially  useful,  as  in  the  example  of  the 
instinct  of  hunger  just  cited. 

In  that  proportion  in  which  the  individual  is  unable  to  effectually 
sublimate  his  libido,  to  break  away  from  earlier,  infantile  ways  of 
pleasure  seeking  to  higher  social  forms  of  behavior  he  is  crippled  in  his 
capacity  to  live  at  his  best,  and  this  crippling  in  its  milder  manifes- 
tations we  call  the  neuroses  and  the  psycho  neuroses,  in  its  severer 
forms  the  psychoses,  and  in  its  most  severe  forms  the  various  grades 
of  defect  extending  to  the  depths  of  imbecility  and  idiocy. 

It  is  quite  obvious  that  the  idiot  and  imbecile  require  the  same  sort 
of  solicitous  care  as  the  normal  infant.  In  the  higher  grades  of  person- 
ality defect,  however,  the  necessity  is  clothed  in  symbols  which  distort 


INTRODUCTION  779 

and  obscure  the  meanings  in  accordance  with  the  mechanisms  already 
described  as  at  work  in  dreams  (q.  v.).  For  example,  a  patient  develops 
the  necessity  of  a  particular  dietary  which  can  only  be  provided  in 
the  home  and  every  effort  to  go  out  from  the  home  results  in  a  gastro- 
intestinal upset  because  of  the  inability  to  obtain  it.  By  this  symbolic 
mechanism  the  patient  is  therefore  permitted,  under  the  guise  of 
invalidism,  to  remain,  like  a  little  child  in  the  home  situation,  cared 
for  and  protected  from  the  world  of  reality  by  the  parents.  In  such  a 
case  the  general  result  pointed  is  not  only  attained  but  analysis  will 
show  that  there  was  an  early  fixation  upon  the  gastro-intestinal  ways 
of  pleasure  seeking  (a  so-called  partial  libido  trend)  which  determines 
the  particular  form  of  the  later  symptoms. 

Then  as  regards  the  more  distinctly  race-preservative  or  sexual 
libido:  in  the  early  infancy  the  child's  love  is  very  naturally  given 
out  to  the  only  people  to  all  intents  and  purposes  who  constitute  his 
milieu,  namely  the  members  of  the  immediate  family,  the  father,  the 
mother,  the  brother,  sister,  and  perhaps  nurse.  This  love,  contrary 
to  the  usual  way  of  thinking  of  it,  is  very  definite  in  its  direction, 
and  from  a  very  early  date  presents  certain  sexual  characteristics. 
Of  these  sexual  characteristics  jealousy  of  a  younger  brother  or  sister 
who  comes  into  the  family  and  deflects  a  certain  amount  of  affection 
which  the  child  would  otherwise  enjoy  is  wdthin  the  observation  of 
most  people,  while  the  fact  that  the  love  of  the  child  is  given  out  to 
the  members  of  the  family,  characteristically  the  parent  of  the  opposite 
sex,  is  not  a  matter  of  such  common  observation,  but  a  matter  of  great 
importance  psychologically.  As  the  child  develops  these  loves  are 
repressed  and  covered  into  that  all-inclusive  amnesia  for  the  infantile 
period,  and  when  adulthood  comes  along  and  the  child  has  grown  to 
manhood  or  womanhood  and  finds  its  mate,  the  love  which  had  before 
been  spent  upon  the  members  of  the  family  now  finds  its  true  object. 

It  is  this  infantile  love  for  the  members  of  the  family  that  is  the  root 
for  so  many  of  the  incest  fantasies  of  the  psychoneuroses  and  the 
psychoses.  This  love  for  the  parent  of  the  opposite  sex,  for  example, 
if  it  breaks  through  into  the  clear  realm  of  consciousness  becomes  a 
horrid  thmg  incompatible  with  the  individual's  peace  of  mind.  Such 
things  are  quite  common.  For  example,  a  patient  marries  a  man 
who  unfortunately  presented  a  number  of  very  close  resemblances 
to  her  father.  These  resemblances  served  to  stir  into  activity  the 
unconscious  love  for  the  father,  and  she  therefore,  in  her  feelings  toward 
her  husband,  is  outraged  beyond  all  endurance, -for  it  is  as  if  she  were 
married  to  her  father.  Life  with  her  husband  is  quite  unendurable.  She 
is  constantly  flying  into  passions,  assaulting  him,  upbraiding  him,  etc. 

If  this  psychology  is  the  usual  psychology,  why  is  it  that  all  people 
are  not  in  danger  from  such  sources  ?  Perhaps  they  are  to  a  lunited 
degree,  but  it  is  necessary  to  bear  certain  things  in  mind  to  under- 
stand how  the  unconscious  becomes  mLxed  up,  as  it  were,  in  the  daily 
life  of  the  individual,  as  in  the  case  just  cited.  Individuals  with  such 
unconscious  father  complexes  will  get  along  in  life  perhaps  quite 


780  NEUROSES,  PSYCHONEUROSES,   PSYCHOSES 

well  until  they  meet  some  difficulty.  The  difficulty  drives  them  back 
within  themselves,  it  prevents  the  outward  flow  of  interest  into  reality, 
makes  them  egocentric,  introspective,  they  are  unable  to  make  an 
efficient  reaction,  and  they  therefore  are  driven  back  to  fantasy 
formation  where  things  come  true  and  the  difficulties  are  all  removed. 
The  reason  why  this  driving  back  of  the  psychophysical  energy  within 
the  individual  mider  conditions  of  stress;  the  reason  why  this  should 
stir  up  a  particular  complex,  is  because  in  the  life  of  the  individual 
there  has  been  an  undue  fixation  at  that  point  in  the  course  of  develop- 
ment. The  patient  just  cited  had  never  been  able  to  emancipate 
herself  as  she  should  have  from  the  necessity  for  the  loving  care  and 
tenderness  and  protection  of  the  father  and  to  go  out  into  the  world 
and,  so  to  speak,  stand  upon  her  own  feet,  and  when  difficulties  arose 
in  her  life  and  she  was  thrown  back  upon  herself,  she  went  back  to 
that  point  at  which  there  had  been  an  infantile  fixation,  where  she  had 
previously  gained  satisfaction. 

From  the  few  words  of  description  of  this  patient's  condition,  who 
had  symbolically  married  her  father,  it  will  be  seen  how  important  it 
becomes  to  know  the  content  of  the  fantasies,  and  this  is  best  deter- 
mined by  a  study  of  the  dreams,  and  without  going  into  the  principles 
of  dream  analysis,  which  are  out  of  place  here,  it  is  well  to  remember 
that  the  neurosis  or  the  psychoneurosis,  like  the  dream,  is  not  only  a 
compromise  between  desire  and  fulfilment,  but  it  is  a  wish-fulfilling 
mechanism  that  brings  to  pass  the  fulfilment  both  of  the  wish  in  the 
foreconscious,  the  wish  with  reference  to  the  difficulty  that  caused  the 
introversion  in  the  first  place,  and  also  the  wish  in  the  unconscious, 
the  wish  at  the  fixation-point,  which  serves  as  a  pull-back  once  the 
introversion  has  started.    (See  Psychoanalysis  in  Chapter  II.) 

Bearing  these  facts  in  mind  it  will  be  easy  to  understand  that  the 
child's  first  sexual  feelings  have  reference  to  its  own  body,  it  is  auto- 
erotic;  that  next  its  sexual  feelings  are  transferred  upon  those  imme- 
diately about  him,  upon  someone  most  like  himself,  therefore  of  the 
same  sex  (homosexual,  narcissistic  stage) .  It  seeks,  in  other  words, 
outside  of  itself,  but  still  an  object  as  much  like  itself  as  possible.  And 
finally,  the  period  of  object  love,  when  fulfilment  is  had  in  an  entirely 
different  individual  and  of  a  different  sex  (heretosexual  stage). 

In  addition  to  the  above  facts  the  child  not  only  passes  through  these 
various  stages  of  psychosexual  development  mentioned,  but  in  its 
earliest  infantile  state  it  is  susceptible,  theoretically  at  least,  of  deflec- 
tion in  any  direction.  So,  for  example,  at  the  period  when  the  love  is 
given  out  to  those  in  the  immediate  surroundings  it  not  infrequently 
is  given  out  to  a  member  of  the  same  sex;  differences  in  sex  are  not 
appreciated  in  these  early  days  and  come  only  with  later  development. 
Other  differences  are  equally  indefinite.  The  erogenous  zones  of  which 
the  genital  organs  are  onlj^  one,  and  the  anus  and  the  lips  constitute 
the  most  important  additional  ones,  are  still  more  or  less  indefinite, 
and  sexual  erethism  may  be  predominantly  focalized  in  any  one  of 
them.    And  so  the  roots  of  the  various  so-called  perversions  are  found 


INTRODUCTION  781 

in  these  early  fixations.  The  determining  factor  in  the  early  fixations, 
the  mechanisms  that  have  brought  them  about,  in  short,  their  uncov- 
ering, can  only  be  accomplished  by  fathoming  the  unconscious.  This 
is  the  work  of  psychoanalysis  and  the  most  prominent  means  at  its 
disposal  at  the  present  time  is  by  the  analysis  of  dreams.  (See  Chapter 
II  on  jNIental  Examinations.) 

The  analysis  of  the  sjTnptoms  not  only  uncovers  such  individual 
material  along  the  lines  indicated  but,  going  deeper,  brings  out  still 
more  fundamental  traits  which  find  normal  expression  in  the  ways  of 
thinking  of  primitive  man.    This  is  the  so-called  archaic  material. 

Bearing  in  mind  the  concept  of  the  individual  as  an  integrated  whole 
and  the  psyche  as  expressing  the  tendencies  of  the  whole  we  are  pre- 
pared to  find  that  instinctive  tendencies,  requiring  as  they  do  the 
functioning  of  the  several  organs  to  bring  about  their  satisfaction, 
should,  when  thwarted  (repressed),  express  themselves  in  states  of 
physiological  tension  in  those  organs  which  would  be  called  upon  to 
function  in  the  event  the  tendencies  could  find  expression.  These 
states  of  physiological  tension  are  expressed  as  visceral  tonicities 
(pylorospasm,  cardiospasm,  spastic  constipation,  increased  blood- 
pressure,  etc.)  and  postural  tensions  (bodily  attitudes,  peculiarities  of 
using  the  limbs,  facial  expression,  characteristic  poses,  mannerisms, 
etc.)  and  the  energy  used  in  effecting  the  repression  can  be  conceived 
as  bound  up  in  these  tensions.^ 

These  are  the  mechanisms  which  recent  studies  of  the  autonomic 
apparatus  and  the  emotions  have  disclosed  and  which  serve  to  fill  that 
long  existing  hiatus  between  mind  and  body  and  do  away  finally  for 
the  need  of  considering  them  as  in  any  way  separate  and  distinct.  They 
are  the  mechanisms  that  explain  that  host  of  somatic  conditions  which 
have  long  been  knowTi  to  be  of  psychogenic  origin  and  to  suggest  an 
explanation  for  many  others  whose  etiology  at  present  is  obscure. 

Symbolism. — All  of  the  various  difficulties  of  the  individual — his 
conflicts — express  themselves  at  the  psychological  level  or  the  s\Ta- 
bolic  level  as  we  have  designated  it  in  this  book,  by  ideas  and  feelings — 
symbols.  Just  as  the  original  tendencies  of  the  child  are  relatively 
few  so  his  later  and  more  elaborated  activities  must  be  the  outgrowth 
from  these  simple  beginnings.  A  highly  developed  curiosity  may 
result  in  a  scientist,  a  child  of  marked  sadistic  traits  may  develop 
into  a  butcher  or  a  surgeon,  the  child  who  was  inordinately  fond  of 
making  mud  pies  may  later  develop  as  an  engineer  or  architect  and 
these  later  developments  can  be  seen  to  be  outgrowths  of  the  earlier 
tendencies  and  to  symbolize  them.  We  see  this  particularly  even  in 
those  individuals  whose  activities  are  plainly  attempts  at  compensa- 
tion for  obvious  defects.  The  crj^Dtorchid  chooses  a  highly  virile 
occupation — cowboy — or  becomes  dictatorial  and  overbearing;  the 
individual  with  a  pronounced  father  hate  leans  toward  anarchistic 
social  views;  the  impotent  man  becomes  a  prude  or  perhaps  spends  his 

'  Kempf,  E.  J.:  The  Autonomic  Functions  and  the  Personality,  Nervous  and  Mental 
Disease  Monograph  Series  No.  28. 


782  NEUROSES,  PSYCHONEUROSES,   PSYCHOSES 

time  and  money  in  trying  to  invent  a  big  gun  or  perfect  a  perpetual 
motion  machine. 

One  can  interpret  the  symbol  in  two  ways,  namely,  from  the  phylo- 
genetic  standpoint,  as  exhibiting  the  stage  in  evolution  and  develop- 
ment exemplified;  or  from  the  ontogenetic  standpoint,  as  traceable 
to  concrete  individual  experiences.  For  example,  a  person  who  shows 
his  extreme  mother  attachment  by  his  choice  of  food,  living  largely 
on  milk  and  soft  foods,  shows  that  in  that  region  of  his  conduct  he  is 
still  acting  like  the  primitive  man  who  has  faith  in  magic — -by  using  the 
s^onbols  of  the  infantile  dependence  upon  his  mother  he  tries  to  continue 
that  dependence  in  existence  and  so  get  the  feeling  of  security  it  gives. 
Such  an  individual,  however,  might,  theoretically  at  least,  have  used 
many  other  sjTnbols  other  than  food  for  the  same  purpose.  For  example, 
he  might  have  used  the  symbol  of  some  particular  style  of  clothing, 
or  have  retained  his  infantile  style  of  handwriting,  or  copied  a  peculiar 
form  of  speech,  such  as  a  lisp  or  stammer,  from  his  mother  or  in  innum- 
erable other  ways  symbolized  his  dependence.  Which  of  the  myriad 
of  available  means  he  uses  depends  upon  what  his  personal  experience 
has  been. 

The  first,  the  phylogenetic,  or  race  interpretation  of  symbols  gives 
us  the  material  for  the  foundation  of  an  interpretative  psychology. 
The  second,  the  ontogenetic  or  individual  interpretation  leads  us  to 
the  actual  experiences  in  the  lives  of  individuals  which  have  led  to  the 
choice  of  the  symbol  and  is  the  method  of  maximum  advantage  in  the 
approach  to  the  concrete  problems  of  the  psychosis  as  it  occurs  in  a 
particular  individual. 

Classification. — Although  we  have  already  dealt  with  the  principles 
underlying  a  classification  of  diseases  of  the  nervous  system  in 
the  Introduction  of  this  work,  it  would  seem  that  an  additional 
short  discussion  of  classification  as  applied  to  diseases  of  the  mind 
is  warranted  at  this  point  if  for  no  other  reason  than  because  classi- 
fications have  occupied  the  attention  of  psychiatrists  so  greatly  in 
,  the  past  and  have  been  the  bugbear  of  the  student  of  this  branch  of 
medicine. 

The  concept  of  disease  as  being  something  that  invaded  the  indi- 
vidual and  possessed  him  like  the  medieval  devil,  has  perhaps  lingered 
here  longer  than  in  other  departments  of  medicine,  where  there  has  been 
greater  success  in  running  down  the  etiological  factor  or  in  defining 
the  pathological  processes.  But  even  in  such  a  definite  group  as  tuber- 
culosis it  is  at  once  recognized  that  the  organism  may  not  only  be 
invaded  in  any  of  its  parts,  but  that  of  two  organisms  similarly  invaded 
the  symptomatic  picture  may  be  widely  different.  In  other  words, 
the  picture  of  the  so-called  disease  in  any  particular  instance  is  depend- 
ent upon  the  nature  of  the  disintegrating  factor,  the  nature  of  the 
invaded  host,  and  the  interaction  between  the  two.  The  resulting 
picture  is  but  the  expression  of  this  conflict  as  shadowed  forth  in  the 
s.ymptoms.  This  is  not  only  true  in  a  narrow  sense,  but  it  is  true  in  the 
broad  sense  that  it  is  the  nature  of  the  host  that  m  the  first  instance 


INTRODUCTION  783 

makes  possible  the  invasion  and,  too,  accounts  for  the  location  of  that 
invasion.  The  "shut  in"  type  of  personality  which  manifests  itself 
in  a  tendency  to  withdraw  from  the  social  environment  and  lead  a 
cloistered  existence  tends  at  the  psychological  level  in  the  direction 
of  precox,  but  at  the  physiological  level  in  the  direction  of  pulmonary 
tuberculosis.  Some  day,  not  far  distant,  we  shall  be  able  to  correlate 
this  t>T)e  of  personality  with  underlying  physiological  conditions 
(organ  inferiority  perhaps)  which  will  enable  us  to  express  this  relation 
in  much  more  definite  terms.  In  any  case  the  implication  is  apparent 
that  the  organism,  in  its  various  reacting  tendencies,  expresses  itself, 
not  alone  at  the  physiological  level,  but  of  necessity  also,  and 
undoubtedly  quite  as  definitely,  at  the  psychological  level.  The 
organism  is  a  biological  unit  and  whatever  affects  it  in  any  of  its  parts 
has  its  psychological  reverberation.  Disease  is  the  resulting  picture  of 
a  process  which  is  disintegrating  in  its  tendencies  and  mental  disease 
is  that  portion  of  the  picture  which  is  visible  at  the  psychological  level. 
The  organism  as  a  mechanism  "goes  off "  as  a  whole  and  the  resulting 
s\Tnptoms  of  its  reaction  are  manifested  at  every  reacting  level  and 
perhaps  should  be  as  accurately  read  at  one  level  as  another.  All 
levels,  however,  need  to  be  interrogated  to  get  an  adequate  under- 
standing of  all  that  is  going  on. 

Classifications  in  the  part  have  only  grouped  those  symptoms  which 
seemed  to  stick  together  for  some  reason  and  have  had  little  else  to 
recommend  them,  founded  as  they  were  upon  a  purely  descriptive 
psychiatry.  The  growth  of  an  interpretative  psychiatry,  however, 
has  resulted  from  a  deeper  search  for  causative  factors  and  the  defini- 
tion of  the  mental  mechanisms  involved  in  the  production  if  the 
sjTnptoms  and  the  day  is  rapidly  approaching  when  mental  disorders 
will,  as  a  result,  be  classified  by  describing  and  defining  the  mechan- 
isms involved  and  showing  how,  in  each  instance,  the  symptoms  have 
come  to  pass  as  a  result  of  their  operation  upon  the  material  at  hand. 
Finally,  these  mechanisms  will  be  correlated  with  the  organism's  reac- 
tions at  other  than  the  psychological  level  and  the  reaction  of  the  organ- 
ism as  a  whole  will  be  understood  in  terms  of  all  the  factors  involved. 
This  is  the  goal  of  present-day  psychiatry  and  rapid  strides  are  being 
made  in  bringing  it  within  the  range  of  a  practical  vision.  It  is  one 
of  the  distinct  efforts  of  the  authors  of  this  work  to  indicate  how  this 
is  coming  about  by  injecting  all  through  the  discussions  of  the  disorders 
predominantly  manifested  at  the  different  reacting  levels  (physico- 
chemical,  sensorimotor  and  symbolic)  indications  of  how  the  given 
reactions  under  discussion  may  manifest  themselves  at  the  other 
levels. 

The  various  mental  disorders  will  be  considered  under  the  usual 
conventional  captions,  but  in  the  interpretative  section  the  mechanisms 
involved  will  be  defined  and  discussed  in  a  manner  that  will  pave  the 
way  for  this  larger  approach  to  the  problems  of  psychopathology. 

It  is  the  desire  of  the  authors  to  keep  the  student's  mind  open  for 
this  broader  vision,  because  it  is  appreciated  that  in  the  past,  with 


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50 


786  NEUROSES,  PSYCHONEUROSES,   PSYCHOSES 

its  emphasis  on  classification,  too  much  effort  was  expended  in  placing 
a  given  psychosis  at  a  certain  point  in  some  scheme  of  classification, 
in  other  words,  giving  it  a  name,  and  only  too  often  interest  stopped 
when  the  appropriate  label  had  been  appended.  Only  by  understand- 
ing the  processes  that  are  involved  can  an  intelligent  therapy  be 
developed.  A  classificatory  scheme  which  does  this  will  remain 
sufficiently  fluid  to  be  useful  rather  than  tending,  as  do  the  older 
schemes,  to  become  static  and  fixed  beyond  the  point  of  capacity  to 
let  in  new  concepts  or  adjust  to  new  hypotheses. 

Such  a  scheme  of  classification  is  that  proposed  by  Kempf.^  The 
psjxhoses  as  a  group  are  first  divided  into  benign  and  pernicious  not, 
however,  on  the  basis  with  which  we  are  already  familiar,  namely, 
that  the  outcome  in  recovery  or  chronicity  shall  be  a  factor  in  deter- 
mining the  diagnosis.  In  such  a  scheme  the  patient  whose  psychosis 
had  been  difficult  to  diagnose  was  put  in  the  manic-depressive  group 
if  he  recovered  but  in  the  precox  group  if  he  did  not.  It  is  similar  to 
the  situation  before  the  days  of  bacteriological  diagnosis.  A  child 
with  sore-throat  might  appear  to  have  diphtheria  but  if  it  got  well  it 
was  tonsillitis.  A  fatal  outcome  was  necessary  to  clinch  the  diagnosis 
of  diphtheria.  In  Kempf's  scheme  the  qualifications  benign,  pernicious 
do  not  refer  to  the  outcome  alone  in  this  way  but  indicate  the  serious- 
ness of  the  psychosis  as  dependent  upon  the  patient's  attitude  toward 
his  difficulties. 

The  psychoses  are  then  divided  into  suppression,  repression,  com- 
pensatory, regression  and  dissociation  types.  The  meanings  of  these 
terms  are  sufficiently  set  forth  in  the  table.  They  represent  a  scale  in 
the  seriousness  of  the  psychological  difficulties.  It  is  important  to 
understand,  in  using  these  terms,  that  they  are  not  mutually  exclusive; 
that  dissociation,  for  example,  is  a  result  of  severe  regression,  and  that 
a  patient  may  begin  with  a  compensatory  psychosis  but  gradually 
lose  ground  and  finally  develop  a  dissociation,  or,  on  the  other  hand, 
come  under  observation  as  a  dissociation  psychosis  but  get  better  and 
present  the  features  of  regression  and  suppression  types  on  the  way  to 
recovery.  In  the  psychiatry  of  the  nineteenth  century  a  simple 
melancholia  today  might  be  a  hallucinated  melancholia  tomorrow. 
In  this  scheme,  however,  it  is  not  the  apparent  symptoms  which  are 
used  as  a  basis  of  classification  but  the  mechanisms  which  produce  them. 

In  the  columns  which  follow  giving  the  principal  symptoms  of  these 
conditions,  those  physiological  disturbances  of  function  are  set  forth 
with  regard  to  which  there  is  no  serious  controversy.  In  the  future 
development  of  psychiatry  there  will  undoubtedly  be  an  enormous 
advance  of  our  knowledge  of  the  specific  correlation  of  psychic  states, 
and  physiological  states  along  the  lines  indicated  here  and  elsewhere 
throughout  this  book.  .  At  least  it  must  be  apparent  that  for  an  accurate 
sizing-up  of  an  individual  it  is  as  important  to  adequately  evaluate 
the  meanings  of  his  bodily  attitudes  (visceral,  postural)  as  it  is  to  eval- 
uate his  attitude  of  mind. 

>  The  Mechanistic  Classification  of  Neuroses  and  Psychoses  Produced  by  Distortion 
of  Autonomic-Affective  Functions,     Jour,  Nerv.  and  Ment.  Dig,,  August,  1919, 


CHAPTER  XV. 
THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES. 

The  field  of  the  neuroses  and  the  psychoneuroses  is  not  only  the 
broadest  field  in  psychiatry,  but  perhaps  the  broadest  field  in  all 
medicine.  Not  only  is  the  field  an  extensive  one  in  point  of  the  actual 
number  of  persons  who  suffer  from  these  afflictions,  but  it  is  a  field 
of  very  great  importance  for  the  understanding  of  mental  phenomena 
both  in  the  realm  of  disease  and  in  the  realm  of  the  healthy.  It  is 
in  the  manifestations  of  these  disorders,  which  have  been  well  termed 
borderland  states,  that  the  early  departures  from  the  normal  can  be 
found  and  studied,  which,  in  much  more  aggravated  form,  appear  in 
the  psychoses. 

Then  again  problems  that  lend  themselves  much  more  satisfac- 
torily to  therapeutic  attack  are  to  be  found  here,  conditions  which, 
although  they  may  represent  practically  any  degree  of  departure  from 
the  normal,  are,  as  a  rule,  capable  of  material  alleviation,  if  not 
actual  cure  by  therapeutic  measures.  When  the  immense  number  of 
people  who  are  affected  by  nem-oses  or  psychoneuroses  is  considered, 
the  great  amount  of  suffering  that  these  diseases  entail,  the  impaired 
efficiency  in  which  they  result,  and  then  consider  that  they  are,  for 
the  most  part,  susceptible  of  great  improvement,  if  not  actual  cure  by 
therapeutic  endeavor,  it  will  be  seen  that  this  department  of  medicine 
is  not  only  the  most  attractive,  but  is  one  which  perhaps  offers  most 
in  the  way  of  results. 

The  number  of  people  actually  afflicted  with  these  conditions  is 
difficult  to  estimate.  The  frank  cases  of  the  psychoneuroses  and  the 
actual  neuroses  are  very  numerous,  as  are  also  more  or  less  larvated 
conditions,  while  on  the  other  hand,  every  specialist  in  medicine  is 
dealing  constantly  with  manifestations  of  these  conditions  as  they 
appear  upon  the  physical  side.  Perhaps  these  physical  manifestations 
are  best  known  to  the  gastro-enterologist,  the  gynecologist,  and  the 
genito-urinary  surgeon,  but  the  ophthalmologist,  the  laryngologist, 
the  internist,  and  in  fact  every  specialist  has  his  share. 

In  the  following  chapters  the  disorders  at  the  symbolic  (psycholog- 
ical) level  will  be  discussed.  It  is  a  fundamental  tenet  of  this  book, 
however,  that  the  three  levels  treated  herein,  viz.,  the  vegetative,  the 
sensorimotor,  and  the  symbolic  are  not  mutually  distinct  but  only 
different  aspects  of  the  strivings  of  the  individual  and  the  component 
parts  thereof  as  expressed  by  the  various  forms  of  solutions  and  com- 
promises made  in  the  processes  of  integration  and  adjustment.  (See 
introduction.)  Not  only  are  the  different  levels  not  distinct,  but  the 
same  symptom  may  arise  as  a  result  of  disturbance  at  any  one  o^  the 
level3.    For  example  constipation  may  be  due  to  a  disturbance  at  the 

(787) 


788         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

vegetative  level  (vagotonic  spastic  constipation),  at  the  sensorimotor 
level,  due  to  injury  or  disease  of  the  cord,  or  at  the  symbolic  level, 
a  purely  psychogenic  symptom.  Further  than  this,  purely  emotional 
causes  if  severe  or  of  long  duration  may  produce  structural  changes 
from  which  recovery  is  very  slow  or  impossible  or  may  precipitate, 
by  affording  favorable  conditions,  severe,  even  fatal  infections.  In  all 
of  these  conditions  the  individual,  as  such,  is  more  or  less  severely 
crippled  but  the  understanding  of  the  symptoms  and  their  proper 
treatment  must  depend  upon  a  comprehension  of  the  mechanisms 
involved  in  their  production. 

THE  PSYCHONEUROSES.    (HYSTERIA,  COMPULSION  NEUROSIS 
AND  "  SHELL  SHOCK.") 

Hysteria. — Historical. — To  write  the  history  of  hysteria  would  mean 
practically  to  write  the  history  of  medicine,  for  hysteria  stands  through- 
out the  ages  as  the  type  of  functional  disturbance  of  the  nervous 
system  which,  protean  in  its  manifestations,  is  found  associated  with 
all  great  therapeutic  movements  in  medicine.  Whether  it  be  the  thera- 
peutics of  religious  conversion,  of  Perkin's  tractors,  or  hypnotism,  or 
more  recently  of  persuasion,  a  considerable  proportion  of  the  patients 
who  recover  and  thereby  become  largely  responsible  for  the  vogue  of 
the  particular  therapeutic  measure  involved,  belong  to  the  great 
clinical  group  of  hysteria. 

Hysterical  manifestations  have  been  prominent  in  mental  epidemics 
that  have  swept  over  whole  continents,  while  the  more  specific  and  the 
more  grotesque  symptoms  have  always  been  observed  and  described. 

The  modern  period  in  the  history  of  hysteria  might  be  said  to  have 
begun  with  Charcot.  This  period  is  still  so  recent  as  to  be  within  the 
memory  of  many,  and  the  influence  which  the  Charcot  school  exerted 
is  still  all  too  dominant  in  certain  quarters.  The  picture  of  hysteria 
as  Charcot  drew  it,  particularly  of  the  grande  hysterie  with  its  regular 
march  of  histrionic  attitudinizing,  as  set  forth  in  the  world-renowned 
pictures  of  Richer,  is  familiar. 

For  many  years  following  Charcot  the  most  brilliant  work  in  eluci- 
dating the  hysteria  problem  was  done  in  France,  and  many  illustrious 
names  are  crov/ded  into  a  few  years.  All  sorts  of  explanations  were 
formulated,  theories  that  were  physiological,  that  were  psychological, 
and  that  were  biological,  with  numerous  variants  of  each.  The  most 
illuminating  worker  in  this  field  for  many  years,  the  one  whose  theories 
produced  the  greatest  influence  in  the  study  of  this  disease  was  Pierre 
Janet,  of  Paris.  His  was  a  theory  of  dissociation,  and  he  believed 
hysteria  to  be  purely  a  mental  malady.  It  was  due  to  a  poor  synthesis 
of  the  personality  which  enabled  certain  groups  of  ideas  to  drop  away 
from  effective  association  with  the  main  portion  of  the  personality 
and  occupy  a  region  which  Janet  termed  the  subconscious,  and  there 
existing  more  or  less  independently,  produce  their  results  irrespective 
of  corrections  from  the  rest  of  the  personality.    The  hysterical  manifes- 


THE  PSYCHONEUROSES  789 

tations,  then,  were  the  manifestations  of  these  split-off  parts  of  the 
personality.  Janet's  views  were  a  great  advance  upon  the  current 
concepts  of  hysteria,  but  although  they  rendered  possible  a  deeper 
insight  into  the  nature  of  the  disease  and  the  disease  processes,  they 
were  still  largely  descriptive,  though,  of  course,  the  description  was 
much  refined  from  that  of  Charcot. 

Various  kinds  of  dissociation  theories  have  been  built  up  by  investi- 
gators since  Janet,  and  the  dissociation  theory  was  variously  elaborated, 
particularly  in  this  country  by  Sidis,  White,  Prince  and  others.  It 
remained,  however,  for  a  Viennese  physician,  Sigmund  Freud,  to  get 
beyond  the  point  of  description  into  a  true  interpretative  attitude 
toward  the  disease. 

Freud  showed  that  the  reason  for  the  dissociation  was  that  the  dis- 
sociated ideas  were  out  of  harmony  with  the  rest  of  the  personality,  that 
they  represented  ideas  and  feelings  that  were  in  conflict  with  the 
conscious  tendencies  of  the  individual,  and  that  they  were  therefore 
repressed.  Regression  became  with  Freud,  then,  the  fundamental 
factor  at  the  basis  of  hysterical  manifestations,  an  active,  not  a  passive 
mental  factor  which  tended  to  put  out  of  mind  certain  inacceptable 
groups  of  ideas,  and  was  therefore  the  cause  of  the  dissociation. 

The  Mechanism  of  Hysteria. — Starting  with  dissociation  as  the  most 
fundamental  descriptive  term  applicable  to  the  hysterical  state — the 
doubling  of  the  personality,  in  the  sense  of  Janet — it  has  been  shown 
that  there  is  at  the  basis  of  this  process  of  dissociation  an  active  process 
called  repression,  which  has  as  its  function  the  splitting  off  of  inac- 
ceptable idea-constellations — complexes — from  the  main  body  of  the 
personality,  and  thus,  so  to  speak,  putting  them  out  of  mind.  It 
has  also  been  intimated  that  these  split-off  complexes  because  of 
being  split  off,  do  not  therefore  cease  to  act.  As  a  matter  of  fact  they 
go  on  functioning,  but  the  functioning  is  independent,  more  or  less, 
of  the  balance  of  the  personality. 

This  process  of  repression  and  dissociation,  following  upon  conflict, 
is  a  very  general  one  and  is  found  in  divers  mental  states  and  is  in 
fact  a  normal  process.  It  is  not  these  processes  or  mechanisms  which 
are  characteristic  of  any  particular  mental  disorder,  but  it  is  the 
way  in  which  the  split-off  complexes  manifest  themsekes  that  produces 
the  difl'erent  types  of  mental  disorders. 

From  what  has  been  said  it  will  be  seen  that  if  the  individual,  or 
more  specially,  the  psyche,  be  considered  as  being  a  complex  of  adaptive 
mechanisms  which  is  always  making  an  effort  to  come  into  closer 
adaptation  with  the  environment,  then  the  meaning  of  a  conflict  is 
that  there  enter  into  this  mechanism  certain  factors  to  which  it  can- 
not make  efficient  adaptation.  This  results  in  repression  and  splitting, 
but  the  whole  tendency  of  the  machine  is  to  readjust  effectively  by 
bringing  about  in  some  way  a  new  state  of  affairs.  In  the  conflict 
there  are  two  groups  of  tendencies  in  the  psyche  which  are  diametri- 
cally opposed  one  to  the  other.  Xo  solution  of  the  conflict  can  possibly 
be  brought  about  by  a  fulfilment  of  one  of  these  groups,  because  mani- 


790  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

festly  the  conflict  would  still  remain.  Therefore  any  readjustment 
that  takes  place  must  in  some  way  bring  to  pass  the  tendencies  of  both 
groups.  Inasmuch  as  these  groups  are  opposed  to  each  other,  such  a 
result  cannot  actually  be  brought  to  pass  in  the  world  of  reality  at  the 
level  of  the  conflict.  Therefore,  unless  an  adequate  adjustment  can  be 
brought  about  by  an  all-inclusive  synthesis  at  a  higher  level  an  artifi- 
cial world  which  is  not  governed  by  the  strict  laws  of  reality  has  to 
be  brought  into  existence  wherein  these  opposing  forces  can  both,  as 
it  were,  allegorically  find  their  ends  attained.  This  is  well  shown  in 
the  following  dream :  The  patient  said  "  she  saw  herself  dead,  lying  in 
a  coffin,  with  a  red  rose  in  her  hand."  The  red  rose  s^Tnbolized  her 
sweetheart  because  of  the  frequent  presents  of  red  roses  which  he  had 
made  to  her.  Being  dead  in  a  coffin  probably  has  several  meanings, 
but  among  others  has  the  meaning  of  a  regression.  The  coffin  is  the 
matrix,  it  s.\Tnbolizes  a  going  back  to  the  protection  of  the  mother, 
and  so  the  dream  s^Tnbolizes  the  two  opposing  desires,  one  infantile, 
the  desire  for  union  with  the  mother,  the  other  adult  and  recent,  the 
desire  for  union  with  her  sweetheart. 

In  the  different  mental  disorders  this  end  is  brought  about  in  differ- 
ent ways.  The  hysterical  mechanism  is  different  from  the  other 
mechanisms  inasmuch  as,  while  it  is  a  general  rule  that  the  painful 
affect  of  the.  split-off  complexes  is  drafted  oft'  by  various  channels  and 
thus  finds  expression,  and  while  it  is  a  general  rule  that  this  expression 
is  not  consciously  associated  with  the  idea  content  of  the  complexes 
themselves  so  that  the  patient  is  saved  from  a  realization  of  their 
true  nature,  is  thua-con served  from  an  appreciation  of  the  pain  that 
WQuld-jesult  if  the}^  were  understood  at  their  true  value,  in  hysteria 
the  painful  affect  is  drafted  off"  intobodily  mnervation,  thus  producing 
tbe_somatic  phenomena  of  hysteria^  l.iiis  is  the  process  of  conversion 
and  IS  characteristic  of  Tiysteria^  I'he  so-to-speak  strangulated, 
unreacted-to  emotion  of  the  split-off  complexes  manifests  itself  as  the 
physical  symptoms  of  the  psychoneurosis  and  in  this  way  the  strong 
affect  of  the  split-off  complex  is  weakened.  The  complex  is  robbed 
of  its  affect,  which  is  the  real  object  of  conversion  and  hence  its  value 
to  the  individual. 

This  is  perhaps  a  somewhat  involved  statement  but  a  simple  example 
will  make  it  clear.  A  patient  ought  to  make  a  call  upon  a  recently 
bereaved  friend.  This  is  recognized  as  a  distinct  obligation  but  the 
patient's  infantile  necessity  of  escaping  reality  and  seeking  pleasure 
makes  the  duty  seem  a  very  onerous  affair.  Thus  arises  a  conflict 
between  duty,  born  of  conscious  appreciation  of  the  social  obligation, 
and  desire,  born  of  the  childish  inability  to  make  the  necessary  sacrifice 
of  personal  comfort.  As  a  result  the  patient  develops  a  headache,  and 
so,  being  ill  does  not  have  to  go.  The  selfish  desire  is  thus  gained  and 
at  the  same  time  the  social  demands  are  satisfied  by  the  illness  which 
offers  an  acceptable  excuse,  while  the  painful  recognition  of  the  patient^s 
own  selfishness  is  converted  into  the  pain  in  the  head.  The  affect  is 
thus  displaced,  the  whole  situation  symbolically  distorted  and  the  two 


THE  PSYCHONEUROSES  791 

opposing  tendencies,  conscious  and  unconscious  both  satisfied.  Inci- 
dentally the  pain  in  the  head  is  a  self-punishment  for  not  obeying  the 
socially  useful  and  unselfish  demand  and  has  as  a  function  the  tendency 
to  drive  the  individual  along  the  path  of  development,  for  only  by 
following  this  path  can  the  pain  be  avoided.  An  adequate  adjustment 
by  a  synthesis  at  a  higher  level  would  result  in  making  the  call,  and 
wanting  to  make  it,  and  deriving  pleasure  and  satisfaction  from  having 
comforted  the  bereaved  person. 

This  is  such  an  example  as  everyone  has  constantly  presented  to 
him  by  all  manner  of  persons  placed  in  disagreeable  circumstances. 
The  tendency  to  develop  some  minor  physical  ill  as  an  excuse  and  an 
escape  from  a  recognized  duty  is  used  very  widely,  one  is  tempted  to 
say,  at  times  by  almost  everyone.  It  is  the  fundamental  hysterical 
(conversion)  mechanism  which  throws  upon  the  body,  makes  it  the 
scapegoat  of,  the  responsibility  for  our  moral  failures.  And  yet  more 
than  this.  It  produces  suft'ering  and  pain,  which  here  as  elsewhere, 
point  the  way  of  relief  by  making  the  wrong  path  as  unattractive  as 
possible. 

Symptoms. — The  symptomatology  of  hysteria  is  naturally  a  very 
complex  one,  but  from  what  has  been  said  it  will  be  seen  that  it  tends 
to  group  itself  more  especially  about  disturbances  of  motion  and  of 
sensation.  In  addition  to  this  it  also  tends  to  manifest  itself  in  certain 
crises. 

All  forms  of  paralyses  and  anesthesias  may  manifest  themselves. 
Paralyses  of  the  limbs,  either  singly  or  hemiplegia  with  or  without 
contracture,  are  common,  while  anesthesias  may  be  distributed  in 
almost  any  way,  involving  the  superficies  or  the  special  senses.  As  a 
rule,  of  course,  the  distribution  of  these  various  phenomena  do  not 
follow  the  anatomical  areas  of  nerve  supply.  They  show  a  symbolic 
grouping. 

The  disturbances  of  sensibility  are  of  many  forms.  Very  characteristic 
are  the  glove  and  stocking  anesthesias,  involving  the  extremities  of  the 
limbs,  hands,  a  lower  part  of  forearm  and  feet,  and  lower  portion  of  legs. 
Patches  of  anesthesia  may  be  found  upon  any  portion  of  the  cutaneous 
surface  and  they  may  be  widely  distributed  and  often  not  constant 
in  location  but  varying  with  different  examinations.  Hemianesthesia, 
especially  of  the  left  side  of  the  body,  crossed  and  alternating  forms 
are  found.  Light  touch  is  more  often  involved,  frequently'  deep 
pain  also,  while  insensitiveness  to  heat  and  cold  also  occurs. 

A  characteristic  form  of  anesthesia  which  is  very  frequently  found 
is  concentric  limitation  of  the  field  of  vision. 

These  anesthesias  do  not  follow  anatomical  areas  and  experiments 
will  readily  determine  that  they  are  psychological.  This  distribution, 
which  is  plainly  not  anatomical  is  as  plainly  psychological,  that  is,  the 
anesthesia  affects  the  hands,  arms,  etc.,  as  they  are  thought  of  by  the 
patient.  If  for  example  an  anesthetic  area  is  stimulated,  the  patient 
will  say  he  feels  nothing,  but  if  asked  to  guess  the  nature  of  the  stimulus, 
will  show  a  surprising  number  of  correct  replies. 


792  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

Hypoesthesias,  hyperesthesias,  various  forms  of  neuralgia,  especially 
visceral,  and  headache  are  also  frequently  hysterical. 

The  disturbances  of  motility  are  largely  various  forms  of  paralysis. 
Hemiplegia,  monoplegia  and  paraplegia,  with  or  without  contractures, 
are  the  commoner  forms.  Astasia  abasia  is  a  characteristic  hysterical 
condition. 

Spasms  of  various  parts  are  not  infrequent.  Spasms  of  the  tongue, 
of  the  face  and  of  the  extremities  when  not  demonstrably  of  organic 
origin  are  hysterical  as  a  rule.  Tremors,  myasthenic  types  of  reaction, 
and  easy  fatigability  are  frequent.  Choreiform  movements,  tics,  and 
certain  occupation  spasms  are  often  hysterical. 

The  speech  is  involved  frequently.  Hj^sterical  aphonia  is  well 
known  and  usually  a  diagnosis  is  warranted  if  the  patient  can  only 
whisper  replies  to  questions  and  an  examination  discloses  healthy 
vocal  cords.  Stuttering  is  frequently  hysterical  and  an  analysis  will 
show  that  the  words  with  which  there  is  difficulty  have  especial 
significance  for  the  patient.  Other  respiratory  disturbances  of  an 
asthmatic  character  may  also  be  hysterical. 

The  distribution  and  characteristics  of  these  motor  disturbances 
are  psychological,  that  is,  disturbances  develop,  not  as  they  would 
result  from  actual  organic  disease,  but  as  the  patient  thinks  they  should 
result. 

Visceral  disturbances,  especially  of  the  gastro-intestinal  tract,  many 
of  the  false  gastropathies  with  gastric  crisis  of  vomiting  and  diarrhea, 
are  quite  frequent.  There  may  also  be  hysterical  attacks  simulating 
renal  or  hepatic  colic,  gastric  ulcer,  etc. 

Vasomotor  disturbances,  localized  edemas,  disturbed  reflexes,  fever, 
secretory  and  trophic  disorders  have  all  been  described. 

The  visceral  disturbances  are  also  psychological  in  origin  but  in 
detail  often  dependent  upon  deeper  disturbances  of  integration. 
Disgust,  for  example,  gives  rise  to  vomiting  and  the  analysis  will  show, 
back  of  the  symptom  (vomiting)  the  feeling  of  disgust  attached  to 
some  sort  of  experience  in  the  individual's  past  (an  actual  experience  or 
an  experience  only  thought  of)  which  is  thought  of  as  disgusting. 

Symptoms  which  cannot  be  accounted  for  on  anatomical  and 
pathological  grounds  should  always  lead  to  an  analytic  examination 
of  the  psyche.  Even  marked  disturbances  may  have  originated 
in  the  psyche  and  continued  so  long  as  to  produce  organic  changes, 
as  for  example,  muscular  atrophy  from  prolonged  disuse  of  a  limb, 
the  paralysis  of  which  was  of  psychogenic  origin. 

Among  the  eijisodic  phonomena  are  found  disturbances  of  emotion, 
either  exaltation  or  depression,  which  can  be  understood  because  of 
the  displacement  of  the  affect.  There  are  various  types  of  delirium 
which  may  or  may  not  be  associated  with  convulsive  seizures,  produc- 
ing, especially,  when  long  drawn  out,  the  so-called  somnambulisms, 
during  which  all  sorts  of  ideas  may  be  manifested  and  the  patient 
be  quite  disoriented.  Dream  states  not  infrequently  occupy  the 
field  and  lead  by  development  to  all  sorts  and  degrees  of  double 


THE  PSYCHONEUROSES  793 

personality,  which  is  simply  a  more  elaborate  expression  of  the  split- 
ofF  complexes,  indicating  that  they  form  a  relatively  large  part  of  the 
personality.  In  fact,  these  split-off  systems,  provided  recovery  is  not 
possible,  tend  to  gather  to  themselves  more  and  more  of  the  person- 
ality and  thereby  to  lead  a  more  and  more  independent  and  broader 
existence. 

Amnesias  of  course  are  frequent  in  the  s^Tuptomatology.  Any 
portion  of  the  personality  which  is  active  may  be  amnesic  for  any 
portion  of  the  personality  which  is  opposed  to  it  in  the  conflict. 

In  the  analysis  of  hysterical  s\Taptoms  it  is  relatively  easy  to 
account  for  them  logically.  \Yhile  the  s\Tnptoms,  on  the  surface, 
often  resemble  dementia  precox,  one  is  not  so  often  brought  face  to 
face  with  the  crude  outcropping  of  the  unconscious.  It  is  more  fre- 
quently found  that  the  symptoms  lead  directly  back  to  actual  situa- 
tions, as  in  the  case  of'  Lucy  R.,  published  by  Breuer  and  Freud.  This 
patient  was  disturbed  by  a  subjective  sensation  of  smell,  which  was 
traced  back  to  a  smell  of  burning  pastry  in  a  perfectly  well-recollected 
scene  where  the  children  had  forgotten  the  pastry  and  it  had  become 
burnt.  Why  the  smell  of  burning  pastry  should  be  chosen  for  hyster- 
ical conversion  was  again  traced  to  the  young  woman's  love  for  the 
children  for  whom  she  was  governess  and  the  repressed  wish  that  she 
might  take  the  mother's  place  as  the  result  of  her  love  for  her  master, 
And  in  the  case  of  Freud's  of^  Elizabeth,  who,  while  engaged  in  nursing 
her  sick  father  spends  one  evening  away  from  home  at  the  solicitation 
of  her  family.  Upon  this  occasion  she  meets  a  young  man  and  on  her 
walk  home  with  him  gives  herself  up  to  the  happiness  of  the  situation. 
On  the  return,  howe\er,  finding  her  father  much  worse,  she  bitterly 
reproaches  herself  for  forgetting  him  in  her  own  pleasure.  This 
thought,  however,  is  repressed.  In  the  course  of  her  caretaking  she 
had  each  morning  to  change  the  dressings  on  her  father's  swollen  leg. 
To  do  this  she  took  his  leg  upon  her  right  thigh.  The  suppressed 
complex  seized  upon  the  feeling  of  weight  and  pain  of  her  father's 
leg  upon  her  thigh  as  an  efficient  avenue  of  expression  for  her  repressed 
wish  which  thus  comes  into  consciousness  under  the  disguise  of  a 
painful  area  on  the  right  thigh  corresponding  in  extent  and  location 
to  the  place  upon  which  the  father's  leg  rested. 

From  these  examples  it  will  be  seen  that  the  hysteric  is  the  victim 
of  the  spontaneous  and  aberrant  activity  of  repressed  and  split-off 
complexes  that  have  to  do  with  past  events  in  the  patient's  life  (actual 
or  psychological)  and  that  the  expression  of  these  complexes  produces 
the  symptoms  of  the  psychoneurosis,  and  that  so  far  as  the  hysterical 
manifestations  are  concerned  the  hysteric  may  bfe  said  to  live  in  the 
past,  for  each  access  of  symptoms  is  but  a  reanimation  of  past  experi- 
ences. Past  thoughts  and  feelings  must  not  be  put  down  as  of  no 
account,  they  are  just  as  much  facts  as  actual  experiences,  they  are 
psychological  facts. 

'  Freud,  S. :  Selected  Papers  on  Hysteria  and  Other  Psychoneuroses,  Nervous  and 
Mental  Disease  Monograph  Series,  No.  4.  ^  Loc.  cit. 


794         THE  PSYCHONEUROSBS  AND  ACTUAL  NEUROSES 

These  split-off  groups  of  ideas  and  feelings  which  tend  to  independent 
existence  as  secondary  states  which  are  reanimated  in  the  hysterical 
somnambulisms  are  groups  of  ideas  and  feelings  at  relatively  high 
levels.  That  is  they  hang  together  quite  as  do  ideas  and  feelings  in 
consciousness  as  we  know  it.  They  are  logically  related  and  tend  to 
reproduce  actual  experiences  or  series  of  experiences.  In  other  words 
they  constitute  minor  personalities  which  maintain  a  sort  of  parasitic 
existence  within  the  larger  personality  whole.  The  splitting  in  hysteria 
tends  to  be  massive  or  molar  in  type  rather  than  molecular  as  it  does 
not  tend  to  involve  the  partial  tendencies  to  the  extent  of  disintegrating 
the  personality  by  involving  those  constituent  elements  which  go  to 
make  up  conduct  as  we  ordinarily  know  it.  The  deeper  splits  in  the 
personality  which  involve  the  more  deep-seated  factors  and  produce 
those  grotesque  symptoms  which  seem  unpsychological  are  character- 
istic of  those  more  grave  disturbances  which  are  found  in  the  dementia 
precox  group  of  psychoses. 

Like  all  psychoneurotics  the  hysteric  is  infantile.  In  other  words, 
there  is  a  certain  defect  in  psychosexual  development,  and  the  difficul- 
ties which  they  meet  in  life  tend  to  drive  them  back  upon  themselves, 
to  cause  an  introversion  of  the  libido,  that  is,  to  remove  their  interest 
from  the  actual  world  of  reality  and  to  center  it  back  again  in  them- 
selves. As  already  explained,  this  introversion  process  tends  to  reani- 
mate progressively  lower  psychosexual  levels,  and  with  a  patient  who 
is  already  infantile  the  tendency  to  reanimate,  for  example,  the  auto- 
erotic  level  is  easily  manifest.  This  is  well  shown  in  certain  s;^Tnbolic 
masturbatory  acts  which  recur  during  the  hysterical  seizures  of  which 
probl^l^y  involuMtary^  micturition  is  one. 
f  Tl^s^^mpto^^^fc£Y-Q£JlVsteria  is  then  the  svmptomflt^^^gY  ^^  ^^^ 
activity  of  the  j^^Bff  dissociated  idea  constellations  or  complexes  and 
theirjnamrest^^^K^'OiieinecJianism  ot  conversion  thereby  producing 
V  symptomFor^BUcn  dioj 

These  split  Jwcumpltxes  tend  always  to  become  dynamic  and  mani- 
fest themselves  episodically  in  the  hysterical  seizures.  The  process  of 
dissociation  or  splitting,  once  begun,  tends  to  continue  and  new  material 
tends  constantly  to  be  added  to  these  split-off  elements  by  further 
cleavage,  and  thus  this  new  portion  of  the  personality  continues  to  grow 
at  the  expense  of  the  total  personality.  Energy  accumulates  in  these 
split-off  systems,  and  when  it  becomes  sufficient  in  amount  it  breaks 
through,  so  to  speak,  and  produces  the  attacks.  These  attacks  are 
made  up  characteristically  of  a  living  over  again  of  those  experiences 
which  constituted  the  etiological  moments  of  the  psychoneurosis.  In 
hysteria,  as  has  been  pointed  out,  the  breaking  through  of  the  energy 
from  the  split-off  complexes  manifests  itself  in  bodily  innervation — the 
s;>Tiiptoms  of  the  disease  are  physical. 

Aside  from  these  episodic  manifestations,  the  crises  or  paroxysms 
of  the  disease,  there  are  the  so-called  inter  paroxysmal  symptoms,  which, 
harking  back  to  a  middle  age  demonology  are  still  termed  stigmata. 
These   are   most   characteristically   various   anesthesias,   anesthesias 


THE  PSYCHONEUROSES  795 

which  are  rarely  complained  of  by  the  patient,  often  entirely  unknown 
to  him,  being  only  brought  out  upon  examination.  It  is  because  of 
this  latter  fact  that  Babinski  has  been  led  into  the  error  of  supposing 
that  they  were  entirely  the  result  of  the  examination,  a  position  the 
erroneousness  of  which  one  can  demonstrate  to  one's  own  satisfaction. 
Even  though  it  were  absolutely  true,  the  fundamental  fact,  the  why 
of  the  sjTnptoms,  the  reason  for  certain  patients  reacting  in  such  a 
way  to  an  examination,  remains  unexplained  by  this  renowned  French 
neurologist. 

An  analysis  of  the  stigmata  shows  also,  and  usually  without  much 
difficulty,  a  logical  connection  with  preceding  experiences,  as  for 
example,  the  smell  in  the  case  of  Lucy  R.,  or  the  anesthesia  of  the  thigh 
in  the  case  of  Elizabeth,  already  cited. 

There  is  another  group  of  s\Tnptoms  which  follow  of  necessity  as  a 
result  of  the  splitting  of  the  personality.  It  can  be  easily  seen  from 
this  dynamic  conception  of  the  nature  of  the  disease  that  a  person  who 
is  not  at  one  with  himself  has  not  at  any  one  time  the  full  quota  of  his 
energies  available,  and  therefore  it  is  found  that  the  general  efficiency 
of  this  class  of  patients,  particularly  in  the  psychic  sphere,  is  very 
greatly  reduced;  it  is  reduced  in  proportion  to  the  amount  of  the 
personality  which  is  represented  by  these  split-ofT  complexes.  These 
patients,  therefore,  are  not  equal  to  the  tasks  they  once  could  do. 
They  are  nervous  and  irritable,  they  tire  easily,  they  lack  capacity  for 
consecutive  application,  and  forget  readily.  These  are  general  sjTup- 
toms  of  the  condition,  secondary  s\Tnptoms,  the  result  of  any  splitting, 
and  which  are  added  to  the  primary  sjTnptoms  which  are  expressions 
of  the  actual  conflict. 

A  connecting  link  between  the  two  portions  of  the  personality  is 
seen  in  the  hysterical  fantasies  and  also,  of  course,  in  the  dreams 
which  themselves  belong  to  the  realm  of  fantasy  formation,  fan- 
tasies which  are  thoughts  that  come  without  being  bidden  at  moments 
of  mental  abstraction,  thoughts  that  flow  along  without  volitional 
choice,  that  replace  one  another  without  the  exercise  of  critique,  in 
other  words,  day-dreaming  or  night-dreaming  as  the  case  ma}'  be. 
These  fantasies  represent  the  activity  of  the  submerged  complexes 
as  they  break  through  and  manifest  themselves  in  the  upper  con- 
sciousness. They  are  of  great  importance  in  discovering  the  nature 
of  the  conflict  and  are  very  common  features  of  the  hysteric,  although 
usually  the  patient  does  not  realize  it  until  his  attention  is  addressed 
to  these  vagrant  mental  manifestations,  because  they  not  onl}''  come 
unbidden,  but  when  they  go  they  leave  no  tell-tale  traces  in  the  con- 
scious memory.  A  further  connection  between  the  unconscious  and 
conscious  are  the  conscious  fantasies.  These  are  fantasy  formations 
which  apparently  lie  in  clear  consciousness  and  are  not  repressed. 
Fantasies  of  this  sort  are  permitted  in  clear  consciousness  only  because 
they  are  not  understood  at  their  true  value.  They  really  represent 
chiefly  repressed  material. 

The  peculiar  characteristic  symptom  of  hysteria,  conversion,  is  of 


796         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

great  importance  to  understand.  In  paranoia  the  unconscious  affects 
which  are  striving  for  expression  are  projected  upon  outside  circum- 
stances and  persons — the  mechanism  of  projection — (See  paranoia). 
In  hysteria  the  conversion  mechanism  is  responsible  for  the  indict- 
ment of  the  bodily  organs.  Errors  of  diagnosis  are  therefore  favored 
and  the  patient  seeks,  so  to  speak,  to  delude  the  doctor  to  believe  in 
some  disease  of  an  organ  that  can  be  treated.  Such  treatment  the 
patient  hopes  will  relieve  the  symptoms  and  the  responsibility  is  trans- 
ferred in  this  way  to  the  doctor  the  patient  assuming  no  responsibility 
for  them.  The  results  may  be  tragic  as  in  cases  where  important 
organs  are  removed  on  this  hypothesis.  There  have  undoubtedly 
been  many  ovaries  removed  in  years  past  to  cure  a  psychic  conflict. 
This  cutting  out  of  the  psychosis  has,  however,  a  very  disastrous 
result  for  the  patient  who  is  thus  projected  still  further  along  the  path 
of  chronic  invalidism. 

To  resume,  hysteria  is  the  result  of  a  splitting  of  the  personality  in 
which  certain  split-off  complexes  are  psychosexually  determined;  and 
leading  an  existence  more  or  less  independent  of  the  total  personality, 
express  themselves  by  the  mechanism  of  conversion  in  bodily  innerva- 
tion. The  hysterical  sjTnptoms,  then,  become  the  representation 
through  conversion  of  the  unconscious  fantasies  which  originate  in 
the  repressed  complexes,  while  the  structure  of  the  hysterical  attack 
is  in  every  way  similar  to  that  of  a  dream.  The  attack  is  the  breaking 
through  of  the  energy  of  the  repressed  systems  and  manifests  itself 
by  a  wish-fulfilling  delirium,  the  elements  of  which  may  be  over- 
determined,  displaced,  aiid  inverted  for  purposes  of  disguise,  as  are 
the  elements  of  a  dream. 

Compulsion  Neurosis. — Compulsion  neurosis  contains  probably  the 
majority  of  that  complex  group  to  which  Janet  gave  the  name  of  psych- 
asthenia.  Janet's  group,  however,  contained  not  only  the  compul- 
sion neuroses,  but  a  number  of  other  things,  particularly  the  anxiety 
neuroses,  probably  many  anxiety  hysterias,  perhaps  some  neuras- 
thenias, hysterias  and  even  schizophrenias. 

The  characteristics  of  the  compulsion  neurosis  are  certain  com- 
pulsive tendencies  to  act  or  to  think  in  certain  ways.  The  patient 
is  forced,  apparently  agamst  his  will  and  without  reason,  to  think 
certain  ideas  or  certain  thoughts  or  to  do  certain  things.  The  com- 
pulsion increases  until  it  is  yielded  to,  then  a  period  of  calm  follows 
which  may  be  of  variable  length,  until,  so  to  speak,  the  energy  has 
again  accumulated,  when  the  compulsion  again  manifests  itself  and 
must  again  be  relieved  by  yielding.  The  patient  has  perfect  insight 
into  the  matter,  knows  the  whole  business  is  foolish,  but  he  cannot 
help  it. 

Mechanism  of  Compulsion  Neurosis. — In  hysteria  the  repressed 
material  manifests  itself  by  conversion.  The  accumulated  affect  of 
the  split-off  complexes  is  drained  off  through  bodily  innervation.  The 
physical  symptom  is  made  the  scapegoat  to  save  the  more  important 
mental  adjustment.     The  highly  affect-ladened  complexes  are  thus 


THE  PSYCHONEUROSES  797 

deprived  of  their  emotion.  The  hysterical  attack  is  a  wish-fulfilHng 
delirium  which  brings  to  pass  in  a  sort  of  allegorical  dramatization  the 
fulfilment  of  both  elements  in  the  conflict. 

In  contradistinction  to  these  characteristics  of  hysteria,  in  the 
compulsion  neurosis  there  is  no  conversion.  The  affect  of  the  repressed 
complexes  is  drained  off,  not  through  bodily  innerv^ation,  but  by  attach- 
ment to  otherwise  indifferent  ideas.  The  affect  is  displaced  to  a  sub- 
stitute. This  substitution,  quite  as  in  the  conversion  of  hysteria,  is  a 
distortion  mechanism  and  serves  equally  with  it  to  disguise  from  the 
patient  the  real  source  of  the  affect. 

Then  again,  while  in  the  hysterical  attack  both  elements  in  the 
conflict  come  to  contemporaneous  fulfilment,  such  unification  through 
the  symptoms  is  less  evident  in  the  compulsion  neurosis,  although  the 
attempt  is  made  to  bring  it  about.  What  occurs  on  the  surface,  at 
least,  is  a  constant  alternation  between  the  ascendency  of  the  two 
factors  in  the  conflict,  which  two  factors  in  their  ultimate  analysis 
resolve  themselves,  perhaps  always,  into  love  and  hate. 

The  compulsion  neurosis  is  a  true  defense  neurosis  and  its  symptoms, 
at  least  the  compulsive  acts,  which  develop  late  in  the  course  of  the 
disorder  are  of  the  nature  of  ceremonials,  which  not  only  serve  to 
disguise  the  true  situation  from  the  patient,  but  so  to  speak,  atone  for 
evil. 

Freud  has  very  well  said  that  it  is  much  more  correct  to  speak  of 
obsessive  thinking  than  of  obsessive  ideas.  It  is  the  obsessive  element, 
the  compulsion,  the  so-called  Zwang  of  the  Germans  which  is  the  essen- 
tial thing  in  this  neurosis,  and  which  may  express  itself  in  all  possible 
ways. 

The  mechanism  of  the  production  of  the  symptoms  of  the  compul- 
sion neurosis  is  a  quite  complicated  one.  It  cannot  be  expressed  better 
than  by  quoting  the  language  of  Freud. ^ 

The  extracts  from  Freud,  which  are  taken  from  his  original  formu- 
lation of  the  compulsion  neurosis  concept,  are  somewhat  involved -and 
difficult  to  understand  so  the  plan  will  be  followed  of  interspersing  them 
with  comments,  explanations,  and  illustrations  for  the  purpose  of 
simplification  and  classification. 

"Sexual  experiences  of  early  childhood  have  the  same  significance 
in  the  etiology  of  the  compulsion  neurosis  as  in  hysteria,  still  we  no 
longer  deal  here  with  sexual  passivity  but  with  pleasurably  accom- 
plished aggressions,  and  with  pleasurably  experienced  participation 
in  sexual  acts,  that  is,  we  deal  here  with  sexual  activity.  It  is  due  to 
this  difference  in  the  etiological  relations  that  the  masculine  sex  seems 
to  be  preferred  in  the  compulsion  neurosis. 

"The  compulsion  neurosis  is  developed  in  its  full-blown  form  as  a 
type  of  expiatory  ceremonial  for  acts  guiltily  participated  in,  i.  e., 
acts  in  which  the  patient  took  an  active  part  if  only  by  acquiescence 
and  for  which  he  therefore  holds  himself  responsible. 

'  Hitschmann:  Freud's  Theories  of  the  Neuroses,  Nervous  and  Mental  Disease  Mono- 
graph Series,  New  York. 


798         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

"  In  all  my  cases  of  compulsion  neurosis  I  have  found,  besides,  a  sub- 
soil of  hysterical  symptoms  which  could  be  traced  to  a  pleasurable 
action  of  sexual  passivity  from  a  precedent  scene.  I  presume  that  this 
coincidence  is  a  lawful  one  and  that  premature  sexual  aggression  always 
presupposes  an  experience  of  seduction.  But  I  am  unable  to  present 
as  yet  a  complete  description  of  the  etiology  of  the  compulsion  neurosis. 
I  only  believe  that  the  final  determination  as  to  whether  a  hysteria 
or  compulsion  neurosis  should  originate  on  the  basis  of  infantile 
traumas  depends  on  the  temporal  relation  of  the  development  of  the 
libido. 

•  "The  essence  of  the  compulsion  neurosis  may  be  expressed  in  the 
following  simple  formula:  Obsessions  are  always  transformed  reproaches 
returning  from  the  repression,  which  always  refer  to  a  pleasurably 
accomplished  sexual  action  of  childhood.  In  order  to  elucidate  this 
sentence  it  will  be  necessary  to  describe  the  typical  course  of  com- 
pulsion neurosis. 

"The  compulsive  ways  of  thinking  and  acting  are  only  substitutes 
for  the  reproaches  which  are  symbolical  distortions  in  order  to  prevent 
a  recognition  on  the  part  of  the  patient  of  the  real  meaning,  in  order 
to  keep  from  his  consciousness  a  realization  of  the  actual  circum- 
stances of  his  guilty  conduct. 

"In  a  first  period — ^period  of  childish  immorality — the  events  con- 
taining the  seeds  of  the  later  neurosis  take  place.  In  the  earliest  child- 
hood there  appear  at  first  the  experiences  of  sexual  seduction  which 
later  makes  the  repression  possible,  and  this  is  followed  by  the  actions 
of  sexual  aggressions  against  the  other  sex  which  later  manifest  them- 
selves as  actions  of  reproach." 

The  original  experience  here  referred  to  as  one  of  "sexual  seduction" 
must  be  understood  in  a  much  broader  sense  than  is  usually  given  these 
terms.  The  term  sexual,  for  example,  refers  to  the  whole  realm  of 
the  race-preservative  libido  as  previously  outlined.  The  listening  to 
"nasty"  stories  might  therefore  be  such  an  original  form  of  "sexual 
seduction"  which  easily  has  its  wrongfulness  emphasized  by  the  child's 
failing  to  tell  the  mother  all  the  things  the  boys  at  school  talked  to  him 
about,  although  warned  by  her  to  do  so  for  the  very  obvious  reason  of 
preventing  this  very  form  of  auto-erotic  indulgence.  Later  on  the  very 
acts  of  exchanging  confidences  and  having  secrets  of  a  sexual  nature 
with  other  boys  becomes  a  matter  for  future  reproaches.  This  is, 
of  course,  but  one  of  innumerable  types  of  experience  which  may  bring 
about  similar  mechanisms  of  defense. 

"  This  period  is  brought  to  an  end  by  the  appearance  of  the — often 
self-ripened — sexual  'maturity.'  A  reproach  then  attaches  itself 
to  the  memory  of  that  pleasurable  action,  and  the  connection  with 
the  initial  experience  of  passivity  makes  it  possible — often  only  after 
conscious  and  recollected  effort — to  repress  it  and  replace  it  by  a 
primary  symptom  of  defense.  The  third  period,  that  of  apparent 
healthiness  but  really  of  successful  defense,  begins  with  the  symptoms 
pf  scrupulousness;  shame  and  diffidence. 


THE  PSYCHONEUROSES  799 

"The  next  period,  the  disease  is  characterized  by  the  return  of  the 
repressed  reminiscences,  hence  by  the  failure  of  the  defense;  but  it 
remains  undecided  whether  the  awakening  of  the  same  is  more  fre- 
quently accidental  and  spontaneous,  or  whether  it  appears  in  conse- 
quence of  actual  sexual  disturbances,  that  is,  as  additional  influences 
of  the  same.  But  the  revived  reminiscences  and  the  reproaches  formed 
from  them  never  enter  into  consciousness  unchanged,  but  what  become 
conscious  as  an  obsession  and  obsessive  affect  and  displace  the 
pathogenic  memory  in  the  conscious  life  are  compromise  formations 
between  the  repressed  and  the  repressing  ideas." 

An  example  will  make  this  clear.  A  young  man,  when  a  very  young 
boy,  w^as  subjected  to  a  homosexual  assault.  The  affair  occured  in  a 
portion  of  a  room  the  floor  of  which  was  covered  with  a  white  bear- 
skin rug.  Following  this  episode  shame  and  diffidence  became  marked 
character  traits.  Later  on  he  indulged  in  the  habit  of  masturbating 
and  for  these  occasions  w^ould  select  a  dark  room.  \Miile  engaged  in 
tljis  forbidden  practice  he  w^ould  fear  discovery  and  imagine  he  could 
see  the  bright  eyes  of  his  accusers  looking  at  him.  The  whites  of  the 
eyes  were  their  worst  feature.  Here  we  find  the  later  return  of  the 
repressed  reminiscences  sjTnbolically  expressed  by  the  fear  of  white 
in  the  fancied  eyes  of  the  discoverer  of  his  habit.  Still  later  this  fear 
became  generalized  into  a  dislike  and  fear  of  white  objects  in  general, 
especially  the  whites  of  eggs.  This  phobia  is  therefore  not  only  a 
SNTnbolic  expression  of  the  repressed  reminiscences  but  a  defense 
against  the  pain  of  their  recognition.  It  is  also  a  mechanism  for 
turning  the  individual  from  his  auto-erotic  practices  upon  the  healthy 
path  of  psychosexual  development. 

"In  order  to  describe  clearly  and  probably  convincingly  the  proc- 
esses of  repression,  the  return  of  the  repression,  and  the  formation  of 
the  pathological  ideas  of  compromise,  we  would  have  to  decide  upon 
very  definite  hj-potheses  concerning  the  substratum  of  the  psychic 
occurrence  and  consciousness.  As  long  as  we  wish  to  avoid  it  we  will 
have  to  rest  content  with  the  following  rather  figuratively  under- 
stood observations.  Depending  on  whether  the  memory  content  of 
the  reproachful  action  alone  forces  an  entrance  into  consciousness  or 
whether  it  takes  with  it  the  accompanying  reproachful  affect,  we  have 
two  forms  of  compulsion  neurosis.  The  first  represents  the  t\'pical 
obsessions,  the  content  of  w^hich  attracts  the  patient's  attention; 
only  an  indefinite  displeasure  is  perceived  as  an  affect,  whereas  for 
the  content  of  the  obsession  the  only  suitable  affect  would  be  one  of 
reproach.  The  content  of  the  obsession  is  doubly  distorted  w^hen 
compared  to  the  content  of  the  infantile  compulsive  act.  First,  some- 
thing actual  replaces  the  past  experience,  and  second,  the  sexual  is 
superseded  by  an  analogous  non-sexual  experience.  These  two 
changes  are  the  results  of  the  constant  tendency  to  the  repression 
still  in  force  which  we  will  attribute  to  the  'ego.'  The  influence 
of  the  revived  pathogenic  memory  is  showii  by  the  fact  that  the 
content  of  the  obsession  is  still  partially  identical  with  the  repressed 


800  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

or  can  be  traced  to  it  by  a  correct  stream  of  thought.  If,  with  the 
help  of  the  psychoanalytic  method,  we  reconstruct  the  origin  of  one 
individual  obsession  we  find  that  one  actual  impression  instigated 
two  diverse  streams  of  thought,  and  that  the  one  which  passed  over 
the  repressed  memory,  though  incapable  of  consciousness  and  cor- 
rection, proves  to  be  just  as  correctly  formed  logically  as  the  other. 
If  the  results  of  the  two  psychic  operations  disagree,  the  contradiction 
between  the  two  may  never  be  brought  to  logical  adjustment,  but  as 
a  compromise  between  the  resistance  and  the  pathological  result 
of  thought  an  apparently  absurd  obsession  enters  into  consciousness 
beside  the  normal  result  of  the  thought.  If  both  streams  of  thought 
yield  the  same  result,  they  reinforce  each  other  so  that  the  normally 
gained  result  of  thought  now  behaves  psychically  like  an  obsession. 
Wherever  neurotic  compulsion  manifests  itself  psychically  it  originates 
from  repression.  The  obsessions  have,  as  it  were,  a  psychical  course 
of  compulsion  which  is  due,  not  to  their  own  validity,  but  to  the  source 
from  which  they  originate,  or  to  the  source  which  furnishes  a  part  of 
their  validity. 

"A  second  form  of  compulsion  neurosis  results  if  the  repressed 
reproach  and  not  the  repressed  content  of  memory  forces  a  replace- 
ment in  the  conscious  psychic  life.  Through  a  psychic  admixture,  the 
affect  of  the  reproach  can  change  itself  into  any  other  affect  of  dis- 
pleasure, and  if  this  occurs  there  is  nothing  to  hinder  the  substituting 
affect  from  becoming  conscious.  Thus  the  reproach  (of  having  per- 
formed in  childhood  some  sexual  actions)  may  be  easily  transformed 
into  shame  (if  someone  else  becomes  aware  of  it),  into  hypochondriacal 
anxiety  (because  of  the  physically  harmful  consequences  of  those 
reproachful  acts) ,  into  social  anxiety  (fearing  punishment  from  others) , 
into  religious  anxiety,  into  delusions  of  observation  (fear  of  betraying 
those  actions  to  others),  into  fear  of  temptations  (justified  distrust 
in  one's  oami  moral  ability  of  resistance),  etc.  Besides,  the  memory 
content  of  the  reproachful  action  may  also  be  represented  in  conscious- 
ness, or  it  may  be  altogether  concealed,  which  makes  the  diagnosis 
very  difficult.  Many  cases  which  on  superficial  examination  are  taken 
as  ordinary  (neurasthenic)  hypochondria  often  belong  to  this  group 
of  compulsive  affects;  the  very  frequently  so-called  'periodic  neuras- 
thenia' or  'periodic  melancholia'  especially  seem  to  be  explained  by 
compulsive  affects  or  obsessions,  a  recognition  not  unimportant 
therapeutically. 

"Besides  these  compromise  symptoms  which  signify  the  return  of 
the  repression  and  hence  a  failure  of  the  originally  achieved  defense, 
the  compulsion  neurosis  forms  a  series  of  other  symptoms  of  a  totally 
different  origin.  The  ego  really  tries  to  defend  itself  against  those 
descendants  of  the  initial  repressed  reminiscence,  and  in  this  conflict 
of  defense  it  produces  symptoms  which  may  be  comprehended  as 
'secondary  defense.'  These  are  throughout  'protective  measures' 
which  have  performed  good  service  in  the  struggle  carried  on  against 
the  obsessions  and  the  obsessing  affects.    If  these  helps  in  the  conflict 


THE  PSYCHONEUROSES  801 

of  the  defense  really  succeed  in  repressing  anew  the  symptoms  of 
return  obtruding  themselves  on  the  ego,  the  compulsion  then  trans- 
mits itself  on  the  protective  measures  themselves  and  produces  a 
third  form  of  the  'compulsion  neurosis,'  the  compulsive  action.  These 
are  never  primary,  they  never  contain  anything  else  but  a  defense, 
never  an  aggression.  Psychic  analysis  shows  that  despite  their  pecu- 
liarity they  can  always  be  fully  explained  by  reduction  to  the  com- 
pulsive reminiscence  which  they  oppose. 

"One  example  instead  of  many:  An  eleven-year-old  boy  has 
obsessively  arranged  for  himself  the  following  ceremonial  before  going 
to  bed:  He  could  not  fall  asleep  unless  he  related  to  his  mother 
most  minutely  all  experiences  of  the  day;  not  the  smallest  scrap  of 
paper  or  any  other  rubbish  was  allowed  in  the  evening  on  the  carpet 
of  his  bedroom.  The  bed  had  to  be  moved  close  to  the  wall,  three  chairs 
had  to  stand  in  front  of  it,  and  the  pillows  had  to  lie  in  just  such  a  par- 
ticular position.  In  order  to  fall  asleep  he  had  to  kick  with  both  legs  a 
number  of  times,  and  then  had  to  lie  on  the  side.  This  was  explained  as 
follows:  Years  before,  while  putting  this  pretty  boy  to  sleep,  the 
servant  girl  made  use  of  this  opportunity  to  lie  over  him  and  assault 
him  sexually,  ^^^len  this  reminiscence  was  later  awakened  by  a  recent 
experience  it  made  itself  known  to  consciousness  by  the  compulsion 
in  the  above-mentioned  ceremonial  which  sense  could  really  be  sur- 
mised and  the  details  verified  by  psychoanalysis.  The  chairs  before 
the  bed  which  was  close  to  the  wall — so  that  no  one  could  have  access 
to  it;  the  arrangement  of  the  pillows  in  a  definite  manner — so  that 
they  should  be  differently  arranged  than  they  were  on  that  evening; 
the  motion  with  the  legs — to  kick  away  the  person  lying  on  him; 
sleeping  on  the  side — because  during  that  scene  he  lay  on  his  back; 
the  detailed  confession  to  his  mother — because  in  consequence  of  the 
prohibition  of  his  seductress  he  concealed  from  his  mother  this  and 
other  sexual  experiences;  finally,  keeping  the  floor  of  his  bedroom 
clean — because  this  was  the  main  reproach  which  he  had  to  hear 
from  his  mother  up  to  that  time. 

"The  secondary  defense  of  the  obsessions  can  be  brought  about  by 
a  forcible  deviation  to  other  thoughts  of  possibly  contrary  content; 
hence  in  case  of  success  there  is  a  compulsive  reasoning  regularly, 
concerning  abstract  and  transcendental  subjects,  because  the  repressed 
ideas  always  occupied  themselves  with  the  sensuous.  Or  the  patient 
tries  to  become  master  of  every  compulsive  idea  through  logical  labor 
and  by  appealing  to  his  conscious  memory;  this  leads  to  compulsive 
thinking  and  examination  to  doubting  mania.  The  priority  of  the 
perception  before  the  memory  in  these  examinations  at  first  induce 
and  then  force  the  patient  to  collect  and  preserve  all  objects  with 
which  he  comes  in  contact.  The  secondary  defense  against  the  com- 
pulsive afiects  results  in  a  greater  number  of  defensive  measures  which 
are  capable  of  being  transformed  into  compulsive  actions.  These  can 
be  grouped  according  to  their  tendency.  We  may  have  measures  of 
penitence  (irksome  ceremonial  and  observation  of  numbers),  of  pre- 
51 


802         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

vention  (diverse  phobias,  superstition,  pedantry,  aggravation  of  the 
primary  symptom  of  scrupulousness),  measures  of  fear  of  betrayal 
(collecting  papers  and  shyness),  and  measures  of  becoming  uncon- 
scious (dipsomania).  Among  these  compulsive  acts  and  impulses 
the  phobias  play  the  greatest  part  as  limitations  of  the  patient's 
existence." 

That  a  line  of  secondary  defenses,  so  to  speak,  becomes  necessary 
means  that  the  original,  the  primary  defenses,  were  not  sufficient. 
These  primary  defenses  were  broken  down  and  a  more  vigorous  effort 
has  had  to  be  made  by  the  psyche  to  protect  itself.  The  psyche,  in 
other  words,  adopts  mechanisms,  which,  so  far  as  possible,  keep  the 
libido  from  resorting  to  infantile  ways  of  pleasure  seeking  and  force  it 
along  the  path  of  social  usefulness. 

The  phobias  keep  spreading  out  as  in  the  case  cited  in  which  the 
fear  of  white  originally  sumbolically  attached  to  the  whites  of  the  eyes 
became  a  fear  of  all  things  white,  and  tend  to  more  and  more  limit 
the  patient's  activities  by  closing  an  ever-increasing  number  of  paths 
of  expression. 

The  ceremonials  in  the  form  of  peculiar  successions  of  movements, 
as  in  the  case  of  the  boy  cited  above  by  Freud,  the  various  tics,  the 
saying  over  of  formulae,  etc.,  are  very  numerous  and  infinitely  varied. 
Among  these  the  various  cleansing  ceremonials  are  common.  One 
patient,  because  her  thoughts  were  unclean  was  in  constant  fear  that 
she  would  offend  God  by  allowing  some  particle  of  secretion  from  her 
body  to  come  between  her  and  Him.  Tears  for  instance  might  have 
been  due  to  unclean  thoughts  and  so  the  greatest  care  in  washing  had 
to  be  exercised  before  she  spoke  God's  name  or  prayed.  This  necessity 
extended  to  all  the  secretions,  and  so  a  great  deal  of  time  was  occupied 
in  most  detailed  and  painstaking  processes  of  washing  and  avoiding 
all  forms  of  pollution. 

"There  are  cases  in  which  we  can  observe  how  the  compulsion 
becomes  transferred  from  the  idea  or  affect  to  the  measure,  and 
other  cases  in  which  the  compulsion  oscillates  between  the  returning 
symptoms  of  secondary  defense.  But  there  are  also  cases  in  which 
no  obsessions  are  really  formed,  but  the  repressed  reminiscence  imme- 
diately becomes  replaced  by  the  apparent  primary  defensive  measure. 
Here  that  stage  is  attained  at  a  bound  which  otherwise  ends  the 
course  of  the  compulsion  neurosis  only  after  the  conflict  of  the  defense. 
Grave  cases  of  this  affection  end  either  with  a  fixation  of  ceremonial 
actions,  general  doubting  mania,  or  in  an  existence  of  eccentricity 
conditioned  by  phobias. 

"That  the  obsessions  and  everything  derived  from  them  are  not 
believed  is  probably  due  to  the  fact  that  the  defense  symptom  of 
scrupulousness  was  formed  during  the  first  repression  and  gained 
compulsive  validity.  The  certainty  of  having  lived  morally  through- 
out the  whole  period  of  the  successful  defense  makes  it  impossible 
to  give  credence  to  the  reproach  which  the  obsession  really  involves. 
Only  transitorily  during  the  appearance  of  a  new  obsession,  and  now 


THE  PSYCHONEUROSES  803 

and  then  in  melancholic  exhaustive  states  of  the  ego  do  the  morbid 
s>Tnptoms  of  the  return  also  enforce  the  belief.  The  'compulsion'  of 
the  psychic  formations  here  described  has  in  general  nothing  to  do 
with  the  recognition  through  belief,  and  is  not  to  be  mistaken  for  that 
moment  which  is  designated  as  'strength'  or  'intensity'  of  an  idea. 
Its  main  characteristic  lies  in  its  inexplicableness  through  psychic 
activities  of  conscious  ability,  and  this  character  undergoes  no  change 
whether  the  idea  to  which  the  compulsion  is  attached  is  stronger  or 
weaker,  more  or  less  intensively '  elucidated,' '  supplied  with  energy,'  etc. 

"The  reason  for  the  unassailableness  of  the  obsession  or  its  deriva- 
tive is  due  only  to  its  connection  with  the  repressed  memory  of  early 
childhood,  for  as  soon  as  we  succeed  in  making  it  conscious,  for  which 
the  psychotherapeutic  methods  already  seem  quite  sufficient,  the  com- 
pulsion, too,  becomes  detached." 

The  mechanism  of  the  compulsion  neurosis  is  therefore  seen  to  be 
an  extremely  complicated  one  and  one  which  produces  a  great  variety 
of  symptoms,  with  all  possible  ramifications  of  meaning. 

This  mechanism,  as  set  forth  in  this  rather  intricate  statement  by 
Freud,  may  be  more  simply  stated  by  saying  that,  in  distinction 
from  hysteria,  in  which  the  disguise  is  brought  about  bv  a  transfer 
of  the  repressed  material  into  symbols_ofbodily  ailment — conversion— 
m  the  compulsion  neurosis  the  disfiguremfiiit^  is_kept  ^sdiollx  within 
fecbgnized  psychological  territory.  '~The  distortion  is  produced__b^ 
displacement  of  the  affect  uponindifferent  ideas  (substitutioii)_and 
the  development  oTaTpuriScatory  ceremonial.'  F^ear  of  animals  (snakes, 
mice,  etc.),  may  be  the  accepted  conscious  equivalent  of  fear  of  sex- 
uality with  a  type  of  ceremonial,  and  is  well  illustrated  in  the  case  of 
the  eleven-year-old  boy  cited. 

The  extracts  thus  far  cited  from  Freud  were  from  his  entire  paper 
(1894-5-6).  A  more  recent  paper  (1909)^  carries  the  subject  somewhat 
further.  In  this  paper  he  discusses  further  the  distortion  by  which  the 
affect  is  displaced  from  the  matter  of  real  moment  to  a  substitute  of 
little  importance  and  how  through  this  mechanism  the  ceremonials 
finally  grow  up. 

As  a  result  of  his  further  studies  Freud  believes  that  the  fundamental 
elements  that  are  opposed  to  one  another  in  the  compulsion  neurosis 
are  love  and  hate.  This  fundamental  conflict  arises  very  easily  and  is 
conditioned  by  the  necessity  on  the  part  of  the  child.  Thus  the  child's 
love  is  frequently  replaced  by  hate  for  the  parent  who  interferes  with 
him  and  prohibits  him  from  exercising  some  pleasurable  activity.  The 
constant  alternations  between  love  and  hate  produce  the  indecision 
which  these  patients  so  constantly  show.  They  are  in  constant  doubt 
as  to  the  course  of  action  they  should  pursue,  their  real  doubt  being 
their  doubt  of  their  power  to  love.  Love  and  hate  exist  side  by  side, 
love  never  having  fully  succeeded  in  dominating  but  only  in  repressing 
the  hate  into  the  unconscious.    This  same  doubt  makes  matters  lead 

'  Abstracted  in  the  Psychoanalytic  Review,  January,  1916,  iii,  No.  1. 


804         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

to  uncertainty  in  the  carrying  out  of  the  various  protective  and  defen- 
sive measures  and  necessitates  the  endless  repetition,  a  typical  char- 
acteristic of  the  ceremonials. 

The  compulsion  neurotics  are_i_aa-aJiil£,_superior  persons  who  are  striv- 
in£Witlvtremendous  energvto  attain  to  higher  things.  The  mechanisms 
they  use  areTised  more  or  less  by  all,  but  for  reasons  which  are  as  yet  not 
fully  worked  out,  lead,  in  some  instances,  to  this  peculiar  form  of  ill- 
ness, Freud  suggests,  provisionally,  that  there  is  a  connection  between 
the  unconscious  hate  and  the  sadistic  component  of  the  sexual  instinct 
which  was  exceptionally  developed  and  was  prematurely  and  too 
profoundly  repressed.  Very  briefly  then,  what  has  happened  to  these 
patients  is  that  some  infantile  way  of  gaining  pleasure  has  received  too 
much  emphasis  in  the  course  of  their  psycho-sexual  development  so 
that,  in  this  region  they  remain  fixed  to  those  ways  of  gaining  pleasure 
which  have  proved  satisfactory  and  are  thus  unable  to  proceed  upon 
the  path  of  development  and  leave  them  behind.  The  conscious  ideals 
of  the  patient  are  such  that  actions  conditioned  by  such  desires  cannot 
even  be  admitted  to  consciousness  and  so  the  neurosis  arises  as  the 
expression  of  this  conflict  and,  too,  an  expression  of  the  essentially 
moral  qualities  of  the  patient,  that  is,  those  qualities  which  insist  upon 
continuing  the  fight  for  further  advance  on  the  road  of  development. 
Everyone  passes  the  same  road  in  development.  It  is  when  some 
phase  which  should  be  brief  is  overemphasized  and  prolonged  that  the 
basis  of  such  a  conflict  is  laid. 

Symptoms. — The  symptoms  of  the  compulsion  neurosis  are  very 
varied.  Thev  have  to  do  with  all  types  of  obsessional  thinking  and 
acting^,  that  is,  thinking  and  actmg  which  takes  place  aside  from  the 
volitjQnLpf  the  patient,  which  he  cannot  prevent "bTrTwhich  he  must 
yi^ld  to,  fls_already  described.  This  is  the  compulsion  element  which 
gives  the  name  to  the  neurosis. 

The  s^'inptoms  have  been  variously  divided  and  may  be  described 
under  the  form  of  m^or  symptoms,  obsessive  acts  of  various  sorts: 
tics,  spasmodic  torticollis,  even  epileptic  attacks;  sensory  symptoms, 
obsessive^  sensations,  amounting  at  times  to  well-marked  hallucina- 
tions ;  affective  symptoms,  obsessive  emotions,  more  particularly  those 
of  doubt  and  fear;  and  ideational  symptoms,  obsessive  ideas,  such 
as  continual  questioning. 

The  commonest  and  best  known  of  the  obsessions  are  thq^^^o6ia£' 
or  fears  which  usually  refer  to  some  very  specific  object  orset  of 
conditions  which  acquire  their  quality  of  fear  as  the  result  of  taking 
over  an  affect  by  displacement  which  is  of  deep  though  unconscious 
significance  to  the  patient.  Thus  there  are  misophobia  (fear  of  dirt 
or  contamination),  metallophobia  (fear  of  metal,  door-knobs,  money, 
etc.),  agoraphobia  (fear  of  wide  or  open  spaces),  claustrophobia  (fear  of 
narrow  or  closed  spaces),  pyrophobia  (fear  of  fire)  and  so  on  indefinitely. 

The  obsessions  of  doubt— folie  de  doute — are  common  and  result 
in  a  state  of  mind  in  which  the  patient  is  torn  between  two  courses  of 
conduct  and  cannot  choose,  or  having  done  something,  such  as  turn 


THE  PSYCHONEUROSES  805 

out  the  gas  before  going  to  bed,  is  seized  with  doubt  as  to  whether 
he  really  did  do  it  or  not  and  must  get  up  and  satisfy  himself.  Then 
doubts  when  they  refer  to  religious  or  philosophical  matters  lead  to 
continuing  questionings  and  elaborate  processes  of  reasoning  from 
which  the  patient  cannot  free  his  mind. 

Quite  allied  to  the  phobias  and  doubts  are  certain  moral  obsessions 
such  as  overconscientiousness  and  exaggerated  scrupuj 

Of  the  various  obsessional  arf\\'\f\p^  fhp^jr^-ca]]p;A(^i^  are  best 
known!  'ihus  there  are  kUpiomama  (a  compulsion  to  steal),  yyro- 
viania  (a  compulsion  to  set  something  on  fire),  dipsomania  (a  com- 
pulsion to  drink),  etc. 

In  addition  there  are  all  sorts  of  less  easily  classified  and  more 
complex  forms  of  obsessional  ways  of  thinking,  feeling  and  acting. 
There  are  complicated  ceremonials,  such  as  that  of  the  eleven-year- 
old  boy  already  cited,  ways  of  arranging  things  that  must  be  carried 
out.  Strange,  and  to  the  patient  inexplicable  attractions  and  repul- 
sions, dislikes  for  certain  kinds  of  food,  or  persons  with  a  particular 
color  of  hair,  all  manner  of  tics,  habits,  mannerisms,  ceremonials, 
the  necessity  for  touching  things — delire  de  toucher — fixed  ideas, 
hypochondrias,  etc. 

Such  psychological  phenomena  must,  of  course,  have  a  reason  for 
their  existence,  andas  the  reason  is  not  apparent  it  cannot  be  explained 
by  the  patient^  it  must  be  sought  by  psychoanalysis  m  the  unconscious. 

These  obsessions  produce  a  tremendous  amount  of  mental  unrest 
and  suffering  if  they  are  not  yielded  to,  and  a  sense  of  relief  is  expe- 
rienced when  they  are  yielded  to,  oftentimes,  however,  with  a  following 
sense  of  remorse  for  having  yielded. 

The  compulsive  ideas  and  acts  represent  compromise  formations 
which  permit  the  patient  to  obtain  satisfactions  in  infantile  ways,  i.  e., 
to  revert  to  old  ways  of  gaining  pleasure  which  were  active  and  impor- 
tant in  infancy  when  the  erogenous  zones  were  as  yet  not  clearly 
differentiated.  Here  are  found  the  explanation  for  urinary  and  fecal 
fantasies,  for  certain  cutaneous,  anal,  and  gastro-intestinal  satis- 
factions which  are  used  as  ways  of  getting  pleasure  when  driven  away 
from  reality.  They  become  infantile  ways  of  reacting  to  reality 
situations  and  so  are  inefficient,  sick  ways. 

Anxiety  Hysteria. — Anxiety  hysteria,  as  the  name  indicates,  occupies 
a  midposition  between  conversion  hysteria  on  the  one  hand  and 
anxiety  neurosis  on  the  other.  There  is,  so  to  speak,  a  combination 
of  the  two  conditions,  although  this  is  not  quite  the  situation.  In 
conversion  hysteria  the  aft'ect  of  the  repressed  complexes  is  drafted 
into  bodily  innervation  and  produces  the  physical  symptoms  of  the 
disease.  In  anxipt"^'  li^tpri?^  fb^  affprt  rpmains  in  thp  mpntfll  ^phprp, 
producing  there  various  phobias.  In  anxiety  neurosis,  as  will  be 
seen  later,  the  anxiety  has  its  origin  not  at  the  psychical,  but  at  the 
physiological  level  and  is  a  representation  in  the  psychic  sphere  of  a 
disturbance  in  the  somatic.  In  anxiety  hysteria,  the  anxiety  is  also 
produced  at  the  physiological  level,  but  it  is  a  secondary  symptom 


806  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

and  is  the  result  of  the  physiological  accompaniments  of  the  emotions 
which  go  with  the  phobias,  such  as  difficulty  of  breathing,  cardiac 
palpitation,  etc.  The  anxiety  or  the  phobia  at  the  conscious  level 
represents  a  desire,  a  wish  in  the  unconscious.  A  fear  of  having  acquired 
syphilis,  for  example,  is  quite  characteristically  motived  by  a  wish  fo£ 
j)otencv The  old  maid's  fear  of  a  man  under  the  bed  is  almost  obvi- 
ously conditioned  by  a  wish  that  there  might  be,  while  the  fear  of 
being  assaulted  and  robbed  is  really  a  fear  (desire)  for  sexual  assault. 
Such  a  fear  in  a  man,  for  instance,  is  quite  typical  of  hemosexual 
cravings. 

Anxiety  hysteria  is  one  of  the  most  widely  distributed  diseases. 
It  is  particularly  the  disease  which  manifests  itself  in  childhood  and 
from  which  most  of  the  so-called  nervous  children  suffer.  It  is  much 
more  easy  of  approach  therapeutically  than  the  compulsion  neurosis, 
its  accessibility  being  comparable  to  that  of  hysteria,  and  so  offers 
greater  opportunities  for  treatment.  Probably  many  cases  of  this 
disorder  are  included  under  Janet's  psychasthenia. 

"Shell  Shock." — "Shell  shock"  is  a  popular  term  which  has  been 
applied  to  a  multitude  of  conditions  during  the  great  war.  While  it  is 
true  that  the  explosion  of  large  caliber  shells  may  produce  actual 
physical  injury  by  Setting  masses  of  air  and  gas  in  violent  motion  or 
by  creating  a  vacuum  and  so  produce  symptoms  of  concussion  or  of 
the  nature  of  caisson  disease,  still  the  vast  majority  of  the  cases  included 
in  this  group  belong  to  the  functional  neuroses  and  psychoneuroses 
with,  of  course,  a  certain  admixture  of  borderland  conditions  and  mild 
psychoses.  The  largest  number  by  far  of  the  true  functional  "shell 
shock"  cases  are  cases  of  true  conversion  hysteria  or  anxiety  hysteria. 
I'he  mechanisms  of  "shell  shock"  are  therefore  mechanisms  witEwEich 
we  are  already  familiar.  The  term  is  an  exceedingly  unfortunate  one, 
as  it  assists  in  deflecting  the  vision  of  both  the  patient  and  the  public 
from  the  true  state  of  affairs  and  thus  of  necessity  makes  it  difficult  to 
apply  an  effective  therapy.  "Shell  shock"  is  really  a  term  for  both 
patient  and  relatives  to  hide  behind. 


THE  ACTUAL  NEUROSES  (ANXIETY  NEUROSIS  AND 
NEURASTHENIA). 

Anxiety  Neurosis. — ^The  anxiety  neurosis  was  separated  from  the 
general  group  of  actual  neuroses  and  psychoneuroses  by  Freud.  The 
name  anxiety  neurosis  indicates  that  the  symptoms  all  group  them- 
selves about  the  cardinal  symptom  of  anxiety,  and  it  is  significant 
of  this  anxiety  that,  while  it  is  a  psychic  fact,  it  is  still  not  of  psychic 
but  of  somatic  origin.  The  anxiety,  therefore,  is  not  susceptible 
of  being  analyzed  into  psychic  components,  but  its  source  can  only 
be  found  at  the  physiological  level.  This  anxiety  arising  at  the  physio- 
logical level  and  manifesting  itself  in  the  psychic  sphere  then  becomes 
a  "free-floating  anxiety"  which  may  attach  itself  to  any  idea  and 


THE  ACTUAL  NEUROSES  807 

therefore  appear  to  be  of  psychic  origm.    On  the  other  hand,  it  may 
express  itself  simply  as  anxiety  without  ideational  content. 

Anxiety  may  thus  be  seen  to  be  the  correlative  of  fear.  While  fear 
is  the  emotion  which  correponds  to  a  danger  threatening  the  organism 
from  outside,  anxiety  corresponds  to  a  danger  which  threatens  the 
organism  from  within. 

It  will  help  to  make  understandable  what  has  to  be  said  about  the 
anxiety  neurosis  if  it  is  understood  at  the  start  that  the  whole  sex 
relationship  which  is  consummated  by  the  sexual  act  consists  of  two 
parts,  a  somatic  and  a  psychic.  Iij_contrast  to  neurasthenia,  which 
results  when  the  discharge  of  energy  is  inadequate  upon  the  somatic" 
side,  anxiet^^^'neurosis  occurs  whenever  the  discharge  is  inadequate 
m  the  psychic  sphere. 

Sjnnptoms. — ^The  following  is  the  description  of  the  sj^mptomatology 
of  anxiety  neurosis  as  given  by  Freud  :^ 

"1.  General  Irritahility. — ^This  is  a  frequent  nervous  symptom,  • 
common  as  such  to  many  nervous  states.  It  is  mentioned  here 
because  it  constantly  occurs  in  the  anxiety  neurosis  and  is  of  theo- 
retical significance.  Increased  irritability  always  points  to  an  accu- 
mulation of  excitement  or  to  an  inability  to  bear  accumulation,  hence 
to  an  absolute  or  relative  accumulation  of  excitement.  The  expres- 
sion of  this  increased  irritability  through  an  auditon.^  hj-peresthesia  is 
especially  worth  mentionuig;  it  is  an  oversensitiveness  for  noises, 
which  sjinptom  is  certainly  to  be  explamed  by  the  congenital  inti- 
mate relationship  between  auditors^  impressions  and  fright.  Auditory 
hyperesthesia  is  frequently  found  as  a  cause  of  insomnia,  of  which 
more  than  one  form  belongs  to  anxiety  neurosis. 

"2.  Anxious  Expectation. — I  cannot  better  explam  the  condition 
that  I  have  in  mind  than  by  this  name  and  by  some  appended  examples. 
A  woman,  for  example,  who  suffers  from  anxious  expectation  thinks 
of  influenza-pneumonia  whenever  her  husband,  who  is  afflicted  with  a 
catarrhal  condition,  has  a  coughing  spell;  and  in  her  mind  she  sees  a 
passing  funeral  procession.  If  on  her  way  home  she  sees  two  persons 
standing  together  in  front  of  her  house  she  cannot  refrain  from  the 
thought  that  one  of  her  children  fell  out  of  the  window;  if  she  hears 
the  bell  ring  she  thinks  that  someone  is  bringing  her  mournful  tidings, 
etc.,  yet  in  none  of  these  cases  is  there  any  special  reason  for  exagger- 
ating a  mere  possibility. 

"The  anxious  expectation  naturally  reflects  itself  constantly  in 
the  normal,  and  embraces  all  that  is  designated  as  'uneasiness  and 
a  tendency  to  a  pessimistic  conception  of  things,'  but  as  often  as 
possible  it  goes  beyond  such  a  plausible  uneasiness,  and  it  is  frequently 
recognized  as  a  part  of  constraint  even  by  the  patient  himself.  Fqjl^ 
one  form  of  anxious  expectation,  nameh'.  that  whicli  refers  to  <^np's 
own  health,  we  can^eserve  the  old  name  of  hypochondriit.  Hypo- 
chondria  does  not  always  run  parallel  with  the  height  of  the  general 

1  Loc.  cit. 


808  THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

anxious  expectation;  as  a  preliminary  stipulation  it  requires  the 
existence  of  paresthesias  and  annoying  somatic  sensations.  Hypo- 
chondria is  thus  the  form  preferred  by  the  genuine  neurasthenics 
whenever  they  merge  into  the  anxiety  neurosis,  a  thing  which  fre- 
quently happens. 

"As  a  further  manifestation  of  anxious  expectation  we  may  men- 
tion the  frequent  tendency  observed  in  morally  sensitive  persons  to 
pangs  of  conscience,  scrupulosity,  and  pedantry,  which  varies,  as  it 
were,  from  the  normal  to  its  aggravation  as  doubting  mania. 

"Anxious  expectation  is  the  most  essential  symptom  of  the  neurosis; 
it  also  clearly  shows  a  part  of  its  theory.  It  can  perhaps  be  said  that 
we  have  here  a  quantum  of  freely  floating  anxiety  which  controls  the 
choice  of  ideas  by  expectation  and  is  forever  ready  to  unite  itself 
with  any  suitable  ideation. 

"3.  This  is  not  the  only  way  in  which  the  anxiousness,  usually 
latent  but  constantly  lurking  in  consciousness,  can  manifest  itself. 
On  the  contrary  it  can  also  suddenly  break  into  consciousness  with- 
out being  aroused  by  the  issue  of  an  idea,  and  thus  provoke  an  attack 
of  anxiety.  Such  an  attack  of  anxiety  consists  of  either  the  anxious 
feeling  alone  without  any  associated  idea,  or  of  the  nearest  interpreta- 
tion of  the  termination  of  life,  such  as  the  idea  of  '  sudden  death'  or 
threatening  insanity;  or  the  feeling  of  anxiety  becomes  mixed  with 
some  paresthesia  (similar  to  the  hysterical  aura) ;  or  finally  the  anxious 
feeling  may  be  combined  with  a  disturbance  of  one  or  many  somatic 
functions  such  as  respiration,  cardiac  activity,  the  vasomotor  inner- 
vation, and  the  glandular  activity.  From  this  combination  the 
patient  renders  especially  prominent  now  this  and  now  the  other 
moment.  He  complains  of  'heart  spasms,'  'heavy  breathing,'  'profuse 
perspiration,'  'inordinate  appetite,'  etc.,  and  in  his  description  the 
feeling  of  anxiety  is  put  to  the  background  or  it  is  rather  vaguely 
described  as  'feeling  badly,"  'uncomfortably,'  etc. 

"4.  What  is  interesting  and  of  diagnostic  significance  is  the  fact 
that  the  amount  of  admixture  of  these  elements  in  the  attack  of 
anxiety  varies  extraordinarily,  and  that  almost  any  accompanying 
sjTnptom  can  alone  constitute  the  attack  as  well  as  the  anxiety  itself. 
Accordingly  there  are  rudimentary  attacks  of  anxiety,  and  equivalents 
for  the  attack  of  anxiety,  probably  all  of  equal  significance  in  showing 
a  profuse  and  hitherto  little  appreciated  richness  in  forms.  A  more 
thorough  study  of  these  larvated  states  of  anxiety  (Hecker)  and  their 
diagnostic  division  from  other  attacks  ought  soon  to  become  the 
necessary  work  for  the  neuropathologist. 

"  I  now  add  a  list  of  those  forms  of  attacks  of  anxiety  with  which 
I  am  acquainted.    There  are  attacks: 

"(a)  With  disturbances  of  heart  action,  such  as  palpitation  with 
transitory  arrhythmia,  with  longer-continued  tachycardia  up  to  grave 
states  of  heart  weakness,  the  differentiation  of  which  from  organic 
heart  affection  is  not  always  easy;  among  such  we  have  the  pseudo- 
angina  pectoris,  a  delicate  diagnostic  sphere. 


THE  ACTUAL  NEUROSES  809 

"(6)  With  disturbances  of  respiration,  many  forms  of  nervous 
dyspnea,  asthma-like  attacks,  etc.  I  assert  that  even  these  attacks 
are  not  always  accompanied  by  conscious  anxiety. 

"(c)  Of  profuse  perspiration,  often  nocturnal. 

"  (rf)  Of  trembling  and  shaking  which  may  readily  be  mistaken  for 
hysterical  attacks. 

"  (e)  Of  inordinate  appetite,  often  combined  with  dizziness. 

"  (/)  Of  attack-like  appearing  diarrhea. 

"  (g)  Of  locomotor  dizziness. 

"  (h)  Of  so-called  congestions,  embracing  all  that  was  called  vaso- 
motor neurasthenia. 

"  (i)  Of  paresthesias  (these  are  seldom  without  anxiety  or  a  simliar 
discomfort) . 

"5.  Very  frequently  thq^^^^octurnal  frigh^  (pavor  nocturnus  of 
adults)  usually  combined  wTth  anxiety,  dyspnea,  perspiration,  etc., 
is  nothing  other  than  a  variety  of  the  attack  of  anxiety.  This  dis- 
turbance determines  a  second  form  of  insomnia  in  the  sphere  of  their 
anxiety  neurosis.  Moreover,  I  became  convinced  that  even  the 
pavor  noctunms  of  children  evinces  a  form  belonging  to  the  anxiety 
neurosis.  The  hysterical  tmge  and  the  connection  of  the  fear  with  the 
reproduction  of  appropriate  experience  or  dream,  makes  the  pavor 
nocturnus  of  children  appear  as  something  peculiar,  but  it  also  occurs 
alone  jwithmrLa  dream  or  a  recurring  hallucination. 

'  Vertigo.'— yThis,  in  its  lightest  forms  better  designated  as 
'dizziness,^  assumes  a  prominent  place  in  the  group  of  symptoms  of 
anxiety  neurosis.  In  its  severer  forms  the  'attack  of  vertigo,'  with 
or  without  fear,  belongs  to  the  gravest  sjTnptoms  of  the  neuroses. 
The  vertigo  of  the  anxiety  neurosis  is  neither  a  rotatory  dizziness  nor 
is  it  confined  to  certain  planes  or  lines  like  ^leniere's  vertigo.  It 
belongs  to  the  locomotor  or  coordmating  vertigo,  like  the  vertigo  in 
paralysis  of  the  ocular  muscles ;  it  consists  in  a  specific  feeling  of  dis- 
comfort which  is  accompanied  by  sensations  of  a  heaving  ground, 
sinking  legs,  of  the  impossibility  to  continue  in  an  upright  position, 
and  at  the  same  time  there  is  a  feeling  that  the  legs  are  as  heavy 
as  lead,  they  shake,  or  give  way.  This  vertigo  never  leads  to  falling. 
On  the  other  hand,  I  would  like  to  state  that  such  an  attack  of  vertigo 
may  also  be  substituted  by  a  profound  attack  of  syncope.  Other 
fainting-like  states  in  the  anxiety  neurosis  seem  to  depend  on  a  cardiac 
collapse. 

''The  vertigo  attack  is  frequently  accompanied  by  the  worst  kind 
of  anxiety  and  is  often  combined  with  cardiac  and  respiratory  dis- 
turbances. Vertigo  of  elevations,  mountains  and  precipices,  can  also 
be  frequently  observed  in  anxiety  neurosis;  moreover,  I  do  not  know 
whether  we  are  still  justified  in  recognizing  a  vertigo  of  stomachic  origin. 

"7.  On  the  basis  of  the  chronic  anxiousness  (anxious  expectation) 
on  the  one  hand,  and  the  tendency  to  vertiginous  attacks  of  anxiety 
on  the  other,  there  develop  two  groups  of  typia^Tphobms))  tiie  first 
refers  to  the  general  physiological  menaces,  whileihe  second  refers 


810         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

to  locomotion.  To  the  first  group  belong  the  fear  for  snakes,  thunder- 
storms, darkness,  vermin,  etc.,  as  well  as  the  typical  moral  over- 
scrupulousness,  and  the  forms  of  doubting  mania.  Here  the  available 
fear  is  merely  used  to  strengthen  those  aversions  which  are  instinctively 
implanted  in  every  man.  But  usually  a  compulsively  acting  phobia 
is  formed  only  after  a  reminiscence  is  added  to  an  experience  in  which 
this  fear  could  manifest  itself;  as,  for  example,  after  the  patient  has 
experienced  a  storm  in  the  open  air.  To  attempt  to  explain  such 
cases  as  mere  continuations  of  strong  impressions  is  incorrect.  What 
makes  these  experiences  significant  and  their  reminiscences  durable 
is  after  all  only  the  fear  which  could  at  that  time  appear  and  can  also 
appear  today.  In  other  words,  such  impressions  remain  forceful  only 
in  persons  with  'anxious  expectations.' 

"The  other  group  contains  agoraphobia  with  all  its  accessory 
forms,  all  of  which  are  characterized  by  their  relation  to  locomotion. 
As  a  determination  of  the  phobia  we  frequently  find  a  precedent  attack 
of  vertigo;  I  do  not  think  that  it  can  always  be  postulated.  Occa- 
sionally, after  a  first  attack  of  vertigo  without  fear,  we  see  that  though 
locomotion  is  always  accompanied  by  the  sensation  of  vertigo,  it 
remains  possible  without  any  restrictions,  but  as  soon  as  fear  attaches 
itself  to  the  attack  of  vertigo,  locomotion  fails,  under  the  conditions 
of  being  alone,  narrow  streets,  etc. 

"The  relation  of  these  phobias  to  the  phobias  of  obsessions,  which 
mechanism  I  discussed  above,^  is  as  follows:  The  agreement  lies  in 
the  fact  that  here  as  there,  an  idea  becomes  obsessive  through  its 
connection  with  an  available  affect.  The  mechanism  of  transposition 
of  the  affect  therefore  holds  true  for  both  kinds  of  phobias.  But  in 
phobias  of  the  anxiety  neurosis  this  affect  is  (1)  a  monotonous  one, 
it  is  always  one  of  anxiety;  (2)  it  does  not  originate  from  a  repressed 
idea,  and  on  psychological  analysis  it  proves  itself  not  further  reducible 
nor  can  it  be  attacked  through  psychotherapy.  The  mechanism  of 
substitution  does  not.  therefore  hold  true  for  the  phobias  of  anxiety 
neurosis. 

"Both  kinds  of  phobias  (or  obsessions)  often  occur  side  by  side, 
though  the  atypical  phobias  which  depend  on  obsessions  need  not 
necessarily  develop  on  the  basis  of  anxiety  neurosis.  A  very  frequent 
ostensibly  complicated  mechanism  appears  if  the  content  of  an  original 
simple  phobia  of  anxiety  neurosis  is  supplanted  by  another  idea,  the 
substitution  is  then  subsequently  added  to  the  phobia.  The  '  protective 
measures'  originally  employed  in  combating  the  phobia  are  most 
frequently  used  as  substitutions.  Thus,  for  example,  from  the  effort 
to  provide  one's  self  with  counter-evidence  that  one  is  not  crazy, 
contrary  to  the  assertion  of  the  hypochondriacal  phobia,  there  results 
a  reasoning  mania.  The  hesitations,  doubts,  and  the  many  repetitions 
of  the  folie  du  doute  originate  from  the  justified  doubt  concerning  the 
certainty  of  one's  own  stream  of  thoughts,  for,  through  the  compul- 

1  Die  Abwehr-Neuropsychosen,  Neurol.  Centralbl.,  1894,  Nos.  x  u.  il. 


THE  ACTUAL  NEUROSES  811 

sive-like  idea  one  is  surely  conscious  of  so  obstinate  a  disturbance,  etc. 
It  may  therefore  be  claimed  that  many  syndromes  of  compulsion 
neurosis,  like  folie  du  doiite  and  similar  ones,  can  clinically,  if  not 
notionally,  be  attributed  to  anxiety  neurosis. ^ 

"8.  The  digestive  functions  in  anxiety  neurosis  are  subject  to  very 
few__bi]^cjiai:acterlslic  dlatUfbances"  ISensations  like  nausea  ancf 
sickly  feeling  are  not  rare,'anH  the  symptom  of  inordinate  appetite 
alone  or  with  other  congestions,  may  serve  as  a  rudimentary  attack 
of  anxiety.  As  a  chronic  alteration  analogous  to  the  anxious  expecta- 
tions one  finds  a  tendency  to  diarrhea  which  has  occasioned  the 
queerest  diagnostic  mistakes.  If  I  am  not  mistaken  it  is  this  diarrhea 
to  which  Mobius^  has  recently  called  attention  in  a  small  article. 
I  believe,  moreover,  that  Peyer's^  reflex  diarrhea  which  he  attributes 
to  a  disease  of  the  prostate  is  nothing  other  than  the  diarrhea  of  anxiety 
neurosis.  The  deceptive  reflex  relation  is  due  to  the  fact  that  the  same 
factors  which  are  active  in  the  origin  of  such  prostatic  affections  also 
come  into  play  in  the  etiology  of  anxiety  neurosis. 

"The  behavior  of  the  gastro-intestinal  function  in  anxiety  neurosis 
shows  a  sharp  contrast  to  the  influence  of  this  same  function  in  neuras- 
thenia. Mixed  cases  often  show  the  familiar  'fluctuations  between 
diarrhea  and  constipation.'  The  desire  to  urinate  in  anxiety  neurosis 
is  analogous  to  the  diarrhea. 

"9.  Th^  paresthesias^which  accompany  the  attack  of  vertigo  or 
anxiety  are  interesting  because  they  associate  themselves  into  a  firm 
sequence,  similar  to  the  sensations  of  the  hysterical  aura.  But  in 
contrast  to  the  hysterical  aura  I  find  these  associated  sensations 
atypical  and  changeable.  Another  similarity  to  hysteria  is  shown  by 
the  fact  that  in  anxiety  neurosis  a  kind  of  conversion*  into  bodily 
sensations,  as,  for  example,  into  rheumatic  muscles,  takes  place  which 
otherwise  can  be  overlooked  at  one's  pleasure.  A  large  number  of 
so-called  rheumatics,  who  are  moreover  demonstrable  as  such,  really 
suffer  from  an  anxiety  neurosis.  Besides  this  aggravation  of  the  sen- 
sation of  pain  I  have  observed  in  a  number  of  cases  of  anxiety  neurosis 
a  tendency  toward  hallucinations  which  could  not  be  explained  as 
hysterical. 

"10._Manv_of  the  so-called  s^Tnptoms  Avhich_3££Qmgany_or  super- 
sede the  attack  of  anxiety  also  appear  iiTq£chronic  maimer^  They 
are  then  still  less  discernible,  for  the  anxious  feeling  accompanying 
them  appears  more  indistinct  than  in  the  attack  of  anxiety.  This 
especially  holds  true  for  the  diarrhea,  vertigo,  and  paresthesias.  Just 
as  the  attack  of  vertigo  can  be  superseded  by  an  attack  of  syncope, 
so  can  the  chronic  vertigo  be  superseded  by  the  continuous  feeling  of 
feebleness,  lassitude,  etc." 

Of  great  importance  is  the  somatic  s\'mptomatology.    The  patient 

•  Obsessions  et  phobies,  R6vue  neurologique,  1895. 

*  Neuropathologische  Beitrage,  1894,  ii,  Heft. 

'  Die  nervosen  Affektionen  des  Darmes,  Wiener  Klinik,  1893. 
<  Freud :  Abwehr-Neuropsychosen. 


812         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

characteristically  stresses  the  somatic  symptoms  and  these  cases  are 
often  treated  as  suffering  from  some  obscm-e  organic  disease.  What 
has  already  been  said  (Introduction)  about  the  relation  of  visceral 
tonicities  and  postural  tensions  to  emotional  states  will  serve  to  explain 
the  somatic  symptoms. 

The  Etiology  and  Occurrence  of  Anxiety  Neurosis. — The  following 
remarks  on  the  etiology  and  occurrences  of  anxiety  neurosis  are 
quoted  from  Freud's  original  paper  :^ 

^'In  some  cases  of  anxiety  neurosis  no  etiology  can  readilv  be  ascer- 
taiiie^  rTjsjioteworthy  that  in  such  cases  it  is  seldom  difficult  to 
demonstrate  a  marked  hereditary  tamt.  < ""'"5^.,-^;''— ~>v 

^ Where  we  have  reason  to  assume  that  the  neurosis  is^acquire^  we 
can  find  by  careful  and  laborious  examination  that  the  etiologically 
effective  moments  are  based  on  a  series  of  injuries  and  influences  from 
the  sexual  life.  These  at  first  appear  to  be  of  a  varied  -nature  but 
easily  display  the  common  character  which  explains  their  homo- 
geneous effect  on  the  nervous  system.  They  are  found  either  alone 
or  with  other  banal  injuries  to  which  a  reinforcing  effect  can  be  attrib- 
uted. This  sexual  etiology  of  anxiety  neurosis  can  be  demonstrated  so 
preponderantly  often  that  I  venture  for  the  purpose  of  this  brief  com- 
munication to  set  aside  all  cases  of  a  doubtful  or  different  etiology. 

"For  the  more  precise  description  of  the  etiological  determinations 
under  which  anxiety  neurosis  occurs,  it  will  be  advisable  to  treat 
separately  those  occurring  in  men  and  those  occurring  in  women. 
Anxiety  neurosis  appears  in  women — disregarding  their  predisposition 
- — in  the  following  cases: 

"  (a)  As  virginal  fear  or  anxiety  in  adults.  A  number  of  unequivocal 
observations  showed  me  that  an  anxiety  neurosis,  which  is  almost 
typically  combined  with  hysteria,  can  be  evoked  in  maturing  girls, 
at  the  first  encounter  with  the  sexual  problem,  that  is,  at  the  sudden 
revelation  of  the  things  hitherto  veiled,  by  either  seeing  the  sexual  act, 
or  by  hearing  or  reading  something  of  that  nature. 

'  (b)  As^ear  in  the  newhLiParried.  Young  women  who  remain 
anesthetic  during  the  first  cohabitation  not  seldom  merge  into  an 
anxiet}^  neurosis  which  disappears  after  the  anesthesia  is  displaced  by 
the  normal  sensation.  As  most  young  women  remain  undisturbed 
through  such  a  beginning  anesthesia,  the  production  of  this  fear 
requires  determinants  which  I  will  mention. 

"  (c)  As  fear  in  women  whose  husbands  suffer  from  ejaculatio  precox 
diimmsheSpotency" 
(d)  TrPFhos&!!^hose_husbands  practise  coitus  interruptus  or  reser- 
vatus.  These  cases  go  together,  for  on  analyzing  a  large  number  of 
Examples  one  can  easily  be  convinced  that  they  only  depend  on  whether 
the  woman  attained  gratification  during  coitus  or  not.  In  the  latter 
case  one  finds  the  determinant  for  the  origin  of  anxiety  neurosis. 
On  the  other  hand,  the  woman  is  spared  from  the  neurosis  if  the  hus- 

'  Loc.  cit. 


THE  ACTUAL  NEUROSES  813 

band  afflicted  by  ejaculatio  precox  can  repeat  the  congress  with  better 
results  immediately  thereafter.  The  congressu  reservatus  by  means 
of  the  condom  is  not  injurious  to  the  woman  if  she  is  quickly  excited 
and  the  husband  is  very  potent;  in  other  cases  the  noxiousness  of  this 
kind  of  preventive  measure  is  not  inferior  to  the  others.  Coitus 
interruptus  is  almost  regularly  injurious;  but  for  the  woman  it  is 
injurious  only  if  the  husband  practises  it  regardless,  that  is,  if  he 
interrupts  coitus  as  soon  as  he  comes  near  ejaculating  without  con- 
cerning himself  about  the  determination  of  the  excitement  of  his 
wife.  On  the  other  hand,  if  the  husband  waits  until  his  wife  is  gratified, 
the  coitus  has  the  same  significance  for  the  latter  as  a  normal  one; 
but  then  the  husband  becomes  afflicted  with  an  anxiety  neiu-osis. 
I  have  collected  and  analyzed  a  number  of  cases  which  furnished 
the  material  for  the  above  statements. 

"(e)  As  fear  in  widows  and  intentional  abstainers,  not  seldom  in 
tj-pical  combination  with  obsessions. 

"  (/)  As  fear  in  the  climacterium  during  the  last  marked  enhance- 
ment of  the  sexual  desire. 

"The  cases  (c),  (d),  and  (e)  contain  the  determinants  under  whicb 
the  anxiety  neurosis  originates  in  the  female  sex  most  frequently  and 
most  independently,  of  hereditary  predisposition.  I  will  endeavor  to 
demonstrate  in  these — curable,  acquired — cases  of  anxiety  neurosis 
that  the  discovered  sexual  injuries  really  represent  the  etiological 
moments  of  the  neurosis.  But  before  proceeding  I  will  mention  the 
sexual  determinants  of  anxiety  neurosis  in  men.  I  would  like  to 
formulate  the  following  groups,  everyone  of  which  finds  its  analogy  in 
women : 

"  (a)  Fear  of  the  intentional  abstainers;  this  is  frequently  combined 
with  symptoms  of  defense  (obsessions,  hysteria).  The  motives  which 
are  decisive  for  intentional  abstinence  carry  along  with  them  the  fact 
that*  a  number  of  hereditarily  burdened  eccentrics,  etc.,  belong  to  this 
category. 

' '  (b)  Fear  in  men  with  frustrated  excitement  (during  the  engagement 
period),  persons  who  out  of  fear  for  the  consequences  of  sexual  rela- 
tions satisfy  themselves  with  handling  or  looking  at  the  woman. 
This  group  of  determinants  which  can  moreover  be  transferred  to 
the  other  sex — engagement  periods,  relations  with  sexual  forbearance 
— furnish  the  purest  cases  of  neurosis. 

"  (c)  Fear  in  men  who  practise  coitus  interruptus.  As  observed 
above,  coitus  interruptus  injures  the  woman  if  it  is  practised  regard- 
less of  the  woman's  gratification;  it  becomes  injurious  to  the  man  if 
in  order  to  bring  about  the  gratification  in  the  woman  he  voluntarily 
controls  the  coitus  by  delaying  the  ejaculation.  In  this  manner  sve  can 
understand  why  it  is  that  in  couples  who  practise  coitus  interruptus 
it  is  usually  only  one  of  them  who  becomes  afflicted.  Moreover,  the 
coitus  interruptus  only  rarely  produces  in  man  a  pure  anxiety  neurosis, 
usually  it  is  a  mixture  of  the  same  with  neurasthenia. 


814         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

"  (d)  Fear  in  men  in  the  senium.  There  are  men  who  show  a  climac- 
terium Hke  women,  and  merge  into  an  anxiety  neurosis  at  the  time 
when  their  potency  diminishes  and  their  Hbido  increases. 

"Finally  I  must  add  two  more  cases  holding  true  for  both  sexes: 

"(e)  Neurasthenics  merge  into  anxiety  neurosis  in  consequence  of 
masturbation  as  soon  as  they  refrain  from  this  manner  of  sexual  grati- 
fication. These  persons  have  especially  made  themselves  unfit  to 
bear  abstinence. 

"What  is  important  for  the  understanding  of  the  anxiety  neurosis 
is  the  fact  that  any  noteworthy  development  of  the  same  occurs  only 
in  men  who  remain  potent,  and  in  non-anesthetic  women.  In  neuras- 
thenics, who  on  account  of  masturbation  have  markedly  injiu-ed  their 
potency,  anxiety  neurosis  as  a  result  of  abstinence  occurs  but  rarely 
and  limits  itself  usually  to  hypochondria  and  light  chronic  dizziness. 
The  majority  of  women  are  really  to  be  considered  as  'potent;'  a 
real  impotent,  that  is,  a  real  anesthetic  woman,  is  also  inaccessible  to 
anxiety  neurosis,  and  bears  strikingly  well  the  injuries  cited. 

"How  far  we  are  perhaps  justified  in  assuming  constant  relations 
between  individual  etiological  moments  and  individual  symptoms  from 
the  complex  of  anxiety  neurosis,  I  do  not  care  to  discuss  here. 

"  (/)  The  last  of  the  etiological  determinants  to  be  mentioned  seems, 
in  the  first  place,  really  not  to  be  of  a  sexual  nature.  Anxiety  neurosis 
originates  in  both  sexes  through  overwork,  exhaustive  exertion,  as, 
for  instance,  after  sleepless  nights,  nursing  the  sick,  and  even  after 
serious  illnesses." 

Neurasthenia. — The  term  neurasthenia,  since  it  first  came  into  use 
only  a  little  over  a  generation  ago,  has  been  applied  to  almost  every 
conceivable  condition.  Almost  all  illnesses  are  accompanied  by  a 
certain  amount  of  easy  fatigability,  emotional  instability,  and  a  general 
out-of-sorts  feeling.  All  combinations  of  this  kind  which  cannot  be 
specifically  diagnosed  and  placed  under  some  well-known  caption  are 
easily  dropped  into  the  miscellaneous  group  of  neurasthenia.  Not 
only  have  all  sorts  of  conditions,  therefore,  been  included  under  this 
term,  but  the  most  varied  symptoms  have  been  thereby  designated  as 
neurasthenic.  Not  only  have  the  more  pronounced  physical  condi- 
tions been  included,  such  as  general  arteriosclerosis,  but  it  is  not 
uncommon  for  some  of  the  more  serious  psychoses,  especially  in  their 
milder  manifestations,  as  the  cyclothymias,  to  be  diagnosed  as  neuras- 
thenia. It  is  highly  desirable,  therefore,  to  limit  the  application  of 
the  term  to  a  definite  condition. 

It  is  better  to  consider  neurasthenia  as  the  expression  of  a  very 
marked  auto-erotic  fixation,  as  a  return  to  that  infantile  period  of 
development  in— mhich  the  child  takes  i\.  preponderating  interest  in_ 
its  own_body.  Masturbation  is  quite  liable  to  be~  mdulged  m  as  a 
'means  oFaiiTo-erotic  satisfaction,  but  the  physical  act  of  masturbation 
is  perhaps  relatively  unimportant  as  compared  with  the  crippling 
effects  of  the  auto-erotic  introversion. 

This__condition  is  known  as  a  primary  fatigue  neurosis  and   has 


THE  ACTUAL  NEUROSES 


815 


certain  quite  characteristic  and  constant  symptoms  which  are  in  the 

ie~top-ef'the4iead7moi£^Dp4e994Gsomnia, 

paresthesias, 
Hl^ion. 
)een  traced  in  most 


■,a^gg^Tg^Q^^gSS1lrp^nT^a 
spmal-^qitation^jwryi^perhags^p^^ 
eas^uEatiga^nityT^motional  irrltabrKtyT-andls^edepre 

Despite  outward  evi3ence~to~the-e©fttrai^t  has 
instances  where  careful  analysis  of  the  symptoms  has  been  made,  to 
a  specific  sexual  etiology,  namely,  to  excessive  masturbation  or  fre- 
quent pollutions  or  to  their  psychological  equivalents,  namely,  auto- 
erotic  fantasies.  It  is  the  auto-erotic  fixation,  the  •  inability  to  get 
beyond  that  period  of  development  in  which  pleasure  is  sought  in  self, 
that  is  the  serious  matter  rather  than  the  concrete  evidences  of  this 
fixation  in  any  particular  acts.  In  contrast  to  the  etiologvof  the 
arvopUT  Tipiirosis^^whicli^  as  has  beensaid,  isdepeiTdenrupoiTTan  ina^e- 
quate  utilization  anJmcgmrTtete  discharge"^ the ^nergv^oTtHeTi^^ 
act_in_jthe__£svcHic~'-5prtierer^Tr^^ 
dependent  upQn^^an^JTtadeqtmtg'^tscharge  in  the~pliysical_..sphere. 

One  has  to  think  in  addition  tu  the  specific  Hiblogy  of  the  fact  that 
in  most  instances  where  masturbation  is  practised  into  adulthood  there 
is  a  serious  moral  conflict.  The  individual  feels  ashamed,  chagrined, 
humiliated  by  having  yielded  to  the  physical  demand.  This,  of  course, 
adds  to  the  difficulty  by  increasing  the  amount  of  energy  dissipated. 
In  addition  to  this  the  moral  conflict  is  usuall}'  very  greatly  enhanced 
either  by  being  told  or  reading  of  the  awful  results  of  this  habit.  This 
is  especially  so  when  these  results  are  told  to  the  child  in  order  to 
frighten  him  into  desisting  and  are  accompanied  by  tlireats  of  cutting 
off  the  organ  and  the  like. 

It  must  not  be  lost  sight  of  in  dealing  with  this  class  of  patients  that 
a  moderate  amount  of  masturbation  during  infancy,  about  the  third 
or  fourth  year,  at  the  end  of  what  Freud  calls  the  first  latency  period, 
is  normal  and  probably  has  as  its  function  the  focalization  of  the 
sexual  erethism  upon  the  sex  organs.  It  will  be  remembered  that 
before  this  time  the  various  erogenous  zones  of  the  body  such  as  the 
sex  organs,  the  lips,  the  anus,  are  of  practically  equal  significance.  For 
the  function  of  reproduction  the  sex  organs  must  emerge  with  a  pre- 
ponderant erethism,  otherwise  some  one  of  the  perversions  will  take 
the  place  of  normal  sexuality.  It  seems,  therefore,  the  function  of 
masturbation  to  help  produce  this  result. 

When  masturbation,  however,  is  indulged  in  about  the  period  of 
puberty  and  later  on  into  adult  life  it  has  certain  dangers  in  addition 
to  those  which  are  more  nearly  at  the  physiological  level  and  which 
are  productive  of  neurasthenia.  The  individual  in  his  psychosexual 
development  passes  through  an  auto-erotic  period  when  he  finds  his 
sexual  interests  in  himself,  then  through  a  period  in  which  his  sexual 
interests  are  transferred  to  the  immediate  members  of  the  family,  the 
period  of  narcissism  in  which  at  first  he  is  most  interested  in  those 
members  of  the  family  most  like  himself,  namely  of  the  same  sex. 
Passing  through  these  auto-erotic  homosexual  and  narcissistic  periods  he 
finally  reaches,  after  having  passed  the  period  of  puberty,  to  the  possi- 


816         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

bility  of  giving  his  love  out  to  someone  else,  not  only  besides  himself, 
but  someone  removed  from  the  family  circle  and  someone  of  the  oppo- 
site sex.  He  becomes  normally  heterosexual  and  attains  the  period  of 
object  love.  Now  one  of  the  serious  dangers  of  masturbation  is  the 
danger  it  has  of  fixing  the  individual  at  some  intermediate  point  in 
his  psychosexual  development.  The  principal  danger  is,  of  course 
fixation  at  the  infantile  auto-erotic  period,  which  is  naturally  the  par- 
ticular quality  of  sexuality  that  masturbation  ministers  to.  This 
fixation  not  only  prevents  the  proper  psychosexual  development,  but 
drags  the  whole  personality  back  upon  itself  and  prevents  that  open, 
free,  and  outward  manifestation  which  is  essential  to  success  in  life,  to 
a  finding  of  one's  place  in  the  world.  These  people  are  too  thoroughly 
occupied  with  themselves  to  be  able  to  deal  with  the  outside  world  of 
reality  with  any  degree  of  efficiency.  An  important  aspect  of  the 
world  of  reality  is  the  social  aspect.  Sublimation  tends  always  toward 
socialization  so  that  a  fixation  which  interferes  with  attaining  a  posi- 
tion of  social  independence  as  a  useful  and  respected  member  of  the 
herd  withholds  the  premium  of  social  esteem  and  the  indulger  in  auto- 
erotic  pleasures  feels,  in  consequence,  that  he  has  lost  the  respect  of 
his  fellows. 

In  the  act  of  masturbation  the  individual  is  both  the  subject  and 
the  object.  He  has  to  supply  the  energies  from  both  sources,  not  only 
the  energies  from  within,  but  all  of  the  energies  and  stimuli  which 
normally  would  come  from  without  from  the  person  of  another.  The 
term  masturbation,  from  these  considerations,  it  will  be  seen,  must 
be  considerably  broadened  in  its  meaning.  Masturbation  is  an 
essentially  auto-erotic  phenomenon.  From  this  point  of  view,  sexual 
intercourse,  which  has  only  the  meaning  of  self-indulgence,  is  mastur- 
bation. Intercourse  only  reaches  its  full  biological  significance  when, 
in  addition  to  affording  individual  gratification,  it  gives  something 
which  is  of  social  benefit.  Concretely,  of  course,  this  is  the  case  when 
its  object  is  the  production  of  a  child.  Equally  is  this  true  when  it  is 
an  expression  of  love  which  serves  to  deepen  the  union  and  mutual 
understanding  between  two  persons  so  that  they  become  of  greater 
service  to  each  other  and  thus,  by  their  combined  and  reinforced 
efforts,  to  the  race.  During  his  indulgences  the  masturbator  develops 
all  manner  of  fantasies  and  these  fantasies  often  throw  a  consider- 
ably light  upon  the  nature  of  his  psychosexual  development,  quite 
similarly  as  do  the  dreams  in  those  patients  who  suffer  from  frequent 
pollutions. 

It  will  be  seen,  therefore,  that  in  neurasthenia  there  is  a  condition 
that  is  by  no  means  simple  and  that  requires  careful  analytical  study 
in  order  to  be  able  to  deal  with  it  intelligently. 

A  final  word  as  to  the  causes  of  neurasthenia:  The  alleged  causes 
of  this  disorder  have  been  as  multiform  as  the  conditions  which  have 
been  ranged  under  it.  There  are  a  large  group  of  cases  which  are  sup- 
posed to  be  dependent  upon  injuries,  traumatic  neurasthenia,  and 
another  large  group  that  is  supposed  to  be  dependent  upon  overwork. 


THE  ACTUAL  NEUROSES  817 

Although  it  is  not  quite  possible  to  speak  dogmatically  with  regard  to 
the  traumatic  group  at  this  time,  still  from  analogy,  as  the  result  of 
cases  studied  in  the  group  supposed  to  be  dependent  upon  overwork,  it 
will  be  seen  that  the  same  reasoning  applies  to  both  and  it  is  again  the 
same  reasoning  that  may  apply  to  anxiety  neurosis,  or  in  fact  to  any 
of  the  conditions  described  in  this  chapter,  but  more  particularly 
perhaps  to  the  actual  neuroses.  The  traumatism  or  the  overwork,  as 
the  case  may  be,  or  any  other  apparent  assigned  cause  can  be  said  not 
to  be  the  true  cause  of  the  neurosis,  but  only  its  occasion.  The  trau- 
matism or  the  overwork  could  not  produce  the  neurosis  in  the  absence 
of  the  specific  etiology.  It  is  quite  understandable  that  a  given  indi- 
vidual may  stand  a  series  of  sexual  traumatisms  over  a  considerable 
period  of  time,  but  be  strong  enough  to  resist  the  development  of  a 
neurosis.  On  the  occasion,  however,  of  having  his  resistance  reduced 
as  the  result  of  an  injury,  or  as  the  result  of  long-continued  over- 
work the  neurosis  crops  out.  This  is  the  explanation  for  many  of 
these  conditions,  and  it  is  the  reason  why  a  banal  cause  may 
develop  a  result  that  is  out  of  all  proportion  in  both  quantity  and 
quality. 

Mixed  Neuroses. — Hysteria,  the  compulsion  neurosis,  anxiety  neu- 
rosis, and  neurasthenia  have  been  described.  If  the  etiology  and 
mechanisms  of  these  four  conditions  be  considered  it  will  be  seen 
that  they  do  not  of  necessity  mutually  exclude  one  another  and  as  a 
matter  of  fact  not  infrequently  certain  admixtures  are  found  in  clinical 
experience.  Anxiety  hysteria,  for  example,  has  taken  a  rather  definite 
place  among  these  conditions,  while  as  will  be  readily  seen  from  the 
natm-e  of  the  etiological  moments  neurasthenia  and  anxiety  neurosis 
are  not  infrequently  found  associated  in  various  proportions,  while, 
of  course,  it  follows  that  the  etiological  moments  of  the  actual  neuroses 
are  not  excluded  from  operating  in  the  same  patient  who  may  have  a 
psychoneurosis.  Such  combinations  are  therefore  of  not  uncommon 
occurrence. 

Aside  from  the  possibility  of  the  mixture  of  the  neuroses  it  should 
also  not  be  lost  sight  of  that  the  etiological  moments  of  the  neuroses 
may  also  operate  in  persons  who  are  suffering  from  the  more  severe 
psychoses,  as  for  example,  manic-depressive  psychosis  and  dementia 
precox.  We  quite  commonly  find  hysterical  symptoms  in  the  precox, 
while  neurasthenic  and  anxiety  states  are  not  infrequent  in  the  depres- 
sions of  manic-depressive  psychosis.  Other  combinations,  of  course, 
might  be  mentioned.  The  important  thing  to  bear  in  mind,  however, 
is  the  nature  of  the  etiological  moment  and  the  mechanism  of  the  con- 
dition, and  then  these  will  be  recognized  when  the  individual  patient 
is  under  analytic  observation. 

Finally,  the  purely  psychic  element  is  found  more  and  more  in 
association  with  the  actual  neuroses.  This  was  intimated  in  the 
description  of  the  etiology  and  mechanisms  of  neurasthenia  and  it  has 
also  been  spoken  of  in  the  description  of  the  anxiety  neurosis.  More 
will  be  said  on  this  point  under  the  head  of  Treatment. 
52 


818 


THE  PSYCHONEUROSES  AND   ACTUAL  NEUROSES 


Treatment  of  the  Neuroses. — In  the  treatment  of  the  actual  neuroses 
the  majn-diiQg^as  indicated  by  the  description  which  has  been  given, 
is  to  l^rrect  the~se?tuaHife^of~thie  patigrij.  In  deahng  with  neuras- 
theniathe  habit  of  masturbation  (using  this  term  in  its  broader  sig- 
nificance, inchiding  the  auto-erotic  fantasies),  if  it  be  present,  must, 
of  course,  be  dealt  with  before  anything  definite  can  be  accomplished, 
while,  of  course,  with  both  neuroses,  matters  of  coitus  interruptus, 
ejaculatio  precox,  abstinence,  etc.,  must  be  carefully  inquired  into  and 
the  sexual  life  modified  as  indicated  so  that  the  evil  effects  which  result 
from  them  may  be  remedied.  These  are  the  simple  things  to  do,  and 
in  many  cases  will  produce  marked  betterment,  if  not  apparent 
recovery. 

Of  course  in  dealing  with  these  conditions  it  is  not  meant  simply 
that  certain  concrete  physical  ways  of  indulgence  should  simply  be 
stopped.  It  is  essential  that  the  whole  scheme  of  living  should  be 
raised  to  a  higher  plane  based  upon  an  understanding  by  the  patient 
and  his  orientation  toward  healthier  ideals. 

test  cures».Jiydrothera^,  r^^^ssa^gp^1pptr^pit^evpr£j^p^^J^^d  all 
suck  therapeutic  agents  have  theirl.pl are  in ^I^Hg^  treatment  of  tlie 
neuroses^particul^Iy'the  actual  neurgsesr  but  theirj)lace  is  a  second^ 
ary  o.ne.^^It  has^alr^ady  been  indicated  that  the  precTpitatmgTact 
in  the  outbreak  of  a  neurosis  may,  for  example,  be  overwork,  but 
that  the  neurosis  would  not  eventuate  in  the  absence  of  the  specific 
etiology.  The  explanation  of  this  occurrence  was  that  the  patient  was 
strong  enough  to  stand  up  under  the  results  of  sexual  traumata  until 
his  general  resistance  was  reduced  by  overwork  and  then  the  neurosis 
appeared.  It  will  be  seen,  therefore,  that  the  usual  methods  of  treat- 
ment very  frequently  bring  about  a  cure,  but  not  in  the  way  in  which 
they  are  supposed  to.  By  changing  the  patient's  sexual  habits, 
removing  him  from  his  surroundings,  sending  him  away  to  a  sani- 
tarium, oftentimes  the  sexual  situation  is  more  or  less  well  solved 
temporarily.  Now,  if  during  this  period  of  residence  in  a  sanitarium 
he  is  on  careful  ^t,  given  regular  exercise  with  baths  and  massage, 
it  is  perfectly  understandable  that  his  general  resistance  will  be 
increased  so  that  he  may  overcome  the  effects  of  the  sexual  traumata. 
In  this  way  he  may  get  well  without  any  direct  attack  upon  the  factors 
of  the  specific  etiology.  This  is  the  principle  which  is  oftentimes  seen 
in  operation  in  the  improvement  and  alleged  recoveries  of  neurotics 
as  a  result  of  the  rest  cure.  The  old  symptoms  may  return  in  full 
vigor  following  a  rest-cure  regimen  as  soon  as  the  patient  undertakes  to 
resume  his  usual  way  of  living. 

Inasmuch,  however,  as  even  the  actual  neuroses  usually  present 
some  admixture  of  purely  mental  symptoms,  either  primary  or  second- 
ary in  origin,  it  may  be  necessary  ultimately  and  before  satisfactory 
results  can  be  obtained  to  deal  with  these  mental  symptoms.  If  so, 
they  must  be  dealt  with  in  precisely  the  same  way  as  the  mental 
symptoms  of  the  psychoneuroses,nan^g]y.  by  psychoanalysis. 

The  main  principle  involved  in(psychoanaly^s  may  be  said  to  be 


THE  ACTUAL  NEUROSES  819 

an  analysis  of  the  patient's  mental  condition  sufficiently  complete  to 
thoroughly  understand,  to  reconstruct  the  s>-mptomatic  manifestations 
of  his  malady.  Before  psychoanalytic  methods  were  employed  usually 
no  explanation  was  sought  for  mental  s\Tnptoms  and  apparently  it 
rarely  entered  anyone's  mind  thpt  they  had  any.  The  patient  who 
had  a  phobia  or  a  tic  was  simply  looked  upon  as  being  nervous,  perhaps 
having  had  some  fright  or  bad  impression  earlier  in  life,  and  was  usually 
treated  by  tonics  or  rest  or  travel  or  some  other  such  means  that  was 
not  addressed  to  the  solution  of  the  problem  in  any  way.  From  what 
has  already  been  said  about  the  psychic  development  of  the  individual 
it  will  be  readily  appreciated  that  no  mental  fact  can  fail  to  have  a 
thoroughly  logical  and  understandable  reason  for  its  existence,  and  it 
is  one  of  the  objects  of  psychoanalysis  to  find  this  out. 

Briefly,  the  technic  of  psychoanalysis  is  about  as  follows,  being 
of  course,  modified  in  detail  to  some  extent  by  the  exigencies  of  the 
occasion  and  as  the  result  of  the  special  predilections  of  the  physician 
practising  it.  ^Yhen  the  patient  calls  upon  the  physician  the  physician 
should  let  the  patient,  as  far  as  possible,  without  interruptmg  him  by 
questions  or  otherwise,  detail  to  him  his  difficulties.  This  may  take 
only  a  few  minutes,  or  may  be  quite  a  lengthy  recital,  but  it  is  usually 
worth  while  to  listen  carefully  to  the  whole  thing,  perhaps  occasion- 
ally by  a  suggestion,  keeping  the  patient  to  the  point  if  he  tends  to  be 
too  circumstantial.  This  original  statement  contains  a  description  of 
the  things  from  which  the  patient  is  suffering,  and  if  careful  attention 
is  paid  to  it  one  may  get  many  hints  as  to  how  to  pursue  the  further 
inquiry.  As  a  result  of  this  conversation  and  perhaps  another,  if  it  is 
necessarv',  the  physician  makes  up  his  mind  whether  the  patient's 
illness  is  a  suitable  one  for  psychoanalytic  treatment,  and  if  it  is  he  so 
states  and  then  makes  arrangements  for  regular  consultations,  prefer- 
ably not  leS)S  than  three  times  per  week,  of  an  hour's  duration  each,  and 
all  other  preliminaries  are  arranged  too.  Should  it  appear  that  the 
patient  has  any  s^Tnptoms  of  physical  illness  he  should  be  sent  to  a 
competent  physician  skilled  in  the  particular  trouble  that  appears 
to  be  present,  for  the  psychoanalyst  should  under  no  circumstances 
undertake  to  treat  the  physical  condition.  The  reasons  for  this  will 
appear  later.  It  is  preferable,  too,  that  the  physical  condition  be 
attended  to  fully,  if  possible,  before  the  psychoanalytic  treatment  be 
taken  up.  In  other  words,  it  is  undesirable  to  do  a  psychoanalysis 
while  the  patient  is  under  the  care  of  another  physician  .V 

Having  arranged  all  the  preliminaries,  the  patient  calls  at  the 
appointed  hour  and  the  psychoanalytic  conversation  proceeds  about 
as  follows:  Bearing  in  mind  the  ultimate  goal,  the  psychological 
explanation  of  the  patient's  symptoms,  the  patient  may  be  approached 
by  beginning  the  discussion  of  one  of  the  symptoms,  either  simply 
asking  about  it  or  else  pursuing  some  line  of  inquiry  that  was  suggested 
in  the  original  conversation.    On  the  other  hand,  the  method  may  be 

'  Jelliffe:  Technique  of  Psychoanalysis,  Nervous  and  Mental  Disease  Monograph 
Series,  No.  26,  1917. 


820  THE  FSYCHONEUROSES  AND  ACTUAL  NEUROSES 

pursued  of  endeavoring  to  first  get  a  clear  understanding  of  the  whole 
life  of  the  patient,  beginning  from  the  earliest  recollections  and  trac- 
ing the  development  to  the  present.  It  really  makes  very  little  differ- 
ence how  one  starts,  because  in  a  very  short  time  there  will  be  all 
manner  of  suggestions  to  develop  inquiries  along  various  lines,  and 
these  will  have  to  be  followed  outJacMxamtangling  of  the  situation. 

During  the  course  of  the  j^gychoanalyjty;  conversations  one  will  get 
very  shortly  to  a  point  from  which  progress  seems  to  be  impossible,  for 
it  does  not  take  long  to  exhaust  the  conscious  material  of  the  patient. 
One  then  has  to  penetrate  the  foreconscious,  which  is  relatively  easy, 
and  the  unconscious,  which  is  relatively  difficult.  The  method  of 
procedure  here  is  the  method  of  free  association.  Perhaps  a  point  has 
been  attained  in  the  conversation,  a  situation  has  been  unfolded,  which 
has  no  apparent  explanation.  The  patient  cannot  give  any  reasons 
which  adequately  account  for  it.  Under  these  circumstances  the 
patient  is  asked  to,  so  to  speak,  take  the  situation  as  a  starting-point, 
and  then  relaxing  into  a  condition  of  perfect  passivity  observe  the 
thoughts  that  come  to  his  mind  and  recount  them  as  fast  as  they 
appear.  In  other  words,  he  is  asked  to  place  himself  in  a  mental 
state  favorable  to  fantasy  formation,  he  is  asked  to  relinquish  his 
grasp  upon  his  mental  life,  to  permit  his  ideas  to  flow  untrammelled 
and  unselected  by  his  volition  and  critique.  He  is  asked,  as  it  were,  to 
become  the  observer  of  his  own  ideas,  to  sit  as  if  he  were  sitting  in  the 
window  of  a  moving  train,  recounting  aloud  the  objects  as  they  passed 
by.  So  he  is  asked  to  observe  his  ideas  and  to  tell  them  as  they  come. 
This  sounds  like  rather  a  simple  procedure,  but  it  is  a  very  difficult 
one  for  the  patient  to  learn,  and  in  fact  when  the  patient  can  do  it  and 
do  it  easily  he  is  approaching  the  end  of  his  treatment. 

Experience  shows  that  when  patients  are  instructed  in  this  way 
.they  take  the  situation  as  directed  as  a  starting-point,  and  with  the 
intention  of  telling  the  ideas  that  come  to  their  mind  they  will  relax 
into  a  condition  of  passivity.  Perhaps  then  for  a  considerable  time 
they  do  not  speak,  and  if  they  are  asked  why  they  do  not  tell  what 
comes  to  their  mind  they  will  say  that  nothing  comes.  This  is,  of 
course,  the  interference  of  the  repressed  complexes ;  it  is  the  resistance 
which  they  have  to  coming  into  consciousness  which  is  manifesting  itself 
in  this  way.  The  whole  thing,  then,  has  to  be  gone  over  again  with  the 
patient.  It  has  to  be  explained  to  them  that  their  mind  cannot  be  an 
absolute  blank,  and  they  have  to  be  warned  especially  not  to  exercise 
choice  as  to  what  ideas  they  shall  tell  and  what  they  shall  not  tell, 
that  it  makes  no  difference  how  absurd  or  inconsequential  the  idea  is 
that  comes  to  their  mind  they  must  tell  it;  even  if  the  idea  is  extremely 
disagreeable  they  must  tell  it,  for  no  matter  how  little  connection  it 
may  appear  to  have  with  their  trouble,  if  the  starting-point  has  been 
from  some  problem  in  the  case  these  ideas  that  come  must  have  some 
connection  with  that  problem.  It  must  be  explained  to  them  that,  of 
course,  they  cannot  see  the  connection,  but  that  they  must  tell  the 
ideas  so  that  the  physician  may  have  them  and  that  he  will  be  able 


THE  ACTUAL  NEUROSES  821 

to  see  what  bearing  they  have  in  the  situation.  Of  course  he  may 
not  be  able  to  see  at  once,  but  it  is  so  much  material  which,  if  it  does 
not  come  in  for  utilization  today,  can  be  used  perhaps  tomorrow,  or 
the  day  after. 

This  is  the  process  of  free  association,  one  which  is  very  difficult 
for  the  patient  to  learn  and  one  which  requires  much  skill  and  no 
little  art  on  the  part  of  the  physician.  The  physician  must  be  ever 
on  the  alert.  It  requires  the  most  intense  application  to  the  question 
in  hand,  for  everything  must  be  watched  with  the  utmost  care.  Every 
little  detail  must  be  observed  as  containing  perhaps  a  hidden  meaning 
behind  it.  The  hesitations,  the  stammerings,  the  mistakes,  the  slips 
of  the  tongue,  all  have  their  significance.  For  example,  in  talking  to  a 
young  man,  who  told  about  his  previous  illness,  he  said  that  the 
physician  had  prescribed  four  quarter-grain  tablets  of  "quinin"  for 
him.  He  had  no  sooner  mentioned  the  name  of  the  drug  than  he 
immediately  corrected  himself  and  said  "calomel."  An  inquiry  into 
the  meaning  of  quinin  to  him  brought  out  a  most  important  event 
in  his  life,  an  event  undoubtedly  of  significance  in  his  neurosis.  The 
repressed  complex  was  struggling  as  it  always  is,  for  expression.  A 
favorable  opportunity  presented  itself.  Calomel  and  quinin  are  easily 
mixed  in  the  pronunciation,  and  the  repression  slipped  its  moorings 
for  a  moment  and  found  expression.  It  remained  for  the  observer  to 
be  sufficiently  keen  to  see  the  possibility  of  meaning  in  such  a  mistake 
and  find  out  that  meaning. 

The  most  important^smgle  aid  in  determining  the  content  of  the 
unconscious  is  th^^eai^u^  The  split-off  complexes  are,  in  accordance 
with  the  theorv"  set  forth,  actively  repressed  by  the  indi^•idual,  they 
are  not  permitted  to  come  to  expression  if  he  can  help  it.  They  there- 
fore can  only  express  themselves  in  symbolic  form,  in  which  form  they 
are  disguised  not  only  to  others,  but,  of  more  importance,  to  the 
patient  himself.  In  this  disguised  form  they  are  not  recognized  for 
what  they  are,  and  therefore  the  painfulness  of  their  recall  to  con- 
sciousness is  avoided.  A  previous  moral  delinquency  may  thus  come, 
to  the  surface  under  a  complete  disguise  without  causing  any  particular 
distress,  whereas  it  would  be  quite  unbearable  if  it  came  forth  in  its 
true  colors.  It  can  be  seen  from  this  why  it  is  that  obstacles  are  so 
quickly  reached  in  the  psychoanalytic  procedure.  The  dream  is  the 
best  and  most  useful  avenue  for  overcoming  this  type  of  obstacle. 
Here  the  repressed  complexes  come  upon  the  stage  in  all  their  para- 
phernalia of  symbolic  disguise  and  find  an  opportunity  for  expression. 
The  patient,  not  understanding  what  the  dream  means,  will  pretty 
generally  recount  it  in  all  its  details,  a  thing  which  he  would  absolutely 
refuse  to  do,  in  many  instances  at  least,  if  he  had  the  slightest  suspicion 
of  what  it  could  mean.  The  physician  is  therefore,  so  to  speak,  in  a 
position  to  come  up  on  the  blind  side  of  the  patient,  to  see  the  play  of 
his  unconscious  fantasies,  to  be  let  behind  the  scenes,  as  it  were.  Now 
if  he  can  penetrate  these  disguises  then  he  not  only  is  capable  of  deter- 
mining the  meaning  of  the  dream,  but  also  he  learns  in  this  way  the 


822         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

nature  of  the  repressed  complexes,  and  is  therefore  in  a  position  to 
begin  to  read  meaning  into  the  symptoms  of  the  neurosis.  The  whole 
matter  of  dream  interpretation  constitutes  a  special  chapter  in  psy- 
chology and  is  hardly  discussable  in  a  text-book  of  this  sort  with  its 
necessary  limitations. 

In  addition  to  the  dream  analyses  one  should  also  inquire  into  the 
fantasy  formations  of  the  patient,  fantasies  which  are  formed 
in  the  daytime.  The}''  are  interpreted  on  the  same  principles  as  the 
dream. 

The  events  uncovered  by  the  method  of  free  association  must  be 
dealt  with  as  facts.  It  makes  little  difference  whether  they  ever  did 
happen  in  reality  or  not,  they  are  nevertheless  psycJioIogical  facts; 
they  represent  the  way  the  patient  thinks  and  so  have  just  as  much 
value  as  if  they  represented  real  occurrences. 

It  will  be  seen,  therefore,  that  psychoanalysis  is  a  lengthy,  pains- 
taking, detailed  dissection  of  the  mind  of  the  patient  sufficient  for 
the  reconstruction  of  the  symptoms.  This  dissection  starts  at  the  sur- 
face and  may  go  to  practically  any  depth.  Bearing  in  mind  the  prin- 
ciples already  elucidated  it  will  be  seen  why  it  is  possible  to  effect  an 
apparent  cure  at  various  levels;  why  sometimes  a  single  conversation 
ma}'  apparently  produce  the  miracle  of  a  cure,  while  in  other  patients 
a  half  dozen  will  produce  the  same  effect,  and  in  still  others  months  of 
careful  work  are  required.  The  individual  has  been  thrown  out  of 
adjustment  by  causes  which,  in  their  last  analysis,  have  been  operative 
the  greater  part  of  his  life.  Up  to  a  certain  point,  however,  up  to  a 
certain  difficulty,  he  has  been  able  to  get  along.  Now,  when  this  diffi- 
culty comes  he  breaks  and  the  neurosis  makes  its  appearance.  If  he  can 
be  patched  up,  so  to  speak,  rehabilitated,  back  to  the  point  where 
the  break  occurred,  an  apparent  cure  results.  For  these  apparent 
cures  it  will  be  seen  that  it  is  only  necessary  to  carry  the  patients  back 
to  a  point  at  which  they  are  capable  of  making  adjustment.  This 
is  what  very  often  happens,  especially  in  methods  of  treatment  other 
than  the  psychoanalytic.  For  a  real  cure  of  the  patient,  however, 
something  very  much  more  radical  than  this  is  required:  the  analyst 
has  to  proceed  to  ever  and  ever  deeper  levels  until  he  has  sought 
out  and  found  the  final  stronghold  of  the  neurosis.  Anything  short  of 
this  can  only  serve  to  effect  a  compromise. 

This  seems  to  be  the  most  advantageous  point  to  answer  the  ques- 
tion which  is  constantly  being  asked  and  which  it  seems  impossible 
to  make  many  people  understand,  namely,  the  question  of  how  the 
anah^sis,  the  unravelling  of  the  symptoms,  produces  a  cure.  In  order 
to  understand  that  it  is  necessary  to  recur  to  the  statement  that  the 
illness  is  due  to  a  conflict  and  that  one  element  of  the  conflict  is  uncon- 
scious to  the  patient;  he  does  not  know  what  it  is  that  he  is  fighting. 
He  therefore  cannot  deal  with  it  frankly,  openly,  intelligently.  One 
is  reminded  of  the  story  of  a  certain  king  who  propounded  the  question 
to  the  wise  men  as  to  why  a  bowl  of  water  was  not  increased  in  weight 
when  a  live  fish  was  put  into  it,  while  it  was  increased  in  weight  when  a 


THE  ACTUAL  NEUROSES  823 

dead  fish  was  put  into  it.  This  created  a  tremendous  disturbance. 
All  sorts  of  arguments  and  reasons  were  propounded,  heated  dis- 
cussions arose,  and  the  wise  men  were  quite  generally  out  of  tune 
with  one  another.  Finally,  it  occurred  to  some  one  to  try  the  experi- 
ment and  see  what  the  facts  were.  As  soon  as  the  experiment  was 
tried  and  the  facts  were  determined  there  was  no  longer  any  cause  for 
argument.  The  conflict  subsided.  This  is  quite  comparable  to  the 
position  in  which  the  patient  finds  himself,  fighting  something  of  which  he 
is  unconscious  and  about  which  he  knows  nothing.  The  facts  in  the 
case  in  regard  to  the  fish  in  the  bowl  of  water  were  unknown,  and  as 
long  as  they  were  unknown  nothing  but  chaos  reigned  among  the  wise 
men.  As  soon  as  the  facts  were  brought  to  light,  however,  by  proper 
experimentation,  then  there  was  no  longer  anything  to  fight  about. 
In  addition  to  these  reasons  for  the  subsidence  of  the  conflict  there  are 
others  that  are  of  more  or  less  importance  in  different  cases;  among 
them  is  a  large  element  of  reeducation  to  which  the  patient  is  subjected 
throughout  the  period  of  psychoanalysis.  The  cause  of  his  neurosis 
implies  that  he  is  somewhat  infantile,  somewhat  undeveloped.  The 
physician,  who  should  be  a  man  of  wide  learning,  whose  business  it  is 
to  deal  with  the  problem  of  right  living,  cannot  help  but  infuse  into  the 
patient  in  the  many  hours  of  conversation  a  philosophy  of  life  which  is 
helpful,  and  this  undoubtedly  happens  as  one  of  the  most  important 
elements  in  the  reeducation,  development  and  rehabilitation  of  the 
patient. 

While  the  simple  uncovering  of  the  meaning  of  a  symptom  is  often 
enough  to  make  it  disappear,  for  the  real  rehabilitation  of  the  patient 
the  whole  meaning  of  infantile  ways  of  reacting  must  be  finally  under- 
stood and  the  patient  must  be  willing  to  forego  this  means  of  obtaining 
pleasure  in  order  to  advance  to  a  higher  level  of  adjustment,  to  solve 
the  difficulties  by  attaining  a  higher  level,  which  means  at  once  renun- 
ciation and  fulfilment. 

And  finally,  about  the  matter  of  transference.  The  neurotics  and 
the  psychoneurotics  and  the  great  majority  of  persons  who  require 
psychotherapeutic  treatment,  are  introverted,  that  is,  their  interests 
are  turned  within,  upon  themselves,  and  they  cannot  be  made  over 
into  efficient  people  capable  of  dealing  with  the  outside  world  of 
reality  until  their  interests  can  be  made  to  flow  outside  of  themselves, 
until  they  can  become  interested  in  persons  and  things  and  events. 
In  the  course  of  psychoanalysis,  if  it  is  to  proceed  successfully,  one  of 
the  earliest  things  that  happens  is  that  the  interest  of  the  patient 
begins  to  flow  upon  the  physician.  It  is  transferred  to  him.  This  is 
a  matter  of  utmost  importance.  It  is  a  matter  which  should  be 
watched  with  the  greatest  care,  for  it  is  the  barometer  of  the  relation- 
ship between  physician  and  patient.  It  is  the  factor  in  the  personal 
equation  which  plays  such  a  great  part  and  which  was  supposed  in 
the  old  days  to  play  practically  the  only  part.  As  soon  as  the  transfer 
begins  to  take  place  then  the  patient  will  begin  to  bring  dreams  for 
analysis,  and  in  other  ways  to  show,  so  to  speak,  every  desire  to  please 


824         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

the  physician  by  doing  as  he  wishes,  and  before  long  one  will  generally 
find  that  the  dreams  are  occupied  with  the  idea  of  the  physician,  they 
are  transfer  dreams.  Now  in  these  dreams  one  may  find  exactly  how 
the  physician  is  held  in  the  mind  of  the  patient.  In  symbolic  form 
the  transfer  dream  may  indicate  that  the  physician  is  held  in  high 
regard  and  that  he  is  respected,  and  this  is  of  course  as  it  should  be. 
On  the  other  hand,  he  may  have  failed  to  deal  with  a  certain  situation 
adequately,  and  the  dream  will  show  that  the  patient  is  disappointed 
or  that  perhaps  some  idea  that  the  physician  suggested  the  patient 
thinks  is  ridiculous  and  silly,  or  the  physician  may  make  the  mistake 
of  talking  over  the  head  of  the  patient  so  that  the  patient  cannot 
understand,  and  the  dream  will  say  what  the  patient  could  not  say 
himself,  that  it  is  all  too  deep  for  him,  that  he  cannot  follow,  and  that 
the  physician  is  altogether  beyond  him  in  the  whole  matter,  and  it 
all  seems  quite  hopeless.  The  transfer  dream  therefore  becomes  a 
very  important  matter  and  requires  the  physician  constantly  to  look 
within  and  to  exercise  his  self-critique,  for  he  must  always  realize 
that  if  he  fails  at  a  certain  point  the  trouble  is  not  with  the  patient, 
but  with  himself,  and  sometimes  the  dream  will  indicate  what  the 
trouble  is.  The  meaning  of  the  transfer  is,  of  course,  that  the  patient 
cannot  unburden  his  A^ery  soul,  cannot  stand  unclothed  in  all  the 
nakedness  of  his  real  self,  cannot,  in  other  words,  completely  confess 
himself  to  an  indifferent  person.  There  must  be  something  in  the 
physician  which  commands  the  patient's  affection,  respect  and  con- 
fidence, and  therefore  the  psychoanalytic  work,  while  it  makes  great 
demands  upon  the  patient  also  makes  great  demands  upon  the  physician. 

Sometimes  the  transfer  is  of  such  a  character  and  sufficiently  intense 
of  itself  to  interfere  with  the  progress  of  the  analysis.  Under  these 
circumstances  it  must  be  discussed  with  the  patient,  sufficiently 
discussed  to  do  away  with  the  resistances  it  has  raised,  and  finally  at 
the  completion  of  the  analysis  the  whole  matter  of  the  transfer  must 
be  freely  gone  into  and  analyzed,  so  that  there  is  no  misunderstanding 
as  to  what  its  real  meaning  might  be.  The  patient  is  to  be  placed  in 
full  and  complete  possession  of  all  of  the  facts,  which  means  a  full  and 
complete  possession  of  himself.  Nothing  must  be  hidden  from  him, 
the  mirror  must  be  held  up  so  that  he  can  see  himself  in  it  in  every 
detail. 

It  is  because  of  the  necessity  of  transfer  to  successful  psychoanalytic 
handling  of  a  case  that  it  is  undesirable  to  have  another  physician 
treating  the  patient  at  the  same  time.  The  other  physician  might 
acquire  the  transfer,  and  this  would  prevent  the  psychoanalyst  from 
accomplishing  anything.  This  is  especially  apt  to  be  the  case  where 
the  other  physician  has  to  do  with  the  physical  condition  of  the  patient 
and  has  to  come  into  personal  contact  with  him  in  making  exami- 
nations and  the  like. 

As  already  said,  the  transfer  is  the  result  of  the  beginning  flowing 
outward  of  the  patient's  interests  into  the  world  of  reality.  The 
physician  naturally  is  the  one  toward  whom  this  interest  first  flows. 


THE  ACTUAL   NEUROSES  825 

He  therefore,  so  to  speak,  puts  himself  in  a  position  to  be  utilized 
by  the  patient;  he  becomes  a  bridge  by  which  the  patient  is  able  to 
get  back  into  the  world.  At  the  completion  of  the  treatment,  when 
the  transfer  has  been  fully  analyzed  and  the  patient  understands 
what  it  means,  then  the  physician  by  so  doing  steps  aside.  Having 
served  the  purpose  of  a  bridge,  having  gotten  the  patient  back  into 
reality,  he  steps  aside  and  leaves  the  patient  there  to  stand  upon  his 
own  feet. 

Transference  is  not  an  isolated  nor  an  unusual  phenomenon  nor  is 
it  one  confined  to  the  psychoanalytic  situation.  It  is  a  universal 
psychic  way  of  progress,  the  way  of  interest,  which  takes  the  individual 
from  lower  to  higher  levels  in  his  ways  of  thinking  and  acting.  It 
is  seen  easily  in  the  little  boy's  desire  to  emulate  his  father  and  later 
to  take  some  great  man  as  his  model  for  imitation.  It  is  the  basis  of 
all  interest  in  reality  and  is  the  constant  tool  used  for  affecting  ade- 
quate adjustments  with  the  outside  world.  Life  is  a  constant  play 
of  transfers  of  interests,  that  is  of  love,  and  in  proportion  to  the  capacity 
to  love,  to  give  of  ourselves  to  some  goal,  some  ideal,  are  we  capable 
of  living  our  lives  at  our  best. 

The  transfer  in  psychoanalysis  is  therefore  a  tremendously  power- 
ful influence  for  good  and  also  for  evil  if  used  ignorantly  or  for  venal 
ends.  It  is  used  in  this  latter  way  by  those  who  do  not  know  what 
they  are  doing  and  sometimes  by  a  certain  tj^je  of  practitioner  who 
uses  it  solely  to  keep  the  patient  coming  to  him  and  paying  fees. 
Psychoanalj'sis  thus  demands  a  high  tj^je  of  conduct  on  the  part  of 
the  physician.  It  demands  that  he  devote  himself  unselfishly  and 
unstintmgly  to  the  sole  object  of  the  patient's  welfare.  It  is  only 
when  he  is  able  to  do  this  that  he  becomes  a  worthy  object  of  emula- 
tion and  so  places  himself  in  an  attitude  toward  the  patient,  to  utilize 
to  its  fullest  extent  for  the  patient's  good,  the  great  power  he  possesses 
in  the  transfer. 

The  great  obstacle  to  the  treatment  of  the  psychoneuroses  is  the 
general  obstacle  that  the  patient  in  a  certam  real  sense  does  not  want 
to  get  well.  There  are  two  contrary  trends  struggling  for  supremacy 
in  his  psyche.  He  has  two  sets  of  desires  each  tf\'ing  to  gain  fulfilment 
and  each  diametrically  opposed  to  the  other.  Now  the  symptoms  of 
his  neurosis  constitute  a  compromise,  and  in  his  then  state  of  mhid 
the  only  compromise,  the  only  solution  of  the  problem  possible  to  him. 
He  therefore,  although  he  wants  to  get  away  from  the  suffermg  of  his 
neurosis,  still  is  unwilling  to  give  up  the  symptoms  which  compensate 
him,  even  though  that  compensation  be  inadequate.  This  is  illustrated 
in  many  ways.  For  example,  the  patient  insists  upon  leading  the 
physician  back  to  infantile  occurrences  in  order  to  escape  a  frank 
discussion  and  facing  of  present  problems.  Again,  by  the  free  asso- 
ciations the  patient  will  lead  the  physician  up  all  sorts  of  blind  alleys 
for  the  same  purpose.  The  patient  always  wants  to  avoid  his  task. 
It  is  the  function  of  the  physician  to  hold  him  to  it.  To  this  end  it  is 
important  that  the  physician  should  have  a  wide  knowledge  of  the 


826         THE  PSYCHONEUROSES  AND  ACTUAL  NEUROSES 

meanings  of  symptoms  and  symbols  and  be  able  fairly  clearly  to  see  at 
once  their  general  significance  otherwise  he  will  be  led  into  interminable 
and  futile  discussions.  It  is  only  in  the  final  stages  of  the  analysis, 
when  the  patient  is  made  whole,  at  one  with  himself,  that  he  can 
understand  why  it  is  that  these  things  have  come  about  and  how  it  is 
that  he  no  longer  needs  his  illness,  but  can  dispense  with  it.  So  for 
a  long  time  the  physician  has  to  contend  against  an  innate  desire  on  the 
patient's  part  to  retain  the  symptoms  of  his  illness.  This  is  particularly 
well  seen  in  the  compulsion  neurotic.  Here  the  patient  has  built  up 
an  elaborate  structure  which  he  considers  quite  as  does  the  artist  his 
work  of  art  and  he,  equally  with  the  artist,  resents  all  attempts  to  tear 
it  down. 

Various  accessory  forms  of  treatment,  such  as  baths,  massage, 
sanitarium  treatment,  travel,  etc.,  have  the  same  place  here  as  with 
the  actual  neuroses  mentioned  before.  They  should  never  be  considered 
primary,  but  only  as  secondary.  If  they  are  utilized  without  a  thorough 
analytic  understanding  of  the  patient  they  are  quite  as  apt  to  do  harm 
as  good,  because  by  no  possibility  can  it  be  foreseen  what  the  results 
will  be  unless  the  matter  which  has  to  be  dealt  with  is  known  before- 
hand. 

At  the  present  writing  it  would  seem  that  one  of  the  most  useful 
adjuncts  to  the  psychoanalytic  treatment  of  the  neuroses  would  prove 
to  be  a  carefully  selected  organotherapy.  The  endocrine  imbalances 
represent  the  organic  handicap  of  the  patient  and  there  is  evidence 
accimiulating  that  a  judicious  effort  at  restoring  the  lost  balance  will 
be  of  value  in  tiding  the  patient  over  a  period  of  reconstruction  that 
he  might  be  unable  to  negotiate  without  such  assistance.  Having 
passed  the  critical  period  the  organotherapy  may  perhaps  be  dispensed 
with  for  the  imbalance  may  well  often  be  the  result  of  a  prolonged 
conflict  and  when  the  vicious  circle  is  once  broken  the  balance  returns. 

It  must  be  borne  in  mind  that  the  s\Tnptoms  of  a  neurosis  or  a 
psychoneurosis  may  cover  and  conceal  a  true  psychosis.  This  will 
appear  in  the  course  of  the  analysis,  but  cannot  always  be  foreseen 
as  the  result  of  the  first  examination.  It  is  a  possibility  that  should 
be  borne  in  mind  so  that  the  physician  may  be  guarded  in  what 
he  says  in  regard  to  the  possibilities  of  treatment  and  the  ultimate 
outcome  of  the  case. 


CHAPTER  XVI. 
MANIC-DEPRESSIVE  PSYCHOSES. 

The  general  concept  of  the  manic-depressive  psychoses  has  been 
an  extremely  difficult  one  for  some  reason  or  other  for  many  people 
to  adequately  grasp.  From  the  earliest  times  the  marked  cases  of 
melancholia  and  of  maniacal  excitement  have  of  necessity  been 
observed  and  in  many  instances  ably  described,  and  at  one  period  of 
time  the  manias  and  the  melancholias  constituted  by  far  the  larger 
portion  of  the  types  of  mental  disorder.  At  that  time  in  the  history 
of  psychiatry,  when  the  diagnostic  hair-splitting  was  at  its  height, 
innumerable  varieties  of  mania  and  melancholia  were  described  and 
gi^•en  specific  names.  They  were  differentiated  on  the  basis  of 
whether  hallucinations  were  present  or  not,  whether  delusions  were 
present  or  not,  and  upon  like  matters  of  what  seems  now  superficial 
observation.  It  was  observed  also  that  there  were  a  certain  few  cases 
in  whjph  states  nf  pYPJtement  alternated  with  states  of  depression. 
TH£^,^-called£ircular  types  have  been  recognized  for  a  long  time. 
It  goes  without  saying  that  during  this  period,  when  psychiatry  was 
in  a  purely  descriptive  stage,  a  stage  from  which  it  has  not  yet  by  any 
means  fully  emerged,  when  the  excitements  and  the  depressions  were 
the  symptoms  in  evidence,  that  excitements  and  depressions  belong- 
ing to  all  sorts  of  conditions,  dementia  precox,  general  paresis,  arterio- 
sclerosis, toxic  and  infectious  "psychoses  were  included  in  the  broad 
concepts  of  mania  and  melancholia  that  were  prevalent  and  that  the 
formulation  of  the  manic-depressive  group  has  been  the  result  of  j, 
gradual  weeding  oiir^om~all  of  these  vanous__smirce^^-ijf-the  thing^^ 
that  belong  together_and  the  elimination  of  those_whicli7u"rther 
analysis  showedwere  not  similar. 

~THe"  manic-depressive  concept,  however,  was  not  definitely  for- 
mulated until  Kraepelin,  by  a  study  of  life  histories,  described  the 
condition  as  a  disease  of  afi'ect  fluctuations  which  might  at  one  time 
maiiifest  itself  by  the  prof oundest  depression_jjid_aLjmDther  time  by 
TheTiighest  grade  of  excitement.  Even  afferuiis  formulation  many 
ttrought  that  the  term  manic-depressive  psychosis  applied  only  to 
the  so-called  cases  of  "circular  insanity"  and  failed  to  appreciate 
that  there  were  inherent  and  fundamental  relations  between  the 
two  extremes  of  afl'ect  disturbance.  It  was  difficult  to  understand 
how  there  could  })e  any  relationship  which  bound  together  cases  of 
such  unlike  outward  appearances  and  they  failed  to  see  that  a  patient 
who  manifested  a  single  attack  either  of  depression  or  excitement 
could  be  diagnosed  as  a  manic-depressive  solely  because  the  depres- 

(827) 


828  MANIC-DEPRESSIVE  PSYCHOSES 

sion  or  the  excitement,  as  the  case  might  be,  presented  the  symp- 
tomatology of  the  depression  or  the  excitement  as  found  in  other 
patients  who  presented  a  series  of  attacks  of  both  kinds. 

The  present-day  concept  of  the  manic-depressive  group  considers 
these  as  mental  disorders  essentially  of  affect  fluctuations  manifesting 
themselves  sometimes  by  depression,  sometimes  by  excitement,  some- 
times by  a  mixture  of  the  two  and  finally  the  concept  has  grown  to 
include,  on  the  one  hand,  a  manic-depressive  character  which  tends 
to  react  on  the  basis  of  a  labile  affectivity,  and  on  the  other,  various 
atypical  manifestations  which  present  secondary  symptoms  that 
tend  to  mask  the  fundamental  affective  ones.  And  so  the  concept 
emerges  that  finds  the  root  of  the  psychosis  in  certain  character 
traits  that  never  amount  to  serious  pathological  manifestations  and 
certain  very  mild  affect  fluctuations,  the  cyclothymias,  and  including 
certain  atypical  varieties  with  secondary  symptoms  of  delusion  for- 
mation and  disorders  of  the  sensorium  that  are  of  greater  practical 
significance  than  the  disturbances  of  affect. 

Here  also,  as  elsewhere,  it  is  seen  that  the  manic-depressive  type  of 
reaction  merges  into  other  types  so  that  reactions  that  closely  resemble 
the  manic-depressive  are  seen  in  certain  phases  of  precox,  whereas 
various  of  the  depressions  and  excitements  from  other  causes  also 
closely  parallel  the  manic-depressive  types  in  their  symptomatology. 
With  this  concept  of  reaction  types  in  mind,  these  mergings  into 
adjacent  territories  are  understandable.  With  the  concept  of  a 
disease  entity,  one  which  looks  upon  disease  as  a  definite  sonaething 
back  of  the  symptoms  and  which  produces  them,  it  is  impossible  to 
understand  the  meanings  of  these  attenuated  and  bojxler-^ates.^ 

Etiology. — In  the  first  place  there  areCcertarffTh erpd i ta ry~l7n^^jjrr» 
to  deal  with  in  this  class  of  cases  as  there  are  in  the  precox  group. 
There  are  certain  families  which  show  a  preponderance  of  the  manic- 
depressive  psychosis,  as  there  are  families  that  shov\^  a  preponderance 
of  the  precox  type  of  reaction.  A  recent  study  of  Riebeth^  would 
indicate  that  this  statement  only  applies  to  a  given  generation.  While 
manic-depressive  and  dementia  precox  psychoses  seem  not  to  be 
found  in  the  same  generation  of  a  given  family,  the  two  ps.ychoses 


may  occur  in  different  generations,  in  which  casejiernentifl  prpcnv^s 
iidantsof 


found  in  the  descen" 
seems  not  to  occurT 

In  ha^-mony  with  the  hereditary  tendencies  which  appear  to  b  ^ 
present  in  this  group  of  cases  it  is  found  that  the  group  may  be  widely 
differentiated  into  two  extremes,  the  one  in  which  the  constitutional 
factors  appear  to  be  predominantly  in  evidence  and  in  which  the 
various  attacks  appear  to  originate  either  without  any  cause  at  all 
or  at  least  without  a  determinable  or  apparent  cause  that  is  sufficient. 

1  For  literature  from  1906  to  1910  see  Referate  by  Hamburger.  Ztschr.  f.  d.  gesamte 
Neurol,  u.  Psych.,  Band  ii,  Heft  9. 

*  Ztschr.  f.  d.  gesamte  Neurol,  u.  Psych.  Orig.,  Bd.  xxxi,  Heft  4  and  5,  p.  429. 


MANIC  PHASE 


829 


On  the  other  hand,  there  is  a  group  •which  appears  to  he_more  or  less 
largely  determined  by  causes  which  are  apparent,_gucb_as  the  inability 
of  the  individual  to  adjust  to  certain  conditions  of  hfe  and  repeated 
breakdowns  with  the  return  of  these  conditions.  It  is  important  to 
recognize  these  two  groups  of  cases,  between  which  of  course  every 
intermediate  variety  may  be  found,  because  of  the  significant  bearing 
which  the  type  of  etiological  factor  has  upon  the  probable  outcome 
of  the  therapeutic  attack. 

In  describing  these  t}-pes  of  mental  disturbance  the  two  principal 
phases — the  manic  and  the  depressive — will  first  be  described  and 
then  the  various  forms  of  periodic 
psychoses  of  the  manic-depressive 
group  will  receive  consideration, 
while  finally  certain  less  common 
combinations  of  s^Tnptoms  known 
as  the  mixed  states  will  receive 
attention. 

Manic  Phase. — The  cardinal 
symptoms  of  the  manic  phase  of 
a  manic-depressive  psychosis  are 
three  in  number,  namely  (I)  flight 
of  ideas,  (2)  psychomotor  hyper- 
activity, (3)  emotional  exaltation. 
These  three  s\Tiiptoms  may  mani- 
fest themselves  with  any  degree 
of  severity,  and  the  severity  of 
the  symptoms  may  vary  within 
wide  limits  at  different  times 
throughout  the  course  of  the 
attack.  The'  three  symptoms, 
too,  may  not  be  all  of  the  same 
degree  of  severity,  for  example, 

the  flight  of  ideas  may  be  extreme  and  out  of  proportion  to  the  degree 
of  psychomotor  activity  which  may  show  only  a  slight  increase. 

Taking  the  attack  as  a  whole  the  ordinary  acute  varieties  are 
generally  designated  as  acute  mania  or^cut^  rnaniacaj__£xciiempig? 
Still  milder  grades  are  spoken  of  as(^'ponumh»^nd  the  more  severe 
grades  are  generally  termed  acute ^ocilribus ^mm^  These  three 
degrees  of  excitement  are  the  most  convenient  captions  under  which 
to  describe  the  manic  phase. 

Hypomania,  which  is  the  mildest  of  these  three  degrees  merges, 
of  course,  upon  the  one  hand  into  higher  grades  of  excitement,  such 
as  the  acute  maniacal,  and  on  the  other  into  conditions  of  cyclo- 
thymia, which  may  be  termed  hypomaniacal,  but  which  constitute  a 
special  group  of  this  class  of  cases  which  will  be  considered  separately. 
In  this  condition,  at  least  in  the  simpler  cases,  there  is  a  disorder 
which  involves  the  process  of  thinking  more  than  it  does  the  content 


Fig.  430. — Facial  expression  of  hypomanic 
with  flight  of  ideas. 


830  MANIC-DEPRESSIVE  PSYCHOSES 

of  thought.  The  particular  ideas  and  acts  may  not  be  unusual  and 
yet  abnormal.  This  state  of  affairs  is  excellently  illustrated  by  the 
hypothetical  case  cited  by  Mercier.^  "Its  subject  rises  early,  full  of 
schemes  of  business  or  pleasure.  He  fusses  noisily  about  the  house, 
indifferent  to  his  disturbance  of  other  people's  slumbers.  He  is  very 
impatient  of  delay,  he  cannot  wait  a  minute  for  anything  that  he 
wants  and  if  it  is  not  forthcoming  on  the  instant,  he  flies  into  a  rage. 
The  course  of  the  post  is  not  expeditious  enough  for  him.  He  sends 
his  letters  by  telegraph  and  his  letters  are  extraordinarily  numerous. 
They  would  be  numerous  in  any  case,  but  their  number  is  doubled, 
and  more  than  doubled,  by  the  frequent  changes  of  his  mind,  and  by 
the  impulsiveness  with  which  he  acts  upon  every  passing  whim.  He 
determines  to  make  some  purchase,  probably  a  very  unnecessary  one, 
but  one  for  which  he  can"  adduce  twenty  plausible  reasons,  and  he 
writes  to  tell  his  solicitor  that  he  will  call  the  next  morning.  Scarcely 
is  the  letter  posted  when  he  sees  that  he  will  attain  his  object  more 
quickly  by  asking  his  solicitor  to  lunch.  He  telegraphs  accordingly. 
Before  his  messenger  returns,  it  occurs  to  him  that  he  had  better  ask 
the  vendor  to  lunch  also.  Another  telegraph  is  dispatched  and  since 
he  cannot  entertain  more  than  one  visitor  at  his  club,  another  must 
be  sent  to  the  solicitor  to  announce  the  change  to  a  hotel.  Then 
he  remembers  that  he  has  been  drawing  heavily  of  late  on  his  banking 
account,  and  that  he  may  not  have  the  necessary  funds  available. 
Another  telegram  to  the  bank.  But  if  there  are  insufficient  funds  in 
the  bank,  he  will  have  to  sell  stock  to  raise  the  funds;  another  telegram 
to  his  broker.  Then  he  determines  that  it  will  be  better  to  pledge  the 
stock  to  the  bank  rather  than  to  sell  it.  More  telegrams  to  the 
broker  and  to  the  bank.  The  broker  won't  like  the  contradictory 
orders — never  mind;  ask  him  to  dinner — ask  them  all  to  dinner.  Put 
off  the  lunch  and  have  a  dinner  instead,  and  ask  the  solicitor,  the 
vendor,  the  banker  and  the  broker.  Yes,  and  why  not  Smith  and 
Jones  and  Robinson  as  well?  More  telegrams;  and  then  since  two  out 
of  three  of  the  invited  guests  decline,  the  whole  thing  is  postponed,  also 
by  telegraph.  Meantime,  in  the  intervals  of  telegraphing,  his  hands 
have  been  full.  He  has  been  constantly  ringing  the  bell  and  giving 
orders — giving  them,  modifying  them  and  countermanding  them — 
constantly  wanting  something  fresh,  running  up  and  down  stairs, 
writing  letters,  haranguing  this  person  and  that,  flying  into  a  rage 
upon  the  slightest  opposition,  tearing  the  bell  down  on  the  slightest 
delay  and  talking  almost  incessantly." 

In  this  example  the  subject's  acts  might,  almost  all  of  them,  be 
considered  normal,  with  of  course  the  exception  of  those  due  to  undue 
irritability  or  anger.  Aside  from  this,  however,  each  act  is  consis- 
tently directed  to  some  definite  aim.  The  disorder  is  not  in  the  con- 
tent of  thought  so  much  as  in  the  process  of  thinking,  and  manifests 

1  A  Text-book  of  Insanity,  The  MacmiUan  Company,  1914 


MANIC  PHASE  831 

itself  by  a  rapid  and  toofrec[uent  change  of  direction.  This  is  the 
phenomenon  known  as(^igE^  Idea^  and  may  be  perhaps  better 
illustrated  by  a  stenogram: 

"Do  you  know  I  was  kidnapped  to  be  sent  here  twice.  I  saw  a 
mock  funeral  of  me  before  I  left  home.  This  was  done  because  I  am 
a  great  inventor.  The  Pope  of  Rome  is  the  greatest  human  being  in 
the  universe.  He  is  the  head  of  the  Catholic  Church.  ]\Iy  head 
(association  of  the  word  head  in  two  different  meanings)  is  good  and 
sound,  and  I  am  certainly  not  insane.  Do  you  hear  the  ticking  of 
the  clock?  (External  association.)  It  says,  'call  the  little  heifer,  the 
heifer  is  sick.'  Did  you  ever  see  the  gloves  veterinary  surgeons  use 
when  they  doctor  sick  cows?  (Internal  association.)  Say!  what  are 
you  keeping  me  here  for  anyhow?  I  want  to  go  home.  (Here  he  was 
asked  how  he  slept  at  night.)  I  have  slept  excellently;  that  is  because 
I  am  of  such  a  strong  constitution.  The  Constitution  of  the  United 
States  (association  as  above  with  the  word  head — probably  the  asso- 
ciation is  in  large  part  at  least  a  sound  or,  as  it  is  called,  a  clang  asso- 
ciation) was  signed  by  Thomas  Jefferson.  He  was  just  a  man,  but 
he  was  not  the  inventor  I  am." 

In  this  phenomenon  of  flight  of  ideas  the  patient  either  has  no 
guiding  idea  or  else  at  once  loses  it  so  that  there  is  no  consistent 
and  sustained  eft'ort  directed  toward  attaining  a  goal  idea,  and  the 
thought  therefore  wanders  here  and  there  under  the  influence  of 
chance  associations.  As  a  result  the  train  of  thought  instead  of 
progressing,  changes  direction,  frequently  returns  upon  itself,  and 
never  reaches  a  logical  end.  The  various  ideas  are  not  on  that 
account,  however,  incoherent,  that  is,  they  do  not  fail  to  cohere  or 
to  be  connected  with  one  another,  although  it  may  be  quite  impossible 
at  times  to  see  wherein  this  connection  lies.  If  the  associations  are 
external,  that  is,  originate  in  the  surroundings,  it  is  often  quite  possible 
to  place  them;  when,  however,  they  are  internal,  that  is,  originate 
within  the  patient's  mind,  it  may  be  quite  impossible  to  conceive 
what  they  are.  In  the  example  just  cited,  while  there  are  many 
places  where  the  connecting  link  is  missing,  probably  because  it  was 
an  association  formed  entirely  within  the  patient's  mind,  still  the 
connection  can  be  made  out  in  a  sufficient  number  of  instances  to 
establish  the  characteristics  of  the  train  of  thought.  One  of  the 
principal  characteristics  of  this  type  of  the  train  of  thought  is,  as 
we  have  seen,  its  great  tendency  to  change  of  direction,  and  when, 
for  example,  this  change  of  direction  takes  place  under  the  influence 
of  external  associations,  such,  for  example,  as  the  ticking  of  the 
clock,  as  noted  in  the  stenogram  given,  the  phenomenon  is  known  as 
distractibility.  Any  sensory  impression  is  liable  to  be  the  starting- 
point  of  idea  association,  so  that  these  patients'  trains  of  thought  may 
be  turned  at  will,  almost,  by  such  devices  as  shaking  a  bunch  of 
keys  before  them,  saying  some  word  loudly,  showing  them  a  newspaper, 
or  in  other  words,  momentarily  distracting  their  attention. 


832  MANIC-DEPRESSIVE  PSYCHOSES 

The  ^motional  exali^w^  is  also  well  shown  in  Mercier's  example. 
The  patienTis  constantly  doing  things  which  testify  to  his  idea  of  his 
own  importance.  One  is  struck,  however,  by  a  s>Tnptom  in  the  emo- 
tional field  which  is  perhaps  more  fundamental  than  the  simple 
exaltation  and  which  corresponds  to  the  symptom  just  cited  and  the 
motility  disorders  to  be  described,  that  is,  the  great  lability  of  the 
emotions,  the  rapid  play  of  different  emotional  reactions,  exaltation 
giving  place  to  irritability,  to  anger,  annoyance  and  the  like.  There 
is  no  sustained  emotional  attitude,  as  there  is  no  sustained  direction 
in  the  train  of  thought.  The  psychomotor  hyperactivity  is  also  well 
illustrated  by  this  case.  The  activity  of  the  patient  is  seen  to  be 
constant  and  unremitting,  but  again  it  has  the  same  qualities  as  the 
train  of  thought  and  the  emotional  attitudes  inasmuch  as  it  is  not 
sustained  for  any  length  of  time  in  any  particular  direction,  it  does  not 
get  anywhere,  while  from  time  to  time,  under  the  influence  of  rapid 
changes  of  emotion  the  acts  tend  to  impulsiveness.  There  is  marked 
pressure  of  activity  just  as  there  is  pressure  of  speech,  and  the  patient 
appears  to  be  living  under  terrific  and  unremitting  tension  without 
power  of  direction. 

In  this  condition,  therefore,  the  patient  is  constantly  active,  busying 
himself  about  one  thing  and  another,  talking  continuously  meanwhile 
often  in  a  loud  and  rather  boisterous  manner,  while  emotionally, 
exaltation  is  manifested  by  good  humor,  a  smiling  countenance  and 
increased  self-esteem,  punctuated  mayhap  by  attacks  of  irritability 
or  impulsive  anger  from  little  or  no  cause.  His  confidence  in  his  own 
ability  is  uncfualified  and  is  shown  in  the  outlining  of  all  manner  of 
schemes  of  work,  investments,  business  enterprises  and  the  like. 
Flight  of  ideas  is  marked,  though  not  of  high  degree,  the  conversation 
changing  at  frequent  intervals  from  subject  to  subject  and  the  activ- 
ities sho\v  a  like  characteristic,  there  being  no  consistent  effort  directed 
at  any  one  aim  for  any  length  of  time.  Letters  are  often  written  in 
great  numbers  and  their  contents  exhibit  the  same  characteristics  as 
do  the  speech  and  conduct.  The  patient  is  fully  oriented,  there  is  no 
clouding  of  consciousness  nor  delusions.  In  spite,  however,  of  the 
lucidity  and  apparent  abundance  of  energy  the  real  efficiency  of  the 
individual  is  greatly  reduced  because  of  the  lack  of  consecutiveness 
in  application. 

Ofttimes  the  picture  is  complicated  by  the  addition  of  symptoms 
due  to  alcoholic  indulgence  which  is  very  common  with  patients  in 
this  condition,  many  of  whom  show  marked  moral  delinquencies, 
but  because  of  their  lucidity  and  facility  of  expression  often  elude  the 
authorities,  being  at  once  discharged  after  examination  when  appre- 
hended because  of  supposed  mental  disorder.  This  complication 
with  alcohol  will  be  spoken  of  again  under  the  head  of  Diagnosis. 
Sexual  excitement  is  also  quite  frequently  and  characteristically  in 
evidence  in  these  cases  and  leads  to  moral  delinquencies  which  show 
a  still  further  departure  from  the  patient's  usual    manner  of  con- 


MANIC  PHASE  833 

ducting  himself.  When  complicated  with  alcohol  the  whole  conduct 
may  be  quite  non-understandable  except  to  those  trained  in  the 
recognition  of  this  class  of  cases.  The  sexual  excitement,  of  course,  is 
an  especially  unfortunate  and  dangerous  s\Tnptom  in  young  women 
and  may  lead  to  particularly  regrettable  actions. 

X^^yte  Mam^. — The  next  grade  of  maniacal  excitement  presents 
perhaps  the  most  characteristic  picture  of  this  phase  of  the  disease, 
exhibiting  the  symptoms  to  best  advantage, -though  it  must  be  under- 
stood that  the  symptoms  of  the  different  grades  differ  only  in  degree, 
intermingle  and  are  found  alike  in  all  the  conditions. 

In  this  degree  of  excitement  the  flight  of  ideas  is  well  marked  and 
may  even  become  so  extreme  at  times  that  the  train  of  thought  has 
the  appearance  of  being  quite  incoherent.  Distractibility  is  a  promi- 
nent feature  and  the  patients  are  constantly  diverted  by  inconse- 
quential happenings  in  their  environment.  The  tendency  to  rhyme 
is  quite  frequent  and  the  words  heard  by  the  patient  spoken  by  those 
about  him,  although  they  may  have  no  reference  to  him  and  are  not 
addressed  to  him  in  any  way  are  often  woven  into  or  form  the  starting- 
point  for  these  rhymes  or  for  associations.  The  characteristic  of  these 
associations  is  their  superficiality  and  when  words  that  are  heard 
are  introduced  into  their  conversation  the  basis  of  their  choice  is 
often  nothing  more  than  the  sound  similarity  (clang  association).  It 
is  quite  remarkable  how  such  a  patient  who  is  apparently  paying  no 
heed  to  what  is  going  on  about  him  M^ill  catch  a  chance  word  or  phrase 
uttered  by  some  one,  perhaps  a  considerable  distance  away,  and  intro- 
duce it  into  the  stream  of  his  conversation.  Consciousness  may  be 
somewhat  clouded  and  there  may  be  at  least  apparent  disorientation, 
particularly  for  persons.  This  apparent  personal  disorientation,  how- 
ever, is  dependent  in  the  main  upon  two  factors.  In  the  first  instance 
the  patient  does  not  adequately  perceive  the  environment,  he  does 
not  dwell  long  enough  upon  any  one  particular  element  of  it  to  com- 
prehensively grasp  it  in  the  rapid  and  transito-^y  survey  which  it 
receives  from  him ;  its  elements  are  not  adequately  perceived  and  there- 
fore are  often  misunderstood,  partly  because  of  this  superficial  atti- 
tude toward  the  environment  and  partly,  also  probably,  because  of 
deeper  reasons.  Slight  resemblances  to  friends  or  relatives  are  often 
seen  in  the  patients  and  nurses,  and  these  resemblances  are  magnified 
out  of  due  proportion,  ^nd  so  these  various  persons  are  addressed  by 
the  names  of  members  of  the  patient's  family  for  instance.  These 
resemblances  do  not  necessarily  result  in  a  permanent  and  fixed  mis- 
take. The  person  who  is  at  one  moment  addressed  by  one  name  is 
a  little  later  addressed  by  another,  and  not  infrequently  the  whole 
situation  is  further  complicated  by  the  wit  reaction  of  the  patient 
who  gets  a  good  deal  of  fun  out  of  his  facetious  remarks  and  his  appar- 
ently meaningless  mistakes.  These  errors  being  not  firmly  fixed  are 
frequently  spontaneously  corrected  by  the  patient,  at  least  at  times. 

The  disorder  of  attention,  flip-lit   pf  irl^as  nnd   di'strnctibilitv  are. 
all  eie'mentT^'hich  produce  a  transitory  and  a  superficial  survey  of  the 
53 


834 


MANIC-DEPRESSIVE  PSYCHOSES 


environment  by  prohibiting  any  fixation  or  dwelling  upon  any  particu- 
lar element  of  the  environment  or  even  of  the  ego  and  tend  to  produce 
a  condition  of  the  content  of  consciousness  in  which  all  of  the  ideas 
are  given  the  same  value.  No  one  thing  is  attended  to  long  enough  to 
enhance  its  importance  over  that  of  others.  The  patient  voices  ideas 
first  about  this  subject  and  then  about  that,  changing  from  one  to  the 
other,  not  because  of  any  appreciation  of  differences  of  importance, 
but  in  response  to  the  pressure  which  makes  it  impossible  for  him  to 
rest  anywhere,  so  that  all  ideas  tend  to  reach  the  same  level  of  im- 
portance in  his  consciousness.  There  is  what  is  called  a  levelling  of 
ideas.         ^ — ^ 

While  ligllucinationsS  are  not  an  essential  part  of  the  picture  they 
may  occur,  but  when  they  do,  like  all  of  the  other  elements,  they  tend 
to  be  only  transitory  and  usually  are  rather  simple  and  elementary 
in  character. 

The  Cdfil^^nl)  also  are  inclined  to  be  changeable.  They  partake 
characteristically,  when  present,  of  the  grandiose  character,  but  usually 
lack  the  element  of  extreme  improbability  found  in  conditions  of 
dementia  precox  and  general  paresis.  Occasionally  a  persecutory 
paranoid  system  of  delusions  develops  in  the  manic  phase  of  this 
disease,  but  this  class  of  delusions  is  more  apt  to  develop  and  present 
a  fairly  well  organized  system  in  the  milder  grades  of  excitement. 

The  psychomotor  activity  is  constant.  The_^atients  are  unable  to 
remain  at  rest-  (pressure  of  activity),  they  run  and  jump  arid~~ttinl 
somersaults,  wave  the  arms  about,  tear  up  clothing,  destroy  plants, 
break  furniture,  howl  and  yell  all  night  long,  and  go  almost  absolutely 
without  sleep.  Genital  excitement  may  be  prominently  in  evidence. 
The  excitement  may  be  so  great  that  the  patient  does  not  even  take 
time  to  eat ;  food  placed  before  him  is  perhaps  tasted  and  then  thrown 
about  like  everything  else  that  comes  in  his  way,  so  that  with  the 
lack  of  nutrition,  lack  of  sleep  and  with  the  unremitting  activity, 
emaciation  is  a  constant  feature.     In  less  marked  degrees  of  excite- 


ment, nowever,^^Here~the"  feeling  of  well-being  is  the  controlling 
factor,  it  is  common  for  the  patient  to  gain  somewhat  in  weight. 

The  emotional  exaltation  is  marked  and  shown  by  boisterous  laugh- 
ter and  remarks  showing  exaggerated  ideas  of  self-esteem.  Patients, 
however,  are  spasmodically  apt  to  be  irritable,  bursting  into  attacks 
of  anger  without  adequate  reason  and  often  are  a  constant  source  of 
trouble,  annoyance  and  agitation  upon  the  wards  where  they  are 
confined.  The  emotional  condition  is  as  changeable  as  the  trend  of 
thought  or  of  the  direction  of  the  activities  and  emotions  of  radically 
ogpo6e4-€f«4iitiesmay  easily  replace  each  other. 

jelirious  Mama)— This  condition  is  merely  an  aggravated  state  of 
the  acuEe  mama  already  described.  The  flight  of  ideasjiere  has  pro- 
ceeded^to^almost  complete  incoherence^  i'he  activity  is^unrenuiltng 
and  consciousness  is  more  clouded  a^i3~hallucinatiQjisjnQre  m  evidence. 


le  lackoTsleep  and  proper  nourishment,  with  rapid  emaciation,  soon 


DEPRESSIVE  PHASE  835 

leads  to  great  physical  exhaustion,  while  the  constant  activity  not 
unusually  results  in  slight  wounds  which,  even  though  properly  dressed, 
are  soon  exposed  to  infection  when  the  patient  tears  off  the  dressing. 
Local  areas  of  suppuration  thus  develop,  there  is  a  mild  degree  of 
infection  with  some  temperature,  which  coupled  with  the  exhaustion 
and  the  toxemia,  produce  a  picture  more  distinctly  delirious  with 
marked  clouding  of  consciousness  and  great  incoherence. 

The  acute  delirious  mania  which  used  to  be  described  and  was 
regarded  as  always  fatal,  was  undoubtedly  in  a  certain  proportion 
of  cases  the  h\'permania  of  the  manic-depressive  psj^chosis  to  which 
perhaps  had  been  added,  as  just  described,  sjinptoms  ofCmfectio^ 
toxemia  and  exhatistion,  which  made  the  picture  one  ofCdeliriumT 
Many  other  cases  were  undoubtedly  also  included  under  this  general 
caption,  more  particularly  deliria  associated  \s4th  acute  diseases  of 
the  internal  organs,  such  as  acute  nephritis  or  pneumonia.  One  who 
has  had  experience  with  these  cases  can  understand  how  such  con- 
ditions might  go  unrecognized,  owing  to  the  almost  physical  impossi- 
bility of  subjecting^uch  patients,  in  their  wildly  excited  condition, 
tojiiiythiB^-approaching  an  adequate  physical  examination. 
CChronic  Mani^.--There  are  a  very  few  cases  that  pass  into  a  con- 
dition of  chronic  mania  and  usually,  though  not  always,  have  mild 
excitements  that  may  last  for  a  number  of  years.  These  conditions, 
on  the  other  hand,  may  be  practically  nothing  else  but  character 
anomalies,  cases  of  constitutionally  heightened  (manic)  mood.  Such 
prolonged  phases  of  the  disease,  however,  must  be  borne  in  mind  as 
possibilities. 

Depressive  Phase. — Like  the  manic  phase  this  phase  also  manifests 
itself  bj^  three  cardinal  sjTuptoms  each  diametrically  opposed  to  the 
corresponding  symptom  of  the  manic  phase,  namely  (1)  difficulty  of 
thinking,  (2)  psychomotor  retardation.  (3)  ^motioiuddeyressim. 

This  group  of  s\'inptoms  may,  as  with  the  manic  group,  manifest 
itself  with  any  degree  of  severity,  and  the  three  symptoms  may  sever- 
ally and  individually  vary,  irrespective  of  each  other.  The  retarda- 
tion, for  example,  may  be  quite  out  of  all  proportion  to  the  depression. 

As  with  the  manic  phase,  it  is  convenient  to  consider  the  depressive 
phase  in  three  different  grades. 

Simple  Retardation. — ^The  word  retardation  is  here  used  to  refer  not 
only  to  psychomotor  retardation,  but  to  the  difficultv'  of  thinking 
also,  probably  quite  similar  phenomena,  the  one  more  particularly  in 
the  sphere  of  thought  and  the  other  more  particularly  in  the  sphere 
of  psychomotility.  These  patients  move  and  speak  slowly  and  per- 
haps in  a  low  voice,  by  preference  answering  questions  in  monosyl- 
lables. These  outward  evidences  of  difficulty  of  thinking  and  moving 
are,  however,  more  marked  in  the  next  stage  of  the  depressive  phase, 
that  is,  in  acute  melancholia,  while  here  it  is  more  usual  to  see  the 
patients  merely  preferring  to  be  by  themselves,  disinclined  to  associate 
with  others,  keeping  to  their  room  and  quite  unable  to  make  any 


836 


MANIC-DEPRESSIVE  PSYCHOSES 


mental  effort.  They  are  not  equal  at  all  to  going  on  with  their  work. 
They  may  not,  for  example,  feel  equal  to  WT-iting  letters  or  even  to 
reading  the  newspaper. 

Emotionally  these  patients  are  usually  somewhat  depressed,  but 
the  depression  may  not  be  especially  marked,  it  may  only  appear  on 
questioning.  Consciousness  is  clear  and  the 
patients  are  fully  oriented  and  often  have  a 
realization  of  theix-mental  invalidism. 
■"j^cute  Melancholia}) — In  this  grade  of  depres- 
sidn  the -three  cardinal  symptoms  are  mani- 
fested in  a  much  more  pronouncedway.  The 
patients  are  characteristically  ^^amv^  sitting 
by  themselves,  showing  little  or  no^fendency  to 
associate  with  others,  their  movements  are  slow 
and  deliberate  (executive  retardation)  and  it 
often  takes  a  considerable  timejjn  initiate 
them  (initial  retardation).  The<igeec^is  sim- 
ilarly affected;  it  is  slow,  often  monosyllabic 
and  sometimes  almost  inaudible.  Initial  re- 
tardation is  noticeable  here  also.  The  emo- 
tional depression  is  profound  and  is  indicated 
in  the  general  attitude  of  the  patient  which  is 
one  of  flexion  of  the  body,  the  hands  lying 
limp  in  the  lap,  the  head  inclined  forward,  the 
chin  resting  on  the  breast  and  a  marked  facial 
expression  of  sadness.  The  subjective  state 
of  these  patients  is  described  by  a  feeling  of  difficulty  of  thinking  and 
grasping  the  meanings  of  things  and  of  their  feeling  of  inadequacy, 
of  incapacity  for  all  effort  or  even  thought.  There  is  a  marked  feeling 
of  decrease  in  the  mental  activities  and  the  patient 
dqes  notjed-that^he  has  control  of  his  mmd  and 
oa^n  use^  it  ef£e£tivelyl  Jtithe  same^way'^c^^^ls 
an  mterference  when  he  comes  to  exert  his  will  in 
the  performance  of  voluntary  acts.  There  is  lack 
of  energy,  lack  of  ability  to  initiate  or  to  sus- 
tain an  act  or  a  series  of  acts,  and  in  the  mental 
sphere  alone  the  patient  finds  himself  quite  un- 
able to  carry  out  a  series  of  consecutive  mental 
acts  which  lead  to  a  logical  issue.  He  cannot 
come  to  conclusions,  he  has  an  overwhelming 
sense  of  weariness,  of  relaxation,  of  inadequacy. 
This  general  feeling  of  inadequacy  and  difficulty 
of  thinking  as  above  described  fits  into  and  forms 
a  part  of  the  emotional  attitude  and  acts  with  it  in  determining  the 
character  of  the  delusions.  The  delusions  are  typically  self-accusa- 
tory and  hjT)ochondriacal.  The  patients  think  themselves  responsible 
for  all  the  sin,  wickedness,  privation  and  suffering  in  the  world;  they 
are  the  cause  of  the  unfortunate  condition  of  their  fellow  patients; 


Fig.  431.— Severe  de- 
pression of  several  years' 
duration. 


Fig.  432. — Depressive 
facies. 


DEPRESSIVE  PHASE  837 

they  themselves  have  committed  some  great  sin  and  their  souls  are 
forever  lost.  As  they  occupy  themselves  with  their  owti  moral  states 
so  they  occupy  themselves  with  their  bodily  condition  and  believe 
themselves  sufferers  from  incurable  disease,  think  that  their  organs 
are  decayed,  something  has  happened  to  their  brains,  their  bowels  are 
stopped  up,  their  bones  broken  and  other  such  somatopsychic  ideas. 
When  the  organic  sensations  are  altered  patients  have  strange  feelings 
which  they  interpret  as  indicating  some  mysterious  thing  going  on 
within  their  body,  and  such  sensations  may  be  at  the  basis  or  asso- 
ciated with  some  of  the  h\'pochondriacal  ideas.  The  emotional 
depression  may  at  times  reach  a  very  high  grade  and  express  itself  in 
anxiety  attacks,  moods  of  apprehension,  fear  of  impending  danger, 
a  nameless  dread  of  something  going  to  happen  and  the  like.  The 
whole  world  is  looked  at,  so  to  speak,  through  blue  glasses.  The  sad, 
depressed  mood  colors  every  perception  and  so  the  perceptions  are 
more  or  less  incorrect  and  distorted  to  fit  the  mood. 

Hallucinations  may  occlu*,  but  consciousness  is  usually  clear  and 
the  patient  well  oriented.  There  may,  however,  be  a  lack  of  orien- 
tation toward  their  surroundings  dependent  upon  the  fact  that  they 
are  \\Tapped  up  in  their  own  thoughts  and  the  environment  is  not 
attended  to. 

Physicalli/  there  is  almost  always  constipation,  a  coated  tongue, 
indicaniu-ia,  poor  appetite,  loss  of  weight,  disturbed  sleep  and  often 
circulatory  disturbances  with  cold  extremities. 

Depressive  Stu2)or. — ^This  is  the  third  and  most  severe  grade  of  the 
depressive  phase.  In  this  condition  the  retardation,  both  in  the  field 
of  psychomotility  and  in  that  of  thought,  has  proceeded  to  the  extent 
that  the  patient  lies  wholly  inactive  and  mute;  he  has  to  be  tube -fed, 
and  his  every  want  ministered  to. 

During  this  period  of  absolute  inactivity  it  may  be  that  the  patient 
is  suffering  from  delusions  and  hallucinations  of  a  depressive  and 
horrifying  nature  which  perhaps  are  shadowed  forth  by  an  anxious 
expression  of  countenance,  but  the  details  of  which  can  only  be  learned 
after  the  patient  has  aroused  sufficiently  from  his  stupor  to  be  able  to 
express  himself.  The  hallucinations  may  appear  to  the  patient  much 
as  in  a  dream  and  absorb  his  attention  to  a  very  great  extent.  This 
condition  of  stupor  is  not  uncommon  in  the  course  of  the  depressive 
phase,  but  usually  occurs  as  an  episode  rather  than  as  a  distinct  form 
of  the  disease. 

Chronv:  Depression. — There  are  certain  patients  who  present  for 
long  periods  of  time  a  depressed  mood.  These  cases  may  be  mild 
depressive  phases  of  manic-depressive  psychosis  or  they  may  be 
character  anomalies,  cases  of  constitutionally  depressed  mood  and 
so  show  the  close  interrelations  between  the  normal  fluctuations  of 
emotion  and  those  that  are  pathological. 

The  Periodical  Types. — Under  this  head  are  included  those  forms  of 
the  manic-depressive  psychosis  which  from  time  to  time  have  been 
severally  described  as  recurrent  mania,  periodic  mania,  intermittent 


838  MANIC-DEPRESSIVE  PSYCHOSES 

mania,  recurrent  melancholia,  insanity  of  double  form,  alternating 
insanity,  circular  insanity,  etc. 

All  of  these  psychoses  are  merely  different  manifestations  of  manic- 
depressive  psychosis,  the  manic  and  depressive  stages  being  represented 
in  various  relations,  often  separated  by  a  recovery  interval.  Thus 
recurrent  mania  would  be  recurrent  attacks  of  a  manic  phase  separated 
by  well  intervals,  similarly  for  recurrent  melancholia,  while  alternating 
insanity  would  consist  of  manic  and  depressive  attacks,  each  followed 
by  a  recovery  interval;  circular  insanity,  on  the  other  hand,  being 
cycles  of  manic  and  depressive  phases  without  intervals  of  separation, 
while  insanity  of  double  form  would  consist  of  cycles  of  excitation  and 
depression,  each  cycle  followed  by  a  lucid  interval.  Other  varieties 
might  be  described,  but  it  suffices  to  say  that  the  three  phases — manic, 
depressive  and  lucid  interval — may  be  combined  in  any  possible  way, 
and  that  further  in  a  given  case  any  degree  of  the  manic  or  depressive 
phase  may  occur.  It  is  common,  too,  to  see  during  attacks  of  the 
manic  phase  transitory  attacks  of  depression,  while  during  the  de- 
pressive phase  it  is  equally  common  to  see  transitory  periods  of 
euphoria. 

In  a  number  of  these  cases  the  attacks  reproduce  themselves  often 
at  very  definite  intervals  with  practically  photographic  accuracy  so 
that  the  patient  leads  a  life  the  events  of  which  can  be  predicted 
with  almost  absolute  precision.  Such  patients  not  infrequently  know 
some  little  time  beforehand  when  an  attack  is  coming  on,  and  the 
physician  may  be  able  to  see  the  approach  of  an  attack  the  moment 
he  steps  into  such  a  patient's  room  by  a  little  difference  in  the  arrange- 
ment of  things  that  indicates  the  way  matters  are  going. 

It  would  seem  that  the  patients  who  present  such  definite  cycles 
occurring  at  stated  intervals,  each  exactly  like  the  others,  belong  to 
the  group  of  cases  with  severe  constitutional  taint.  In  the  other  group 
of  cases,  that  group  in  which  external  conditions  seem  to  play  a  large 
part  in  the  etiology  of  the  several  attacks,  there  is  much  less  tendency 
to  regularity  in  their  occurrence,  and  as  heretofore  intimated,  much 
greater  hope  for  the  results  of  therapeutics. 

The  following  is  the  account  of  an  intelligent  woman  of  hejJe^lings 
inJaotL-periods  of  exdtemeiil-and^depressionT    ^~       " 

"I  have  suffered  all  my  life  from  excitements  and  depressions, 
although  it  was  not  until  I  was  fifty-eight  years  of  age  that  my  family 
and  I  realized  I  was  really  mentally  sick  and  required  institutional 
care.  During  youth  and  middle  age  my  excitements  were  of  a  mild 
character,  and  during  these  periods  I  considered  myself  normal.  I 
felt  peculiarly  happy  and  care-free.  I  managed  my  household  affairs 
with  the  greatest  care.  I  entertained  and  mingled  in  society  with 
pleasure  and  zest.  I  was  lively,  talkative  and  I  have  reason  to  believe 
I  was  witty  and  entertaining.  I  could  work  without  an  effort.  I 
at  times  accomplished  almost  Herculean  tasks.  On  one  occasion  I 
remember  preparing  and  conducting  a  church  entertainment  by  which 
the  sum  of  $800  was  raised.    Of  late  years  my  excitements  have 


DEPRESSIVE  PHASE  839 

grown  more  severe.  I  begin  by  taking  an  overactive  interest  in 
everything  going  on  around  me.  Everything  seems  rosy.  I  feel 
happy  and  nothing  depresses  me.  I  feel  propelled  by  some  unknown 
force  to  constant  action.  I  am  possessed  with  the  idea  of  righting 
wrongs  and  straightening  out  things  in  general.  All  the  faults  in  the. 
administration  of  the  ward,  the  hospital  and  the  government  must 
be  corrected. 

"My  excitements  have  never  led  me  to  commit  any  acts  of  violence. 
I  occupy  myself  largely  in  talking  and  writing  letters.  My  room  is 
often  in  disorder  because  I  cannot  stay  at  one  job  long  enough  to 
complete  it.  As  I  feel  these  excitements  approaching,  I  request  the 
physician  in  charge  of  me  to  take  up  my  parole,  as  I  know  I  shall  be 
moved  to  do  and  say  many  foolish  things  of  which  I  will  be  ashamed 
later.  No  one  who  has  not  had  experience  can  realize  the  mortification 
of  having  been  insane. 

"My  depressions  in  early  life  were  as  mild  as  my  excitements,  the 
onset  was  gradual.  I  felt  a  disinclination  to  mingle  in  society.  When 
forced  to  do  so  I  sat  like  a  'dummy'  and  could  think  of  nothing  to 
say.  My  household  duties  became  a  burden.  One  after  another  of 
these  was  dropped  until  the  care  of  the  household  was  entirely  given 
over  to  relatives  or  servants.  I  learned  from  experience  a  treatment 
of  my  own.  As  soon  as  I  felt  a  depression  approaching,  I  promptly 
dropped  everything  and  left  home  for  a  time.  I  found  by  getting 
away  from  family  cares  and  responsibilities,  and  from  the  demands 
of  society,  to  some  quiet  spot,  I  could  shorten  the  duration  of  these 
depressions.  In  recent  years  the  depressions  have  appeared  suddenly. 
One  day  I  went  to  town  to  do  some  shopping  for  a  friend.  I  went  to  a 
grocery  store  to  make  some  purchases.  It  suddenlj^  occurred  to  me 
that  I  could  make  these  to  much  better  advantage  at  the  market 
only  a  block  away.  Suddenly  I  realized  that  I  did  not  have  sufficient 
energy  to  go  to  the  market  and  that  another  depression  was  upon  me. 
It  was  with  the  greatest  difficulty  that  I  ordered  the  goods,  paid 
for  them  and  came  home.  At  these  times  my  brain  feels  paralyzed. 
I  have  not  the  strength  or  ambition  to  do  anything.  I  am  apprehen- 
sive lest  some  harm  has  befallen  the  members  of  my  family,  but  to 
save  my  life,  I  could  not  write  or  telephone  to  find  out  if  my  fears 
are  true.  I  have  the  impulse  to  act,  but  it  seems  as  if  something 
shuts  down  and  prohibits  action.  I  see  my  clothes  becoming  soiled 
— I  know  I  should  change  them,  but  I  cannot  pull  out  the  drawer  of 
my  bureau  and  get  clean  ones.  This  inertia  is  greater  in  the  morning 
than  at  night.  Before  I  came  to  the  hospital  for  treatment  I  had 
servants  who  slept  at  home  and  came  to  my  house  early  in  the  morning. 
When  my  husband  was  away  and  my  children  were  small,  it  devolved 
upon  me  to  admit  these  servants  early  in  the  morning.  I  knew  that 
when  morning  came  to  dress  and  go  downstairs  would  be  impossible. 
I  solved  the  difficulty  by  dressing  the  night  before  and  sleeping  in  my 
clothes.  When  the  depression  is  most  profound,  I  move  in  a  fixed 
groove.     I  never  vary  a  hair's  breadth.     At  first  I  have  a  desire  to 


840  MANIC-DEPRESSIVE  PSYCHOSES 

remain  in  bed.  Once  this  is  overcome  I  have  no  choice  but  to  remain 
up.  I  sit  in  the  same  seat  and  in  the  same  attitude  for  weeks.  As 
I  come  down  stairs  in  the  morning  I  am  apprehensive  lest  my  seat  be 
taken,  and  I  wonder  what  I  shall  do  if  it  should  be  occupied,  although 
the  sitting  room  is  well  supplied  with  comfortable  seats.  I  bring  a 
shawl  with  me  and  place  it  in  the  chair  so  that  no  one  will  appropriate 
it  while  I  am  at  breakfast. 

"After  each  depression,  I  suffer  from  intense  pain  in  my  back,  side, 
shoulders  and  arms.  This  is  dull  and  aching  in  character  and  remains 
with  me  for  weeks  after  the  depression  has  disappeared.  After  the 
last  depression  I  suffered  from  a  severe  attack  of  the  shingles.  The 
skin  eruption  has  now  disappeared  but  the  pain  still  remains." 

The  Cyclothymias. — This  group  of  cases  presents  the  mildest  excite- 
ments and  depressions.  They  deviate  less  from  the  normal  than  the 
other  groups  and  are  only  considered  separately  because  of  their 
great  practical  importance.  They  are  quite  usually  not  recognized 
and  the  sjTnptoms  are  attributed  to  all  sorts  of  things  other  than  the 
real  trouble.  It  must  not  be  lost  sight  of  in  considering  these  mild 
manic-depressive  fluctuations  that  a  slight  depression  may  recur  with- 
out the  psychosis  expressing  itself  by  a  fluctuation  to  the  opposite 
condition  of  excitement  and  vice  versa.  So  that  the  picture  is  seen 
of  patients  presenting  from  time  to  time  mild  degrees  of  depression 
or  mild  degrees  of  excitement  without  anything  approaching  delu- 
sional formation  or  disorders  of  the  sensorium  and  therefore  attracting 
no  particular  attention  from  the  mental  side.  The  following  example 
illustrates  this  exceedingly  well:  He  is  a  man  who  devotes  himself 
largely  to  literary  work,  and  the  fluctuations  in  his  mental  state  are 
shown  excellently  well  by  his  ability  to  write.  The  onset  of  a  depres- 
sive phase  is  usually  shown  by  a  gradual,  though  more  or  less  rapid, 
falling  off  in  his  literary  ability.  He  is  first  unable  to  compose,  then 
he  gets  progressively  less  able  to  write  until  he  is  only  able  to  write 
the  simplest  things.  It  is  the  same  way  in  his  reading.  He  gravitates 
all  the  way  from  reading  connected  with  his  work  down  through  the 
different  grades  of  literature  until  he  gets  to  fiction.  He  finally  finds 
himself  quite  incapacitated,  sitting  for  hours  gazing  out  of  the  window 
or  at  a  blank  wall  and  while  rather  enjoying  company,  it  is  almost 
impossible  for  him  to  initiate  the  procedure  that  is  necessary  to  go 
anywhere.  He  finds  it  almost  impossible  to  dress,  to  get  out,  to  take 
the  cars  and  the  like.  This  state  is  one  almost  entirely  of  retardation 
without  marked  emotional  depression.  During  the  opposite  condi- 
tion of  affairs  he  has  a  feeling  of  well-being  and  efficiency  in  marked 
contrast  to  his  feelings  during  the  depressive  period  and  finds  himself 
quite  able  to  work  for  long  intervals  very  effectively. 

The  remarkable  transitions  from  phase  to  phase  are  shown  well  by 
one  of  his  experiences.  One  day,  having  been  writing  all  of  the  after- 
noon, he,  as  usual,  went  out  to  dinner,  leaving  his  papers  on  the  table, 
intending  to  resume  work  on  his  return.  When  he  came  back  and 
took  up  the  pen  to  write  he  found  that  the  incubus  of  his  depression 


THE  CYCLOTHYMIAS  841 

was  upon  him.  He  had  diflScuhy  in  finding  words  and  finally  after  two 
hours'  effort  he  gave  it  up.  This  was  the  beginning  of  a  depression 
which  lasted  about  a  month.  During  this  time  he  constantly  tested 
his  ability  for  composition  but  without  favorable  result.  Almost 
exactly  a  month  after  this  incident  he  undertook  to  answer  some 
personal  letters,  intending  to  write  only  short  letters  of  perhaps  three 
or  four  pages,  but  when  he  started  to  write  them  he  found  himself 
writing  easily  and  his  letters  spontaneously  expanded  to  eight  or 
twelve  pages  and  he  went  on  into  his  work  again. 

The  hyperthyinic  tyyes  show  exaggerated  activities  in  the  way  of 
the  usual  business  occupations,  writing  letters  and  the  like.  The 
judgment  is  apt  to  be  rather  poor  at  these  times  and  many  of  the 
business  ventures  come  to  grief,  though  not  necessarily  so.  AVork  is 
easily  done,  without  having  made  eflPort,  and  the  patient  expends 
enormous  amounts  of  energy  over  long  periods  of  time.  Certain 
types  of  cases  are  meddlesome  and  troublesome,  tending  to  engage 
in  disputes  and  altercations  and  to  bring  lawsuits,  while  exaggerated 
eroticism  and  alcoholic  predilections  oftentimes  very  considerably 
color  the  picture. 

In  the  dysthwic  types  are  found  the  depressions  which  are  attributed 
in  large  part  to  neurasthenia  and  to  various  visceral  disturbances. 

These  cycloth\Tnic  cases  not  infrequently  show  fluctuations  at 
periods  of  recurrent  physiological  activity  such  as  the  menstrual 
period,  while  it  must  never  be  lost  sight  of  that  not  a  few  so-called 
dipsomanias  are  really  recurrent  manic-depressive  attacks  in  which  the 
alcohol  is  resorted  to  shortly  after  the  attack  commences  and  then 
quite  usually  all  the  symptoms  from  which  the  patient  suffers  are 
attributed  to  the  alcoholic  indulgence.  It  is  important  to  bear  this 
class  of  cases  in  mind,  not  only  for  diagnostic  purposes,  but  in  order 
that  the  patient  should  be  dealt  with  fairly  as  a  sick  man. 

Perhaps  the  most  important  of  the  disturbances  in  this  group  of 
cases  are  the  visceral  disturbances.  There  are  a  large  number  of 
conditions,  particularly  the  false  gastropathies,  enteropathies,  cardi- 
opathies of  Dejerine,  etc.,  many  of  which  belong  here.  Inasmuch  as 
the  psychosis  is  not  recognized,  these  conditions  are  quite  naturally 
credited  with  being  the  cause  of  the  condition  of  the  patient.  Patients 
with  mild  depression  are  called  neurasthenic,  those  with  mild  excite- 
ment are  called  nervous  and  the  accompanying  physical  condition  is 
credited  with  making  the  trouble.  The  patient  and  the  relatives 
consistently  take  this  attitude  and  the  physician  naturally  falls  into 
it.  Xo  one  wishes  to  acknowledge  the  possibility  of  a  mental  dis- 
order, and  therefore  these  other  explanations  are  readily  accepted. 
In  fact,  the  condition  is  hardly  recognizable  at  its  true  value,  even  by 
the  practised  observer,  unless  a  full  account  of  the  patient's  life  is 
available. 

After  a  while  the  symptoms  of  visceral  disturbance  clear  up  along 
with  the  disappearance  of  the  mental  symptoms  and  the  change  is 
attributed  to  some  form  of  treatment,  a  special  dietary  regimen,  or 


842  MANIC-DEPRESSIVE  PSYCHOSES 

whatever  has  been  resorted  to  for  the  rehef  of  the  sjTiiptoms.  Here  is 
a  group  of  cases  who  during  their  attacks  regularly  seek  the  specialist 
and  are  subjected  to  all  sorts  of  gastro-intestinal  treatment,  gastric 
lavage,  special  dietaries,  gynecological  manipulations  of  one  sort  and 
another,  metabolism  experiments,  endocrine  therapy,  treatment  for  auto- 
intoxication, eye-strain  and  almost  everything  in  the  category  of  medical 
specialism,  and  yet  characteristically  in  these  cases  nothing  is  found  in 
the  physical  condition  that  adequately  accounts  for  the  symptoms. 
Another  group  of  these  cases  are  the  paranoid  types.  These  patients 
present  typical  paranoid  symptoms  with  emotional  accompaniments 
that  seem  to  be  hardly  in  excess  of  what  is  demanded  as  normal  reac- 
tions to  the  delusional  state.  This  is  the  group  of  cases  that  has  given 
origin  to  a  great  deal  of  recent  discussion  with  regard  to  the  real  basis 
of  paranoia,  its  relations  to  manic-depressive  psychosis,  the  affective 
origin  of  paranoia  and  its  basis  in  what  Specht  calls  the  "affect  of 
suspiciousness." 

The  Mixed  States. — The  mixed  states  are  forms  of  manic-depressive 
psychosis  in  which  the  three  cardinal  symptoms  of  the  manic  and 
depressive  phases  are  mixed  so  that  the  resulting  state  is  neither  one. 
They  are:  (1)  maniacal  stupor,  (2)  agitated-  depression,  (3)  unpro- 
ductive mania,  (4)  depressive  mania,  (5)  depression  with  flight  of  ideas, 
(6)  akinetic  mania.  It  will  suffice  to  merely  mention  the  s;>Tnptoms 
of  these  groups. 

Maniacal  Stupor. — Emotional  exaltation,  decreased  psychomotor 
activity,  difficulty  of  thinking. 

Agitated  Depression. — Emotional  depression,  increased  psychomotor 
activity,  flight  of  ideas. 

Unproductive  Mania. — Emotional  exaltation,  increased  psychomotor 
activity,  difficulty  of  thinking. 

Depressive  Mania. — Emotional  depression,  difficulty  of  thinking, 
increased  psychomotor  activity. 

Depression  with  Flight  of  Ideas. — Emotional  depression,  flight  of 
ideas,  decreased  psychomotor  activity. 

Akinetic  Mania. — ^Emotional  exaltation,  flight  of  ideas,  decreased 
psychomotor  activity. 

Still  the  possibilities  are  not  exhausted.  It  is  quite  uncommon 
to  see  any  one  of  the  conditions  already  described  continue  pure  from 
the  commencement  to  the  end  of  the  attack.  In  the  manic  phase 
symptoms  of  depression  not  infrequently  crop  up  and  occupy  the 
field  temporarily,  while  during  the  depressive  phase  it  is  quite  as 
common  to  note  transitory  periods  of  excitement.  Then  it  is  quite 
common  for  manic  attacks  to  be  preceded  by  a  longer  or  shorter 
attack  of  depression  and  sometimes  such  a  period  of  depression 
follows,  not  infrequently  but  partial  depression,  of  the  type  of  unpro- 
ductive mania.  The  depressive  phase  shows  similar  variations,  more 
particularly  it  is  followed  by  a  short  period  of  exaltation.  Then, 
again,  at  any  stage  of  the  disease  a  mixed  state  may  crop  up  for  a 
time,  so  that  we  may  see  during  the  course  of  the  manic  phase  psycho- 


NATURE  OF  MANIC-DEPRESSIVE  PSYCHOSIS  843 

motor  retardation  occur  or  during  the  phase  of  depression  emotional 
exaltation  may  develop,  while  in  the  various  forms  of  the  periodic 
psychoses  it  is  quite  the  rule  to  find  these  mixed  states  at  the  transi- 
tion places  from  one  phase  to  the  other,  all  of  the  s^Tnptoms  of  one 
phase  not  equally  and  contemporaneously  graduating  into  their 
opposites.  Thus  during  the  course  of  a  circular  psychosis  the  affect 
may  change  from  depression  to  exaltation  before  the  psychomotor 
retardation  has  given  place  to  increased  psychomotor  activity,  thus 
producing  a  temporary  mixed  state. 

Involution  Melancholia. — The  group  of  cases  comprised  under  the 
term  involution  melancholia,  which  was  originally  used  by  Kraepelm, 
has  now  been  pretty  generally  conceded  to  belong  to  the  manic- 
depressive  group.  The  characteristics  of  the  disease  are  those  of  an 
anxious  depression  occurring  m  later  life.  The  group' is  such  a  con- 
siderable one  and  of  such  practical  importance,  however,  that  it  will 
be  specially  considered  along  with  other  depressions  of  later  life  m 
another  chapter.     (See  Chapter  XXIV.) 

Pathology. — There  are  no  specific  patho]op;ira.1  findjpp^^  in  this  psv- 
chosis,  althouglT  certain  degenerative  products  have  been  described 
m  cases  ot  death  f rom  (jepressive  jtnpQT-  Patients,  however,  char- 
acteristically recover  from  this  condition,  or  if  they  die  during  attacks, 
the  death  is  due  to  some  intercurrent  disease  which  itself  would  pro- 
duce changes  in  the  nervous  system  that  would  gradually  cloud  and 
perhaps  entirely  obscure  any  pathology  that  the  psychosis  might  have. 
A  condition  producing  death  itself  must  produce  serious  alterations  of 
the  central  nerv^ous  system,  that  most  sensitive  of  reacting  portions 
of  the  human  body. 

Nature  of  Manic-depressive  Psychosis. — This  psychosis  perhaps,  as 
thoroughly  as  any  other,  has  withstood  throughout  the  years  any 
attempt  at  understanding  it,  while  as  opposed  to  dementia  precox 
the  symptoms  of  which  appear  quite  unpsychological,  the  symptoms 
of  the  manic-depressive  psychosis  in  either  one  of  its  phases,  more 
particularly  perhaps  in  the  depressive  phase,  are  quite  psychological, 
that  is,  quite  understandable.  The  patients,  to  begin  with,  present 
largely  average  types  ofpersonality  before  theadvent  of  thepsychosis, 
and  during  the  s^^nptoms""of"the^psychos^s  they~^T(!marily  are  not  so 
far  disordered  in  their  conduct  or  in  the  character  of  their  ideas  as  tc) 
place  them,  so  to  speak,  in  a,  cU:ss  bv  tlTemselves.  Thev  arp  still  quite 
like  the  reat  of  iIs.  The  r(U)ts  ol  the  psychosis  appear  to  sprhig  more 
distinctly  from  the  usual  life,  the  fluctuations  of  the  emotions  being 
quite  comparable  to  the  fluctuations  that  occur  in  everyone. 

However,  it  seemed  quite  impossible  to  understand  how  the  patients 
could  vary'  from  one  extreme  to  its  diametrical  opposite  and  what  could 
possibly  be  the  explanation  of  such  shifts  of  position.  For  many  years, 
under  the  domination  of  ]VIe}Tiert,  the  changes  were  supposed  to 
depend  upon  changes  of  blood  supply,  upon  anemias  and  h;\'peremias. 
When  psychiatry,  however,  advanced  beyond  such  crass  t>T)es  of 
explanation  it  was  left  practically  without  anything  to  fall  back  upon. 


844  MANIC-DEPRESSIVE  PSYCHOSES 

Recently,  however,  the  suggestive  work  of  Bleuler^  has  seemed  to 
indicate  what  may  at  bottom  be  the  true  explanation.  He  has 
demonstrated  what  he  calls  the  ambivalency  of  ideas.  This  ambi- 
valency  gives,  as  he  understands  it,  to  the  same  idea  two  contrary 
feeling  tones  and  invests  the  same  thought  simultaneously  with  a 
positive  and  a  negative  character.  Along  with  this  ambivalency 
there  is  an  ambitendency  which  sets  free  with  every  tendency  a  counter- 
tendency.  With  this  basal  supposition  it  can  be  understood  why 
the  fluctuation  of  the  manic-depressive   is   a   fluctuation   between 

conditions  which  are  diametrically  opposed.     If  each.  

ciated  with  i_t  bv  preference  the  idea_which  js  ahaoliilely  itsoppos^te, 
Ueach  feeling  has  associated  with  it  by  preference  thefeeling  wliich 
represents  its  exact  antithesis,  then  there  is  reason  for  understanding 
how  the  manic-depressive  gravitates  between  these  two  extremes. 
It  is  the  path  of  opposites  which  is  met  with  at  every  turn  in  psychi- 
atric experience.  Nothing  suggests  white  more  surely  than  does 
black,  nothing  suggests  love  more  readily  than  hate.  The  opposed 
ideas  and  feelings  stand  with  relation  to  each  other  in  the  path  of 
least  resistance,  and  when  one  would  go  from  a  certain  idea  or  a 
certain  feeling  in  any  direction  he  finds  the  path  to  the  antithetical 
idea  or  feeling  more  easily  passable  than  the  path  to  any  other  goal. 
This  fluctuation  of  the  feelings  along  the  path  of  opposites  exhibits 
a  close  analogy  to  the  mechanism  of  the  compulsion  neurosis  consid- 
ered as  a  fluctuation  between  love  and  hate.  This  explains  some  of 
the  symptoms  which  can  be  seen,  from  this  point  of  view,  to  be  com- 
promise formations.  For  example,  one  patient  wills  most  of  her 
property  to  her  daughter  (love)  but  so  ties  it  up  in  a  trust  that  she 
will  never  get  it  (hate).  She  has  solved  the  problem  of  expressing 
both  antithetical  tendencies  in  the  same  act  (the  will) . 

Assuming  the  h^^pothesis  of  ambivalency  and  ambitendency,  still 
what  is  the  explanation  of  the  affect  fluctuations  in  this  psychosis? 
Here  as  elsewhere  in  the  mental  field  some  fundamental  psychic  con- 
flict undoubtedly  has  to  be  sought  to  which  the  patient  is  making 
efforts  of  adjustment.  This  is  precisely  the  starting-point  from  which, 
for  example,  dementia  precox  has  to  be  viewed.  But  here  are  indi- 
viduals who  present  a  different  possibility  of  reaction,  a  different 
reaction  t^^pe  to  the  conflict  than  do  the  precox  patients.  This 
statement,  of  course,  must  not  be  taken  as  meaning  any  more  than  a 
mere  putting  into  words  of  what  is  found,  because  it  is  not  understood 
what  the  differences  are  that  make  different  people  react  in  different 
ways.  Psychiatrists  are  only  upon  the  verge  of  being  able  to  ask  such 
a  question  intelligently.  They  are  not  yet  able  fully  to  answer  it. 
An  indication  of  what  is  at  the  bottom  of  the  manic-depressive 
reaction  may  perhaps  be  reached. 

The  manic-depressive  psychosis  may  be  conceived  of  as  an  effort 
at  compromise  and  at  defense,  resulting  from  an  endopsychic  conflict. 

1  The  Theory  of  Schizophrenic  Negativism,  Nervous  and  Mental  Disease  Monograph 
Series,  No.  11. 


NATURE  OF  MANIC-DEPRESSIVE  PSYCHOSIS  845 

In  the  depressive  phase  the  affect  has  broken  through  and  mvades 
consciousness,  while  in  the  manic  phase  the  patient  by  feverish  and 
restless  activity,  by  a  constant  alertness,  fights  off  every  approach 
that  might  touch  him  on  a  painful  point,  that  might  reach  a  \Tilner- 
able  spot.  It  would  seem  as  if  he  were  wildly  beating  about  to  keep 
off  all  intruders,  not  only  real  intruders,  but  all  possible,  prospective, 
or  though t-of  intruders.  And  so  the  manic  patient  is  already  quite 
inaccessible  and  all  of  his  reactions  are  especially  superficial,  as  witness 
the  word  associations  and  the  clang  associations.  He  moves  over  the 
surface,  which  he  endeavors  to  cover  completely,  in  order  to  prevent 
penetration  at  any  point. 

This  constant  activity  of  the  manic,  however,  has  another  aspect 
than  simply  that  of  defense.  In  this  constant  activity  of  which  such 
symptoms  as  flight  of  ideas,  clang  association  and  distractibility  are 
types  the  patient  is  constantly  occupied  with  reality.  In  fact  he  is  so 
acutely  interested  in  reality  that  little  that  occurs  about  him  escapes 
him  and  he  is  constantly  showmg  extremely  keen  powers  of  observa- 
tion. This  might  be  termed  a  flight  into  reality  as  a  means  of  escap- 
ing from  the  conflict  and  as  such  stamps  the  psychosis  as  belonging 
to  the  extraverted  t^'pe  as  distmguished  from  the  introverted  type  (of 
which  precox  is  the  best  example)  in  which  the  libido  turns  back  to 
reanimate  channels  in  which  it  used  to  flow  but  which  have  long  since 
been  abandoned. 

The  consideration  of  the  manic-depressive  reaction  from  the  point 
of  view  of  an  extraversion  type  of  psychosis  is  extremely  helpful  in 
affording  a  basis  of  explanation  for  many  of  the  mooted  points  which 
have  been  raised  in  the  recent  literature  regarding  this  much  discussed 
psychosis.  In  fact  it  would  appear  that  a  great  deal  of  the  difficulty 
encountered  by  observers  depends  upon  dealing  with  the  psychoses 
solely  at  the  descriptive  rather  than  at  the  interpretative  level.  There 
is  a  good  deal  of  effort  being  expended  in  trying  to  split  up  the  whole 
group  into  smaller  subdivisions  and  undoubtedly  the  future  will  show 
an  increasing  success  in  these  efforts.  Still  it  is  already  not  difficult 
to  see  why,  for  example,  one  should  find  hysterical  sNTuptoms  asso- 
ciated with  the  more  purely  manic  since  hysteria  is  also  an  extra- 
version  psychosis  as  is  well  sho\vTi  by  the  extremely  strong  phenomena 
of  transfer  exhibited  by  hysterics. 

Of  special  importance,  however,  seems  the  relation  of  extraversion 
to  the  relatively  benign  nature  of  the  attacks  as  compared  with  the 
relatively  malignant  character  of  the  introversion  in  precox.  The 
fact  that  the  patient  attempts  to  escape  from  his  conflict  by  a  flight 
into  reality  rather  than  by  a  path  that  leads,  by  introversion,  through 
the  conflict,  as  is  so  often  the  case  in  precox,  seems  to  insure  that  he 
again  and  again  is  able  to  rehabilitate  himself.  The  efficiency  of  one's 
relation  to  reality  is  the  measure  of  one's  normality  and  so,  in  this  psy- 
chosis, the  constant  effort  to  plunge  into  reality,  to  become  immersed 
in  it,  to  deal  with  it  at  everv'  point,  becomes  a  saving  grace  and  is 
largely  accountable  for  the  frequent  recoveries, 


846  MANIC-DEPRESSIVE  PSYCHOSES 

Undoubtedly  certain  groups  of  sjonptoms  in  this  great  mass  of 
material  will  be  found  to  hang  together  with  sufEcient  certainty 
and  frequency  to  be  classed  as  disease  types  but  still  the  manic- 
depressive  way  of  reaction  may  still  be  considered  as  such  aside 
from  such  groups.  When  so  considered  it  would  seem  that  it  con- 
stitutes a  method  of  handling  the  conflict  that  belongs  relatively  high 
in  the  scheme  of  the  psychic  organization.  In  other  words,  the 
manic-depressive  patient  belongs  to  a  relatively  superior  tj^^e  of 
personality.  This  is  seen  by  the  comparative  normality  of  the  per- 
sonality tN^ies  involved,  by  the  psychological,  that  is,  understandable 
character  of  the  symptoms  which  seem  to  be  only  exaggerations 
of  normal  reactions  and  also  perhaps  by  such  a  fact  as  that  brought 
out  by  Riebeth  (see  Etiology)  that  while  manic-depressive  psychosis 
is  found  in  the  ascendants  of  precox  the  reverse  does  not  seem  to  occur. 

Still  considering  the  manic-depressive  reaction  as  an  extraversion 
type  one  is  prepared  to  find  in  individual  cases  that  the  symptoms 
do  not  necessarily  run  pure  to  type.  It  is  understandable  that  in 
some  cases  there  should  be  a  mixture  of  introverted  mechanisms  and 
when  this  occurs  it  can  be  seen  why  such  a  symptom  as  negativism 
should  be  apt  to  appear  because  the  symptom  belongs  to  the  catatonic 
type  of  precox  which,  like  manic  reaction  seeks  to  solve  the  conflict 
by  running  away  from  it,  although  in  a  different  direction.  The 
constant  strain  of  seeking  rehabilitation  by  a  flight  into  reality  causes 
a  break  and  a  resort  to  a  simpler  mechanism  of  escape.  In  the  same 
way  manic-like  flights  in  certain  cases  of  catatonic  excitement  may 
be  expected.  The  manic  episode  evidences  a  specially  great  effort  to 
get  back  to  reality  which  is  only  partially  or  briefly  successful  because 
making  too  great  demands  upon  the  t}n[>e  of  personality  involved. 

The  manic-depressive  way  of  reacting  may  also  be  seen  in  paresis, 
arteriosclerosis  and  other  organic  conditions  and  also  in  certain  psy- 
chopathic and  degenerative  tj^es  as  in  the  prison  psychosis,  certain 
litigious  paranoiacs  and  pseudologia  phantastica.  In  all  these  cases 
the  extreme  activity,  the  constant  occupation  with  reality  and  the 
generally  exalted  mood  indicate  an  extraversion  which  must  be  further 
studied  in  its  roots  to  know  its  real  meaning. 

The  physical  conditions  have  been  dwelt  on  more  particularly  in 
discussing  the  cycloth\Tiiias  because  here  the  physical  symptoms 
occupy  the  foreground.  In  the  more  severe  psychotic  disturbances 
physical  sjTiiptoms  if  present  may  easily  be  overlooked.  The  anam- 
nesis will  show  very  frequently,  in  the  prodromal  period,  such  s;^Tnp- 
toms  as  headache,  neuralgias,  herpetiform  eruptions,  etc.  It  is  not 
infrequent,  however,  to  see  marked  evidences  of  biological  maladjust- 
ment in  the  way  of  the  general  habitus  of  the  opposite  sex,  evidences 
of  endocrinopathies  (glandular  imbalances),  and  homosex-ual  s>Tnbol- 
izations  at  the  psychological  level.  These  physical  accompaniments 
can  be  understood  in  accordance  with  the  principles  laid  dowm  in  the 
introduction.  Undoubtedly  the  finer  studies  of  the  future  will  uncover 
manv  more  of  them  than  are  now  known  and  indicate  further  their 


DIFFERENTIAL  DIAGNOSIS  847 

special  meanings  in  the  whole  picture.  A  careful  analysis  of  the  vis- 
ceral states  and  postural  tension  accompanying  these  extreme  emo- 
tional reactions  will  prove  of  the  greatest  importance  to  their  adequate 
understanding. 

Course  and  Prognosis. — The  mdividual  attacks  vary  in  duration  from 
a  few  days  to  several  months^  l^ecovpry  fropi  the  single  attack  is 
fKe~^le,  while  tlTe  likelihood  of  subsequent  attacks  is  considerable. 
In  general,  therefore,  the  prognosis  is  good  for  the  separate  attacks 
and  is  rather  poor  as  to  ultimate  freedom  from  attacks.  As  pointed 
out  previously,  the  severe  constitutional  tjqjes  have  a  worse  prognosis 
than  those  tj^pes  in  which  the  etiological  factors  are  capable  of  removal. 
In  the  severe  constitutional  tj^jes  also  the  recurrent  attacks  tend  to 
repeat  with  photographic  accuracy  preceding  attacks,  while  in  the 
long  run  the  general  tendency  is  for  an  increase  in  the  length  of  attacks 
and  a  decrease  in  the  length  of  the  free  intervals. 

The  disease  pursues  its  coiir^o  without  any  .special  tendencvto 
deterioration^  Although  mild  grades  of  dementia  have  been  described, 
terminatmg  protracted  attacks,  the  dementia  which  usually  super- 
venes in  the  course  of  the  disease  is  that  which  is  superadded  as  the 
result  of  the  changes  incident  to  arteriosclerosis  and  the  senium. 
Inasmuch  as  this  psychosis  tends  to  recur  throughout  life,  not  a  few 
of  the  patients  ultimately  reach  senescence. 

Differential  Diagnosis. — The  manic  phase  in  its  mildest  forms  is  often 
mistaken,  especially  w^here  it  leads  to  alcoholic  and  sexual  excesses, 
for  a  form  of  moral  obliquity.  In  the  somewhat  more  pronounced 
attacks  it  may  be  difficult  to  differentiate  it  from  other  excitements, 
more  particularly  catatonic  excitement.  In  general  the  manic  excite- 
ment is  more  free  and  open,  there  is  less  tendency  to  constraint  along 
any  particular  line,  while  the  productivity  and  the  psychomotility  are 
not  as  meaningless  or  non-understandable  as  with  dementia  precox. 

The  depressive  phase  in  its  milder  manifestations  is  not  infrequentlv 
misialcen  for  neurasthenia  a^ndT  in^Tts~lnore  pronounced  fornT  it  is 
^n:i'etIiely"'HlfflcuHjtojttffgT^nt^^  it  from  the  depression  of  dementia 
precox7~^liiris  pailicuIaH}^^ 


the  manic-depressive  is  very  close  to  the  empty  feelings  with  loss  of 
affect  of  the  precox,  while  the  blocking  of  movement  and  expression 
in  the  latter  condition  outwardly  closely  resemble  the  retardation  in 
manic-depressive  psychosis.  The  stupor  of  catatonia  outwardly  also 
closely  resembles  the  manic-depressive  stupor,  except  that  it  is  more 
apt  to  be  associated  with  marked  negativism,  muscular  tension  and 
perhaps  grimacing. 

The  greatest  difficulty,  as  between  manic-depressive  psychosis  and 
dementia  precox,  lies  with  the  differentiation  of  the  mixed  states. 
Here  the  resemblances  are  quite  close  and  prolonged  observation  often 
necessary  to  make  the  differentiation. 

It  must  not  be  forgotten  that  the  manic-depressive  psychosis  is 
by  no  means  a  clear-cut  definite  entity,  that  it  merges  in  all  directions 
into  other  conditions,  and  that  its  closest  affiliation  with  the  other 


848  MANIC-DEPRESSIVE  PSYCHOSES 

psychoses  appears  on  the  surface  to  be  with  the  dementia  precox 
group.  There  are  quite  a  considerable  number  of  cases  in  which  a 
study  of  the  individual  attack  leaves  one  in  doubt  as  to  which  group 
to  place  the  patient  in,  manic  excitement,  for  example,  being  asso- 
ciated with  certain  catatoniform  s^Tuptoms,  catatonic  excitement 
presenting  a  fairly  typical  flight  of  ideas  and  the  like.  In  general 
the  principle  of  difterentiation  is  first,  the  historv  of  previous  attacks, 
and  np\'t  to  this  history  of  repeated  atjtacksjsahistory  of  attacks  of 
both  manic  and  depressive  character  occurring  m  the  individual  and 
showing  no  markedtendency  to  deterioratioiLu  Practically,  however, 
it  IS  quite  impossible  to  make  a  differentiation  in  many  cases  and 
patients  that  are  at  one  time  placed  in  one  group,  for  example,  in  the 
manic-depressive  group,  are  found  later  to  probably  belong  in  the 
precox  group,  because,  for  example,  of  the  appearance  of  deterioration. 
And  so  the  history  of  the  diagnosis  in  these  two  groups  shows  a  con- 
stant tendency  to  fluctuation,  at  one  time  the  dementia  precox  group 
bemg  enhanced  by  cases  which  at  another  time  are  placed  in  the 
manic-depressive  group.  And  so  the  situation  fluctuates  back  and 
forth,  the  best  evidence  of  an  inherent  relationship  between  the  two. 
The  reasons  for  this  state  of  affairs  have  been  set  forth  in  the  section 
on  the  nature  of  the  psychosis  while  a  consideration  of  the  extraversion 
features  of  the  reaction  as  against  the  introverted  features  of  the 
precox  t>T)e  will  materially  aid  in  the  understanding  of  the  meanings 
of  the  s^Tnptoms. 

Themost  important  group  from  the  practical  standpoint  is  the 
^ycTotli^Tnicy  These  patients  are  practically  always  wrongly  diag- 
nosed  aFfirst  and  often  over  and  over  again  for  considerable  periods 
of  time.  Most  generally,  as  already  mentioned,  they  fall  into  the 
hands  of  the  specialist  under  the  theory  of  some  disorder  of  the  inter- 
nal organs.  When  an  attempt  is  made  to  really  understand  these 
patients  one  is  impressed  with  their  close  resemblance  to  the  obses- 
sional neurosis.  It  may  be  quite  impossible,  at  least  by  any  means 
other  than  a  very  careful  and  detailed  study  of  the  patient,  to  differ- 
entiate between  the  two  conditions  and  from  the  discussion  on  the 
nature  of  the  manic-depressive  psychosis  it  will  be  seen  why  this 
similarity  exists.  It  should  be  borne  in  mind  also  that  it  is  not  diffi- 
cult to  confuse  mild  cycloth\Tnic  attacks  with  the  anxiety  neurosis. 

The  relation  to  compulsion  neurosis  has  already  been  mentioned  in 
discussing  the  nature  of  the  psychosis. 

It  should,  of  course,  be  added  that  one  must  be  careful  and  not 
confuse  excitements  and  depressions  that  may  have  other  origins  as, 
for  example,  particularly  paresis,  the  symptomatic  and  toxic  psychoses 
and  the  more  clearly  psychogenic  states  such  as  prison  psychosis— the 
so-called  situation  psychoses. 

Treatment. — There  has  been  the  general  feeling  in  years  past  about 
this  psychosis  that  the  attacks  were  self-limited.  This  has  probably 
been  dependent  to  no  small  extent  at  least  upon  the  extreme  regularity 
of  the  attacks  in  certain  patients.    These  patients,  however,  are  the 


TREATMENT  849 

patients  with  the  more  profound  constitutional  taints  and  it  is  perhaps 
generally  true  that  in  this  class  of  patients  attacks  do  tend  to  run  a 
regular  course,  each  attack  being  approximately  of  the  same  duration 
as  the  former  similar  one.  With  the  more  frankly  reactive  types, 
however,  this  becomes  progressively  less  true,  so  that  the  matter  of 
treatment  here  easily  becomes  something  more  than  mere  intelligent 
custodial  care. 

In  the  ver\'  mild  types  of  the  disease  the  patients  have  to  be 
:;garefiill\'  safeguanie^,  because  their  condition  is  not  appreciated  by 
others.     In  the  excited  phases  alcoholic  and  sexual  excesses  are  espe- 
cially apt  to  occur,  and  it  must  never  be  lost  sight  of  that  during  what 
outwardly  appear  to  be  the  mildest  depressions  suicicfe  is  a  possibility. 

In  the  more  pronounced  attacks  the  handling  of  the  patient  calls 
for  the  very  greatest  amount  of  tact.  During  the  excitement  the 
patient's  strength  must  be  guarded,  as  insomnia  is  a  constant  s\Tiip- 
tom  and  food  may  be  taken  in  insufficient  quantities.  Mechanical 
restraint  should,  of  course,  be  avoided~if  possible  and  it  may  be  said 
that  it  is  practically  never  necessary.  Its  application  in  the  high 
degrees  of  excitement  is  often  not  understood  by  the  patient,  and 
produces  an  increase  of  excitement  and  resistance  and  perhaps  a 
state  of  anxious  apprehension  and  even  terror.  Chemical  restraint 
b  equally  undesirable  as  it  tends  to  shut  out  the  real  world  and 
thereby  increase  the  difficulty  of  adjustment  to  reality.  H\'pnotics 
may  be  necessary  and  such  simple  ones  as  veronal  are  the  best — 
paraldehyde,  trional,  sulphonal,  chloralamid  are  useful  but  opiiun  and 
its  derivatives  should  be  avoided  if  possible.  Whereas  the  various 
kinds  of  restraint  are  highly  undesirable,  isolation  may  be  resorted 
to  and  IS  not  nifrequently  welcomed  by  the  patient.  All  that  may  be 
necessary  is  simply  to  put  the  patient  in  a  room  by  himself  without 
locking  the  door.  He  may  be  very  glad  to  stay  there  and  so  escape 
from  outside  sources  of  irritation. 

It  is  in  the  condition  of  excitement  that  the  continuous  bathisso 
valuable.  The  patient  is  placed  in  a  tub,  preferably  one  constructed 
especially  for  the  purpose,  being  long  enough  to  permit  the  body  to 
lie  in  it  without  the  limbs  being  flexed.  The  water,  which  should 
cover  the  body  completely,  is  kept  at  a  temperature  of  from  96°  to  98" 
F.,  that  is,  just  above  the  normal  surface  temperature.  It  adds  to  the 
comfort  of  the  patient  if  a  canvas  hammock  can  be  slung  in  the  tub 
on  which  he  can  lie  and  a  rubber  air  pillow  be  placed  under  his  head. 
The  warm  water  of  the  continuous  bath  is  the  best  sedative  treatment 
we  have  for  this  class  of  patients,  as  it  produces  sedation  without  any 
clouding  of  consciousness  or  other  disagreeable  features.  The  patient 
may  be  left  in  the  tub  for  such  a  period  of  time  as  is  deemed  necessary, 
usually  three  or  four  hours  at  a  time.  On  the  Continent  patients 
are  not  infrequently  kept  in  the  tub  not  only  for  days,  but  even 
months,  sleeping  in  the  tub  and  being  fed  in  it.  On  the  whole  the 
patients  enjoy  this,  the  warm  water  is  soothing  and  they  are  grateful 
for  its  calming  influence. 
.54 


850  MANIC-DEPRESSIVE  PSYCHOSES 

It  is  in  the  excited  phases  that  the  nurse's  ingenuity  will  be  taxed 
to  the  utmost  and  if  she  is  not  tactful  all  sorts  of  artificial  symptoms 
will  be  created  in  the  way  of  antagonisms  toward  the  nurse,  increased 
irritability,  etc.,  so  that  the  adjustment  of  the  nurse  to  the  patient, 
particularly  in  excited  conditions,  becomes  an  important  practical 
problem. 

In  the  depressed  phase  of  the  disease  the  patient  is  often  best  treated 
in  bed,  particularly  if  the  depression  approaches  the  stuporous  stage. 
Under  these  circumstances  refusal  of  food  is  a  common  symptom  and 
tube  feeding  must  be  resorted  to  at  regular  intervals.  With  the  tube 
feeding  it  is  easy  to  give  such  medicines  as  may  be  required,  either 
hypnotics,  cathartics  or  anything  else  indicated. 

One  must  remember  that  in  endeavoring  to  probe  the  consciousness 
of  depressed  patients  the  depression  may  be  increased  and  if  suicidal 
tendencies  are  present  this  should  be  borne  in  mind  and  guarded 
against. 

If  the  patients  are  stuporous  the  usual  precaution  should  be  taken 
to  see  that  the  position  of  the  body  is  changed  from  time  to  time  and 
that  the  bladder  and  rectum  are  not  permitted  to  become  overloaded. 
The  danger  of  suicide  in  depressed  conditions  is  an  ever-present  one. 
The  only  safe  way  to  deal  with  these  patients  is  to  assume  that  they 
are  all  suicidal.  Probably  one  of  the  reasons  why  more  patients  in 
in  this  condition  do  not  commit  suicide  is  because  of  the  marked 
retardation  which  makes  it  so  difficult  for  them  to  initiate  any  form 
of  activity.  It  is  the  depressed  patients  who  are  suicidal  who  most 
frequently  require  to  be  sent  to  a  hospital,  although  their  general 
condition  may  not  seem  to  warrant  such  a  move.  The  watching  and 
the  caring  for  depressed  patients  with  a  view  to  preventing  them  from 
committing  suicide  is  practically  only  understood  in  institutions  for 
the  treatment  of  mental  disease.  The  general  hospital  nurse,  the 
general  practitioner  and  the  family  rarely  have  any  idea  of  the  degree 
of  watchfulness  that  is  necessary  and  for  this  reason  alone  oftentimes 
the  patients  must  be  sent  to  institutions. 

During  the  period  of  convalescence,  occupation,  outdoor  exercise 
and  the  like  are  all  in  order.  Care  should  be  taken  not  to  force  the 
patient  too  fast. 

Here  as  elsewhere  in  mental  medicine  an  attempt  should  be  made  to 
analyze  the  mind  sufficiently  at  least  to  understand  the  nature  of  the 
disturbing  factors  that  are  at  work,  and  if  possible  the  way  in  which 
they  have  brought  about  the  psychosis.  This,  of  course,  is  essential 
to  an  intelligent  treatment  of  the  patient.  Such  analysis,  however,  is 
almost  impossible  with  many  patients,  particularly  during  the  attack, 
and  can  only  be  resorted  to  when  the  patient  is  at  least  approaching 
the  normal  condition.  All  the  information  gained,  however,  is  valuable 
as  pointing  the  way  toward  regulating  the  patient's  life  and  in  many 
instances  as  indicating  the  nature  of  the  etiological  factors  and  thereby 
showing  what  must  be  avoided  in  the  future  if  further  attacks  are  to 
be  prevented.     Of  course  much  more  is  to  be  hoped  for  in  the  frank 


PROPHYLAXIS  851 

reactive  than  in  the  profoundly  constitutional  types.  An  adequate 
psychoanalysis,  however,  affords  the  best  prospects  for  ultimate' 
recovery  ^perhaps  furthered  by  an  endocrine  therapy  calculated  to" 
relieve  the  "glandular  imbalances  which  may  be  operating  to  prevent 
a  synthesis  of  the  personality  at  a  sufficiently  high  level  of"  efficiency. 
"  Psy choanal Y2is)^hould,  however,  be  used  with  great  care  as  the  uncov- 
ering  of  the  complexes  may  have  the  initial  effect  of  plunging  the  patient 
into  a  profound  depression  in  which  suicide  should  always  be  con- 
sidered as  a  possibility.  Such  patients  should,  therefore,  be  in  espe- 
cially protected  situations  during  such  a  crisis.  The  best  time  to 
undertake  an  analysis  is  during  the  interval  between  attacks  or  during 
a  mild  hypomanic  period  when  the  patient  is  inclined  to  be  communi- 
cative and  the  repressions  are  not  so  much  in  evidence. 

Prophylaxis. — Prophylaxis  resolves  itself  into  two  parts:  (1)  the 
prevention  of  the  disease,  and  (2)  the  prevention  of  subsequent 
attacks  after  the  disease  has  manifested  itself.  The  prevention  of 
the  disease  is  perhaps  a  problem  of  eugenics,  but  of  equal  or  greater 
importance  a  problem  in  education;  the  adequate  unfolding  of  the 
developing  personality  in  relation  to  its  problems.  Very  much  more 
information  is  needed  as  to  the  way  in  which  the  manic-depressive 
psychosis  conducts  itself  with  reference  to  the  laws  of  heredity.  It 
has  not  as  yet  been  adequately  worked  out  so  that  definite  advice  is 
possible. 

As  to  the  prevention  of  subsequent  attacks  the  most  important 
thing  is  to  prevent  if  possible  the  recurrence  of  the  etiological  factors 
that  have  been  found  to  play  a  part  by  the  psychoanalytic  study 
of  the  patient  and  the  reeducation  and  reorientation  of  the  patient 
toward  his  problems  of  adaptation. 


CHAPTER  XVII. 
THE  PARANOIA  GROUP. 

Perhaps  no  term  in  psychiatry  has  undergone  wider  variations 
of  meaning  than  the  term  paranoia.  In  its  earhest  days,  in  the  Greek 
period,  it  meant  little  more  than  just  craziness,  although  perhaps 
it  may  have  been  used  somewhat  more  specifically  in  some  instances, 
and  later  on,  well  into  the  middle  ages,  it  was  still  a  term  that  was 
not  infrequently  used  to  include  the  whole  group  of  the  so-called 
insanities. 

The  term^  did  not  come  into  general  use  as  applied  to  a  special 
grouping  of  mental  symptoms  until  the  early  part  of  the  nineteenth 
century,  when  a  German  psychiatrist,  Heinroth,  made  an  effort  to 
classify  various  mental  diseases  and  gave  paranoia  a  distinct  place- 
ment in  his  scheme.  The  classifications  of  this  day,  however,  were 
extremely  complex  and  there  was  a  marked  tendency  to  follow  the 
dichotomous  method  with  its  binomial  nomenclature,  which  had  come 
into  such  popular  vogue  with  the  appearance  of  the  work  of  the  great 
Swedish  botanist,  Linnaeus,  in  the  middle  of  the  eighteenth  century. 
A  disease  that  was  classified  under  the  specific  names  of  hallucinatory, 
confusional,  depressed,  or  what  not,  might  change  its  name  and  its 
nature  overnight,  as  it  were.  This  led  to  great  confusion  and  to  the 
final  throwing  out  of  the  whole  scheme  by  the  French,  under  the 
leadership  of  Pinel,  who  reduced  the  classification  to  manias,  melan- 
cholias, and  dementias.  Esquirol  followed  with  his  monomania,  under 
which  the  paranoias  found  a  place,  and  this  term  has  been  in  use 
ever  since,  largely  by  the  English  school,  and  it  still  finds  application 
in  the  courts.  It  is  based  upon  the  simplistic  conception  that  the 
brain  is  one  organ  and  that  it  has  one  disease,  and  that  disease  is 
insanity,  and  not  only  simplistic  to  this  extent,  but  that  the  disease 
may  affect  any  part  of  the  organ  and  therefore  a  person  may  be  insane 
upon  one  subject,  conceptions  which  are  hardly  worthy  of  a  school 
boy,  but  yet  are  still  held  in  some  quarters  today. 

From  this  time  on  the  general  concept  of  paranoia  became  some- 
what more  definite  and  it  tended  more  and  more  to  concentrate  and 
^Tyc+gTMvf  itgplf  about ~a"coiiditJim  which  presented  essentiallv  delu- 
sions, more  or  less  clearly  formed  and  of  a  persecutory  type_,_genera11y 
associated_with  hallucinations^  especially^  auditorv.  Even  this  con- 
c^pt,  liowever,  included  such  a  mass  oi  material  of  such  dissimilar 
types  that  it  never  became  very  well  defined.    The  paranoia  contro- 

1  Jelliffe:  Study   of  the   Origin,   Transformations   and   Present   Day   Trends  of  the 
Paranoia  Concept,  Jour.  Nerv.  and  Ment.  Dis.,  1913. 
(852) 


THE  PARANOIA  GROUP  853 

versy  during  this  period,  namely  from  the  middle  to  the  end  of  the 
nineteenth  century,  is  largely  taken  up  with  a  discussion  upon  the 
basis  of  the  old  faculty  psychology,  and  the  division  of  the  mind  into 
three  parts,  the  intellect,  the  emotions,  and  the  will.  There  had 
already  been  a  tendency  to  consider  paranoia  as  a  primary  intellec- 
tual disorder.  The  discussion  took  up  the  question  as  to  whether 
the  emotions  were  involved  primarily,  secondarily,  or  at  all.  This 
whole  bootless  procedure,  basfd  upon  the  faculty  psychology,  of 
com-se  came  to  naught,  simply  because  the  mind  is  not  split  up  into 
mutually  exclusive  compartments.  Another  one  of  the  concepts  upon 
which  such  discussions  were  based,  and  which  was  assumed  in  the  dis- 
cussion, was  that  there  were  such  things  as  mental  disease  entities 
w^hich  had  as  much  individuality  and  definiteness  in  the  conception 
of  the  psychiatrist  as  tumors  had  in  the  mind  of  the  pathologist. 

^Yithout  going  into  a  description  of  the  different  ideas  of  paranoia 
which  have  been  extant,  and  which  in  their  later  development  will 
be  described  in  the  body  of  the  chapter,  it  need  only  be  added  that 
the  general  result  of  all  this  discussion  is  first  that  the  brain  is  not  a 
single  organ.  It  is  a  great  number  of  organs  crowded  into  a  very  close 
space  and  the  functions  of  its  different  parts  need  be  no  more  closely 
related  to  one  another  than  the  functions  of  the  adrenal  glands  and 
the  hypophysis.  The  cortex  alone  consists  of  at  least  fifty  histologi- 
cally differentiated  organs,  while  the  thalamus  is  composed  of  at 
least  nine  ganglia.  The  red  nucleus  is  an  organ  by  itself,  as  are  the 
different  portions  of  the  lenticular  nucleus,  and  the  separate  ganglia 
innervating  the  ocular  muscles.  In  some  way  or  other  there  issues 
from  this  complex  of  organs,  or  more  properly  is  associated  with  it, 
the  phenomenon  of  mind.  ]\Iind  is  not  a  single  thing  any  more  than 
is  the  brain.  It  is  not  only  as  complex  as  the  organ  v.hich  subserves 
its  function,  but  infinitely  more  complex  than  this  organ  as  it  is  known 
today.  The  mind  caimot  be  conceived  as  divided  into  compart- 
ments like  the  will,  the  intellect,  and  the  feelings,  each  presided  over 
by  a  mythological  demon,  so  to  speak,  but  must  be  conceived  of  as  a 
complex  of  adaptive  mechanisms  interrelated  with  one  another  in  the 
most  intricate  manner,  so  that  the  miid  must  be  conceived  of  as 
capable  of  having  not  only  one,  but  many  kinds  of  disorders,  which 
disorders  are  not  entities  in  the  sense  of  foreign  bodies  or  diseases 
which  enter  from  outside,  but  are  inefficient  ways  of  functioning, 
special  combinations  of  mechanisms,  and  so  there  are  not  so  many 
mental  diseases  after  all  as  tj-pes  of  mental  reaction.  The  disease  is 
not,  therefore,  something  which  comes  from  without,  but  it  is  a  func- 
tion of  the  interrelation  between  the  individual  and  his  environment, 
and  only  in  proportion  as  this  interrelation  is  inefficient  may  it  be 
conceived  of  as  disease,  and  only  in  accordance  with  the  type  of 
mechanism  which  is  utilized,  the  special  trend  of  reaction,  can  a 
disease  be  spoken  of  in  any  specific  sense  at  all.  This  is  quite  parallel 
with  the  concepts  on  the  physical  side  and  is  important  to  bear  in 
mhul  if  one  is  not  to  be  enslaved  by  a  limiting  terminology. 


854  THE  PARANOIA   GROUP 

The  most,  recent  advances,  therefore,  hi  the  concept  of  paranoia 
are,  a  getting  away  from  the  consideration  of  it  as  a  disease  entity, 
or  as  involving  a  special  faculty  of  the  mind,  or  as  a  merely  mono- 
symptomatic  classification,  and  a  coming  to  consider  it  as  a  type  of 
reaction  which  manifests  itself  in  certain  individuals.  The  descriptive 
attitude  toward  the  problem  is  being  replaced  by  the  interpretative. 
This  is  significant,  as  paranoia  has  long  been  the  stronghold  for  descrip- 
tive psychiatry  and  it  has  been  the  last  to  yield  to  anything  like  an 
interpretative  approach. 

Description. — The  general  concept  of  paranoia  which  has  been  preva- 
lent for  many  years  is  that  of  a  ps^xhosis  presenting  delusions  of  per- 
secution of  a  prettv  clearlv  defined  type,  well  supported  and_dffended .. 
bythe  patient,  in  other  words,  systemati7,ed.  These  delusions  generally 
mvolve  a  more  or  less  circumscribed  portion  of  the  mentality,  although 
they  tend  to  spread  out  slowly  and  involve  more  and  more.  With  this 
statej^f  mind^here  isjio  marked  tendencv  toward  deterioration t_tne 
disease  having  essentially  a  chronic  course  and  remaining  unchanged 
for  years.  Associated  with  the  delusions  and  harmonized  with  them 
in^oirtent  are  frequently  auditofy~hallucinations — voices. 

This  is  the  general  concept  of  the  disease  which  has  received  various 
modifications  and  descriptive  clothings  by  different  authors.  For 
many  years  the  delire  chronique  a  evolution  systematique  of  Magnan 
in  France  and  the  paranoia  of  Krafft-Ebing  in  Germany  have  been 
the  paradigma  under  which  the  various  forms  have  been  arranged. 
The  delire  chronique  of  Magnan  was  a  disease  which  progressed 
regularly  through  four  stages:  first,  a  hypochrondriacal  stage,  or  stage 
of  subjective  analysis;  second,  a  stage  of  persecution;  third,  a  stage  of 
transformation  of  the  personality;  and  sometimes,  fourth,  a  stage  of 
deterioration. 

In_th£_first  or  the  hypochondriacal  stage,  or  stage  of  subjective 
an"alysis,  the  patient  is  self -centered  and  depressed  and  has  ideas  of 
reference.  He  also  complains  of  many  physical  symptoms,  such  as 
dizziness,  weakness,  headaches,  etc.  Everything  that  happens  about 
him  tends  to  be  referred  to  himself,  so  that  he  is  in  a  constant  state 
of  morbid  introspection  about  things  which  he  does  not  understand. 
In  the  second  stage  the  explanation  of  all  these  things  finds  itself  in 
the  delusions  of  persecution.  The  reasons  why  people  have  slighted 
him,  why  they  have  said  disagreeable  things  about  him,  why  they 
talk  about  him  and  spread  rumors  about  him  is  all  understandable 
because  of  the  conspiracy  which  there  is  against  him.  These  delu- 
sional ideas  are  reenlorced  by  hallucinations  of  hearmg,  and  he  hears 
actual  evidences  of  all  of  the  things  which  are  being  done  to  annoy, 
to  persecute,  or  to  destroy  him.  Explanatory  delusions  follow  which 
give  the  reasons  to  the  patient  why  he  is  thus  persecuted,  and  usually 
attribute  the  persecution  to  some  special  person  or  group  of  persons, 
or  some  society  or  institution.  In  this  persecutory  period  the  patient 
may  be  very  dangerous.  He  may  react  upon  his  persecutors  if  he 
knows  who  they  are,  that  is,  if  he  has  defined  them  in  his  own  mind. 


DESCRIPTION    .  855 

On  the  other  hand,  he  may  develop  elaborate  defenses  against  them  or 
may  endeavor  to  flee  from  them  entirely.  Later  on,  as  a  result  of  the 
elaboration  of  the  whole  system  with  the  effort  at  finding  further 
explanations  for  them  all,  there  are  developed  the  ideas  of  self-impor- 
tance.  The  patient  concludes  that  if  so  many  people  are  leagued 
against  himThat~he  must  indeed~t)e  an  important  personage^  When 
this  occurs  the  third  phase  oft  he  disorder,  nameR',  the  transforma- 
tion of  the  personality,  has  developed.  By  this  time  the  delusional 
system  has  become  extremely  complex,  with  interminable  ramifica- 
tions in  all  directions  involving  all  sorts  of  occurrences,  all  manner  of 
people,  and  reaching  backward  into  the  past  and  finding  delusional 
explanations  of  events  even  in  the  patient's  childhood.  These  expla- 
nations often  refer  to  facts  which  antedate  the  period  of  the  psychosis, 
and  are  therefore  known  as  retrospective  falsifications.  Following 
the  transformation  of  the  personality  there  may  occur  a  certain  amount 
of  mental  enfeeblement. 

This,  in  a  few  words,  is  Magnan's  delire  chronique,  a  disease  which 
is  extremely  rare  if  this  gradual  evolution  of  the  several  stages  described 
by  him  is  looked  for;  that  it  does  occur  from  time  to  time  cannot  be 
disputed.  In  all  probability  a  number  of  cases  of  paranoid  dementia 
precox  were  included  in  this  group,  while  today  the  group  has  been 
recognized  in  a  much  restricted  sense  by  Kraepelin^  in  his  paraphre- 
nias. 

KJrafft-Ebing,  in  Germany,  developed  a  picture  of  the  disease  which 
for  many  years  had  much  vogue  in  this  country,  and  found  particular 
favor,  largely,  probably  because  of  its  easy  applicability.     In  the 
first  place  he  divided  all  paranoias  into  two  great  groups,  th^^rmnat) 
and  th^^^aafuired^    The  original  paranoias,  after  Sander,  werethose_ 
which  presented  their'psychosis  asjhejiatural  uSoTdTng  of  analmor- 
maJ-chaTracterTwhile'the  acquired  or  late  paranoias  were  the^paranoias 
A\^hich  developed  in' p"eopIe~who  up  to  a  certain  point  had  appeared^ 
normal.     A  further  subdivisioii  of  these  groups  was  into  paranoia 
hallucinatoria,  in  which  hallucinations  played  a  prominent  part,  and 
paranoia  combinatoria,  in  which  they  were  absent  or  of  minor  impor- 
tance.    Following  these  great   groups  there  were  secondary  groups 
which  stood  upon  a  purely  s\Tnptomatic  basis,  and  so  he  described 
persecutory  paranoia,  expansive  paranoia,  querulous  or  litigious  para- 
noia, and  inventive,  reformatory,  religious,  and  erotic  types. 

More  recently  there  has  been  a  tendency  to  gradually  restrict  the 
paranoia  group  to  narrower  and  narrower  limits.  One  of  the  most 
recent  attempts  at  more  clear  definition  is  that  of  the  French  authors 
Serieux  and  Capgras,^  who  have  described  two  types  of  psychosis 
to  which  alone  they  think  the  term  paranoia  applicable,  namely,  the 
delirium  of  interpretation  and  the  delirium  of  renndlcatiou.  In  the 
delirium  ■  of  interpretation  the  patient   has  ideas  of  reference,   and 

1  Psychiatric,  8te  Auflage. 

«  Les  folies  raisonnantes,  Paris,  1909;  and  Diagnostic  du  d6lire  d'interpretation, 
Revue  de  Psychiatric,  January,  1908. 


856  THE  PARANOIA  GROUP 

because  of  his  lack  of  critique  and  egocentricity  comes  to  all  sorts  of 
false  interpretations  of  what  is  going  on  about  him.  These  delusional 
interpretations  become  systematized  and  reach  more  or  less  coherency 
without  any  special  dependence  upon  disorders  of  the  sensorium. 
There  is  no  deterioration,  and  lucidity  is  maintained  throughout  the 
evolution  of  the  psychosis.  Unlike  certain  other  paranoid  conditions 
the  false  interpretations  have  their  origin  in  actual  facts. 

In  the  delirium  of  revindication  a  chronic  systematized  psychosis 
which  takes  its  origin  in  a  fixed  idea  appears.  It  is  a  monoideism,  and 
its  various  ramifications,  like  the  other  form  of  paranoia,  do  not  tend 
toward  dementia.  They  descibe  two  varieties  of  this  psychosis,  the 
egocentric  type,  the  subjects  of  which  are  usually  persecutors  making 
claims  for  wrongs  sufliered  that  may  or  may  not  have  some  founda- 
tion in  fact.  Then  there  is  the  altruistic  type,  characterized  by 
abstractions  and  impersonal  theories.  To  this  group  belong  the 
inventors,  the  reformers,  and  the  prophets,  becoming,  however,  in 
their  endeavor  to  realize  their  ideals,  oftentimes  dangerous  fanatics, 
mystics,  anarchists,  regicides. 

In  Germany  Kraepelin  limited  the  paranoia  concept  perhaps  more 
than  anyone  else.  He  confined  the  term  to  a  very  circumscribed 
and  very  small  group.  His  conception  of  the  disease  is  a  chronic 
incurable  psychosis  of  insidious  origin  developing  slowly  by  the 
gradual  systematizing  of  endogenous  delusions.  This  system  of 
delusions  is  enduring  and  unshakable  and  exists  along  with  the  reten- 
tion of  the  logical  and  orderly  process  of  thinking.  There  is  no  marked 
tendency  to  mental  deterioration,  and  hallucinations  play  no  essential 
part  in  the  ])icture. 

Kraepelin  has  recently,  in  the  eighth  edition  of  his  work,  still  more 
clearly  defined  his  paranoid  group  by  describing  a  group,  paraphrenia, 
which  contains  certain  paranoid  types  that  closely  resemble  his  para- 
noia, but  which  provisionally  he  includes  in  this  group  for  purposes 
of  greater  definition.  This  group  of  paraphrenia  is  divided  into  four 
subgroups,  as  follows : 

Paraphrenia  systematica,  which  is  for  the  most  part  Magnan's  delire 
chronique,  with  the  exception  that  the  well-defined  precox  types  with 
marked  deteriorations  are  excluded. 

Paraphrenia  expansiva.- — This  form  affects  only  women  and  is 
marked  by  the  development  of  ideas  of  grandeur  with  mild  excitement 
and  exaltation.    No  dementia  follows. 

Paraphrenia  confabidans. — Here  the  delusions  both  of  persecution 
and  grandeur  are  specially  marked  by  their  foundation  upon  and 
reference  to  memory  falsification,  as  the  name  indicates. 

Paraphrenia  phantastica  is  the  term  applied  to  cases  with  a  certain 
amount  of  exaltation,  with  the  recounting  of  remarkable  adventures 
and  incoherent  changing  delusions.  This  condition  is  characteristic- 
ally accompanied  by  hallucinations  of  hearing.  This  group  includes 
the  cases  previously  described  by  him  under  the  term  dementia 
paranoides. 


INTERPRETATION  857 

Interpretation. — The  rather  simpHstic  attitude  which  dominated 
shortly  after  the  term  paranoia  came  into  general  use  and  which  saw 
in  every  combination  of  fairly  well  defined  and  fixed  persecutory 
ideas,  especially  those  supported  by  hallucinations  of  hearing,  the 
disease  paranoia  had  to  give  way  in  a  very  few  years  to  a  broader, 
if  somewhat  less  well  defined,  attitude.  It  soon  became  evident  that  a 
fairly  systematized  and  fixed  delusional  system  of  persecutory  charac- 
ter might  occur  a?  the  expression  of  a  psychosis  from  which  recovery 
took  place.  And  so  the  element  of  the  concept  of  paranoia  which 
considered  it  as  essentially  chronic,  progressive,  and  incurable  had  to 
be  readjusted.  These  so-called  acute  paranoias  have  been  recently 
studied  quite  extensively  by  Friedmann/  and  their  origin  traced  to 
actual  situations  in  the  patient's  life,  so  that  the  delusions  appear  as 
logical  outgrowths  of  experience,  and  have  as  a  consequence  fallen 
into  the  group  of  the  psychogenic  psychoses. 

Not  only  was  the  idea  of  chronicity  associated  with  paranoia  seriously 
shaken,  but  from  other  sources  the  idea  of  the  specificity  of  the  per- 
secutors' delusion  also  had  to  give  way,  for  it  was  soon  found  that 
ideas  of  persecution  of  paranoiac  character  were  not  at  all  infrequent 
in  connection  with  other  psychoses.  This  was  particularly  evident  in 
the  psychoses  of  chronic  alcoholism.  It  soon  developed  that  there 
was  a  special  form  of  dementia  precox  presenting  paranoid  ideas, 
while  later  studies  showed  luetic  forms  with  paranoid  symptomatology, 
presenile  forms,  paranoid  states  of  mind  of  the  deaf,  and  others  who 
are  isolated  from  close  contact  with  the  world,  to  say  nothing  of  the 
recent  paraphrenia  group  of  Kraepelin  and  many  other  less  well 
defined  conditions,  which  have  included  more  recently  not  only  the 
manic-depressive  psychosis,  but  certain  of  the  milder  cyclothymic 
manifestations  of  this  disorder. 

From  these  considerations  it  appears  that  here,  as  elsewhere  in  the 
field  of  psychiatry,  the  important  thing  to  consider  is  not  so  much 
the  special  content  of  the  particular  psychosis  in  a  given  individual 
as  the  mechanisms  which  are  involved,  for  here  is  seen  a  similar  con- 
tent in  all  sorts  of  mental  disorders,  some  acute,  some  chronic,  and  we 
are  therefore  forced  to  look  beneath  and  see  whether  it  is  not  possible 
to  understand  these  manifestations  by  attributing  them  to  a  common 
mechanism. 

The  studies  of  Friedmann,  already  mentioned,  went  a  long  way 
tow^ard  showing  the  dependence  of  paranoid  trends  upon  actual  situa- 
tions in  the  patient's  life,  and  demonstrated  how  paranoid  delusions 
in  given  cases  might  grow  as  a  result  from  these  situations.  In  other 
words,  they  are  of  psychogenic  origin  and  are  perfectly  understand- 
able when  all  of  the  circumstances  have  been  uncovered. 

Among  others,   Gierlich^   has   shown    that    paranoid    ideas    often 

'  Contributions  to  the  Study  of  Paranoia,  in  Studies  in  Paranoia,  Nervous  and  Mental 
Disease  Monograph  Series,  No.  2. 

-  Periodic  Paranoia  and  the  Origin  of  Paranoid  Delusions,  in  Studies  in  Paranoia, 
Nervous  and  Mental  Disease  Monograph  Series,  No.  2. 


THE  PARANOIA  GROUP 

accompany  fluctuations  of  afl:'ect  which  could  only  be  considered  as 
manifestations  of  a  manic-depressive  psychosis  and  that  many  of  the 
paranoid  conditions  which  were  associated  with  only  slight  affect 
manifestations  which  belonged  to  the  manic-depressive  psychosis 
might  easily  be  overlooked  as  coming  under  that  group  and  be  mis- 
taken for  true  paranoia.  In  this  way  he  accounted  for  a  very  large 
number  at  least  of  the  so-called  acute  paranoias,  as  these  patients 
of  course  got  well  from  the  attacks  as  the  manic-depressive  cases 
usually  do. 

More  recently  Specht^  has  at  great  length  endeavored  to  demon- 
strate that  the  underlying  condition  in  paranoia  was  an  affect  of 
suspiciousness  and  therefore  he  brought  the  paranoid  group  into  close 
alliance  with  the  great  affect  group  of  the  psychoses,  namely,  the 
manic-depressi\'e  group.  This  whole  discussion  has  broadened  out 
in  all  directions  and  has  become  very  complex  and  extremely  involved, 
and  therefore  it  is  not  a  proper  subject  for  further  elaboration  in  a 
text-b.ook.  It  might  be  added,  however,  that  Bleuler,^  who  has  made 
a  most  incisive  study  of  the  psychology  of  paranoia,  denies  absolutely 
that  suspiciousness  is  an  affect  at  all,  and  therefore  departs  radically 
from  Specht's  position.  He  believes  suspiciousness  is  a  state  of  mind 
based  entirely  upon  perceptions  and  the  resulting  conclusions,  and  is 
therefore  of  .purely  intellectual  origin,  but  that  it  is  accompanied  by 
affect,  as  are  all  mental  states.  Here,  again,  the  fallaciousness  of  the 
old  faculty  psychology  that  would  separate  the  mind  into  different 
parts,  such  as  the  intellect  and  the  emotions,  should  be  emphasized. 
The  two  invariably  occur  together,  and  suspiciousness,  of  course, 
therefore  is  accompanied  by  its  affect. 

Bleuler  is  of  the  opinion  that  paranoia  takes  its  origin  in  certain 
constellations  of  ideas  or  complexes  and  the  dominant  affect  with 
which  they  are  loaded,  that  these  complexes  are  precisely  of  the  same 
nature  as  are  found  in  healthy  individuals,  and  that  the  disease  ele- 
ment which  leads  to  the  elaboration  of  a  psychosis  is  the  fixation  upon 
this  complex,  the  inability  to  get  away  from  it,  or  as  might  be  said, 
the  inability'  to  reach  an  efficient  adjustment  to  it. 

One  here  sees  what  is  everyw^here  apparent  in  dealing  with  mental 
disorders,  that  the  delusion  is  not  the  disease,  the  delusion  is  only 
one  expression  of  the  disease.  The  mechanism  involved  has  to  deal 
with  a  certain  content;  this  content  is  delusional,  but  is  therefore 
only  the  outward  expression  of  the  disorder  beneath.  Therefore  the 
delusion  really  expresses  an  effort  upon  the  part  of  the  individual  to 
reach  an  efficient  adjustment.  The  constellation  of  ideas  with  its 
dominant,  painful  affect  has  been  one  to  which  the  patient  could  not 
eft'ectively  relate  himself,  and  therefore  the  next  best  thing  had  to 
be  done,  and  this  next  best  thing  was  the  formation  of  certain  delu- 
sions which  rendered  the  existence  of  the  painfully  affective  complex 

1  Ueber  den  pathologischen  Affekt  in  der  chronischen  Paranoia.  Cited  by  Bleuler, 
in  Affecti\'ity,  Suggestibility,  Paranoia,  New  York  State  Hospitals  Bulletin,  vol.  iv. 

2  Affectivity,  Suggestibility,  Paranoia,  New  York  State  Hospitals  Bulletin,  vol.  iv, 
February  15,  1912. 


INTERPRETATION  859 

more  endurable.  The  delusion,  therefore,  speakmg  m  physical  terms, 
is  more  comparable  to  scar  tissue  than  to  disease  tissue.  It  represents 
the  location  of  the  wound  and  the  result  of  the  reparative  process. 
To  make  the  matter  more  clear,  an  individual  who  is  ambitious,  and 
yet  who  lacks  ability,  may  develop  the  delusion  that  his  lack  of  suc- 
cess is  due,  not  to  his  lack  of  ability,  which  he  persistently  refuses  to 
see,  but  to  the  interference  of  enemies  who  are  jealous  of  him  and  who 
persecute  him  and  try  to  belittle  him  in  the  eyes  of  his  superiors.  In 
this  way  an  inacceptable  fact — his  mefficiency  to  which  he  cannot 
make  adequate  adjustment — is  so  distorted  that  it  would  appear  that 
the  results  of  this  inefficiency  emanate  not  from  within,  but  from 
interferences  from  without  (projection).  This  illustration  shows  well 
the  simultaneous  presence  of  both  delusions  of  persecution  and  delu- 
sions of  grandeur.  And  so  the  patient  creates  a  situation  in  which 
he,  so  to  speak,  finds  himself  able  to  get  along,  for  as  painful  as  a 
system  of  persecution  of  the  sort  which  he  creates  for  himself  may 
be  to  him,  it  is  less  so  than  a  realization  of  his  o\\ai  mherent  defects. 
It  will  be  seen,  therefore,  what  is  meant  when  it  is  said  that  the  delu- 
sion, speaking  in  physical  terms,  represents  scar  tissue,  and  it  will 
be  seen  also  how  a  destruction  of  the  delusion  could  in  no  way  cure 
the  disease.  If  the  delusion  could  actually  be  destroyed  the  patient 
would  be  in  much  the  same  position  as  a  patient  who  had  a  scar 
cut  out;  another  delusion  would  have  to  take  its  place,  because  the 
patient  w^ould  be  thrown  back  upon  the  same  inacceptable  situation 
to  which  he  would  find  himself  again  incapable  of  making  efficient 
adaptation. 

Another  mechanism  which  is  revealed  in  this  illustration  is  of  great 
importance  as  being  characteristic  of  the  paranoid  reaction  t^'pe, 
namely,  ^tlTe,  mechanism  of  projectigpi,  wherebX-l^lg- "^dividual  jpip- 
jpctR^^_ag  it  wprp^  impu  the  outside  world  his  owTt  mental  difficulties 
wli^h_return  to  him  in  this  instance  in  the  form  of  persecutions.  This 
mecHamsm  of  projection  is  a  very  common  one  and  appears  to  be 
fundamental  in  paranoid  trends.  It  is  at  the  basis  also  of  the  ideas 
of  grandeur.  Here  the  patient  projects,  not  his  ditficulties,_but  his 
ambitions,  and  his  hopes  comj_ba£k_to_hinljEraiD_llie__Quter  world^ 
realized.  In  fact,  the  mechanism  of  projection  is  not  a  pathological 
mechanism  at  all  but  is  constantly  used  by  healthy  persons.  This 
is  true  of  all  the  mechanisms  found  at  work  in  the  psychoses,  it  is  not 
the  mechanism  that  is  wrong,  it  is  the  failure  of  the  mechanism  to 
effect  adequate  adjustment  which  brings  the  individual  to  grief.  Ideas 
of  grandeur  are  always  present.  Persecutory  and  grandiose  ideas  are 
theopposite  sides  of  the  same  mechanism^  Either  may  dommate  the 
picture  but  both  are  always  present. 

The  most  elaborate  attempt  at  interpretation  of  the  paranoia  syn- 
drome was  made  recently  by  Freud^  in  his  analysis  of  the  Schreber 

1  Psj'cho-analytische  Bemerkungen  iiber  einen  autobioghraphisch  beschriebenen  Fall 
von  Paranoia  (Dementia  paranoides),  Jahrbuch  fiir  psycho-analytische  unci  psycho- 
pathologische  Forschungen,  Bd.  iii,  1911.  A  very  excellent  and  full  account  in  English 
of  Freud's  analysis  of  this  case  can  be  found  in  the  Psychoanalytic  Review,  vol.  i,  No.  1. 


860  THE  PARANOIA   GROUP 

case.  Herein  Freud  voiced  the  view  that  paranoia  was  dependent 
upon  a  homosexual  fixation  in  the  psychosexual  development  of  the 
individual.  To  make  this  statement  somewhat  clearer  it  should  be 
recalled  that  the  individual  in  his  psychosexual  development  is  first 
auto-erotic,  that  is,  interested  only  in  his  own  body,  that  his  next 
interest  is  in  those  immediately  about  him,  the  members  of  his  own 
family,  and  particularly  those  of  his  own  sex,  in  other  words,  those 
who  have  bodies  most  like  his  own,  and  that  these  stages  have  to  be 
passed  through  before  the  normal  end-result  in  a  heterosexual  love 
object  is  attained.  The  unconscious  auto-erotic  interest  in  his  own  body 
or  someone  like  himself  (homosexual)  is  designated  as  narcissism,  and 
the  paranoiac  mechanism  is  dependent  upon  a  fixation  and  develop- 
ment at  this  period.  In  the  normal  development  of  the  individual  the 
unconscious  homosexual  tendencies  are  not  entirely  eliminated  by  any 
means,  but  the  homosexual  libido  is  sublimated,  that  is,  its  energies  are 
utilized  in  other  channels,  more  particularly  it  is  utilized  in  all  those 
forms  of  association  with  the  same  sex  that  one  sees  in  friendships, 
social  organizations,  clubs,  games,  and  in  the  higher  social  activities. 
But  with  a  fixation-point  at  the  narcissistic  homosexual  period  of  psycho- 
sexual development  the  patient  is  constantly  in  danger.  Any  serious 
conflict  is  liable  to  cause  a  regression  of  the  sublimation  to  the  point 
of  fixation,  and  this  is  considered  by  Freud  to  be  the  mechanism  at  the 
basis  of  paranoia.  "  Persons  who  cannot  rise  completely  out  of  the  stage 
of  narcissism  and  are  thus  prematurely  fixed  or  arrested  in  the  evolution 
of  their  dispositions,  are  exposed  to  the  danger  that  a  flood  of  libido- 
which  finds  no  outlet,  sexualizes  their  social  tendencies  and  reverts  the 
sublimations  achieved  in  the  course  of  development."  The  libido  of 
the  paranoiac  is  then  projected  upon  those  about  him. 

The  whole  process  is  briefly  and  ingeniously  set  forth  by  Freud  by 
means  of  ringing  the  changes — supposing  the  paranoiac  to  be  a  male 
— upon  the  basal  sentence  "I  love  him,"  thus: 

Delusions  of  yersecution  contradict  the  verb.  "I  love  him"  is 
resented  by  the  individual  who  reacts  to  the  feeling  by  "I  do  not 
love — I  rather  hate  him."  Then  this  feeling  of  hate  is  projected  with 
the  result  "he  hates  (persecutes)  me,  which  justifies  my  hating  him." 
As  a  result,  this  feeling,  appearing  to  come  from  an  outer  perception 
becomes  "  I  really  do  not  love  him — I  hate  him — because  he  persecutes 
me." 

Erotomania  contradicts  the  object.  "I  do  not  love  him — I  love 
her,"  then  "I  notice  that  she  loves  me,"  then  finally,  "I  do  not  love 
him — I  love  her — because  she  loves  me." 

Delusions  of  jealousy  contradict  the  subject.  "  Not,  I  love  the  man 
— she  loves  him." 

Delusions  of  grandeur  result  from  a  total  contradiction,  a  rejection 
of  the  whole  sentence.  "I  do  not  love  at  all,"  and  hence,  "I  love 
nobody."  As  the  libido  must  be  accounted  for,  this  is  equivalent  to 
"I  love  only  myself." 

This  mechanism  is  an  excellent  illustration  of  the  necessity  for 


TREATMENT  861 

never  forgetting  that  the  symptoms  are  the  creation  of  the  patient  and 
not  of  his  environment.  The  paranoiac  tries  by  the  nicest  subtleties 
to  prove  that  the  trouble  is  not  with  himself  but  is  due  to  outside 
circumstances,  unfortunate  occurrences,  the  machinations  of  enemies, 
and_he  succeeds  onl>-  too  often  m  convmcmg  others_that  this  i^^the 
fact,  The  s\Tnptoms  can  only  be  understood  when  the  alleged  disturb- 
mg  factors,  the  persecutors,  for  example,  are  understood  to  be  but 
symbols  of  one  aspect  of  his  psyche,  the  regressive  aspect  which  really 
embodies  the  energy  which  is  working  to  his  detriment.  In  hysteria 
an  analogous  situation  is  produced  by  a  projection,  so  to  speak,  of  the 
patients'  difficulties  upon  their  own  body.     (See  Hysteria.) 

From  this  point  of  view  paranoia,  and  paranoid  types  of  reaction, 
are  problems  resulting  from  an  inadequate  sublimation  at  the  homo- 
sexual stage  of  psychosexual  development.  The  problem  of  paranoia  is 
therefore  but  one  aspect  of  the  much  larger  problem  of  homosexuality 
the  fundamental  nature  of  which  we  are  still  far  from  understanding. 
Using  the  terminology  of  Ferenczi^  LazelP  believes  that  the  male, 
persecuted  paranoid  is  a  subject  homo-erotic.  That  is,  he  is  a  subject, 
passive,  feminine  type  of  homosexual  and  tlie  persecutions  are  sym- 
bolized sexual  aggressions. 

Diagnosis. — Attention  has  already  been  called  in  the  body  of  this 
chapter  to  the  different  conditions  which  have  to  be  borne  in  mind 
in  making  a  diagnosis.  There  are  many  paranoid  states,  and  wherever 
the  paranoid  mechanism  is  present  then  it  is  proper  to  speak  of  a  para- 
noid state.  These  paranoid  states  are  found  in  many  of  the  psychoses. 
They  may  be  more  or  less  permanently  associated  with  the  special 
attack,  as  in  the  manic-depressive  psychosis,  or  as  in  alcoholic  hallu- 
cinosis, or  there  may  be  transient  episodes,  as  in  general  paresis.  It 
will  be  seen,  therefore,  that  there  are  many  and  various  types  of  symp- 
tomatic paranoid  states  and  that  perhaps  the  main  consideration  in 
the  matter  of  diagnosis  is  that  a  condition  which  is  symptomatic  and 
transitory  should  not  be  mistaken  for  a  chronic,  progressive,  and 
probably  irrecoverable  psychQsis.  This  differentiation  cannot  always 
be  made  on  the  basis  of  a  cross-section,  but  the  patient  must  be  studied 
carefully  over  a  considerable  period  of  time,  and  a  reasonably  full 
history  antedating  the  period  at  which  he  came  under  observation 
must  also  be  had  in  order  to  see  what  the  general  progress  indicates. 
A  psychoanalytic  investigation  of  the  symbolic  meanings  of  the 
delusional  ideas  is  essential. 

Treatment. — For  a  considerable  time  past  the  general  attitude  toAvard 
the  group  of  cases  included  under  the  designation  of  i)aran()ia  has 
been  that  they  were  incurable.  The  outlook  has  been  an  extremely 
dark  and  pessimistic  one,  and  correspondingly  therapeutic  efforts 
have  been  paralyzed  at  their  very  inception.  A  somewhat  changed 
attitude  toward  the  whole  group  was  the  natural  result  of  the  develop- 

'  Contributions  to  Psychoanalysis,  Tr.  by  Jones.    Pub.  by  Richard  G.  Badger,  Bostor, 
1916. 
2  Personal  communication. 


862  THE  PARANOIA   GROUP 

ment  of  a  concept  of  paranoia  which  was  more  circumscribed  and 
apphcable  to  a  more  hmited  number  of  patients,  and  when  corre- 
spondingly it  was  learned  that  there  were  many  paranoid  states 
associated  with  essentially  recoverable  psychoses;  in  other  words,  as 
the  paranoia  concept  has  become  more  and  more  contracted  it  has 
been  realized  that  a  great  many  of  the  paranoid  conditions,  which 
formerly  were  grouped  under  the  head  of  paranoia,  really  belonged  to 
recoverable  transient  conditions,  and  therefore  the  outlook  for  them 
was  good.  On  the^other  hand,_.as^lhe_Baranoia  concept  has  contracte,d 
it  cannot  be  said  that  there  has  been  any  increase,  at  leastjiiitiL:^£ry 
recently,  in  the  hopefulness  for  this  limited  groujToFcases.^ 

It  "could  hardly  be  expected  that~"ar~artiine ^^hen^  paranoia  was 
considered  to  be  an  absolutely  hopeless  and  irrecoverable  psychosis, 
chronic  and  progressive  in  its  very  nature,  that  therapeutic  results 
would  ofler  much,  or  that  there  would  be  found  many  who  would 
even  give  any  material  effort  in  this  direction,  but  here  and  there 
scattered  through  the  literature  are  reports  of  cases  of  paranoid  type 
which  seemed  to  have  been  influenced  by  this  or  that  form  of  thera- 
peutic procedure,  and  more  recently,  since  the  doors  have  been  opened 
and  one  has  been  able  to  enter  more  intimately  into  a  knowledge  of 
the  mechanisms  that  are  involved  in  the  development. of  the  psychoses 
and  when  it  has  been  seen  that  these  mechanisms  at  least  are  quite 
the  same  in  mental  disease  as  they  are  in  health  even,  and  that  the 
mechanisms  of  chronic  psychoses  are  quite  the  same  as  those  found 
in  recoverable  conditions  and  also  in  health,  and  that  the  diseased 
feature  was  not  so  much  the  mechanism  as  the  fixation  of  the  individ- 
ual at  certain  periods  of  development  or  with  reference  to  certain 
constellations  of  ideas,  it  became  at  once  an  open  question  whether 
these  conditions  might  not  be  susceptible  of  the  same  sort  of  modifi- 
cation as  they  are  in  less  serious  conditions.  And  so  within  the  past 
few  years  there  has  been  an  awakening  of  interest  in  these  chronic 
psychoses  and  efforts  are  being  made  here  and  there  to  penetrate  their 
mysteries  and  to  modify  their  course,  with  the  result  that  already  a 
number  of  cases  of  paranoia  have  been  reported  as  having  had  a 
favorable  outcome. 

The  general  prmciple  of  treatment,  at  the  psychological  level, 
resolves  itself  into  as  complete  as  possible  an  unravelling  of  the  tangled 
skein  of  the  patient's  mental  life,  an  uncovering  of  the  activating 
circumstances  in  his  career  which  have  been  the  etiological  factors  in 
the  development  of  the  psychosis,  and  by  so  doing  modifying  his 
mental  trends  by  a  progressive  process  of  readjustments  and  reeduca- 
tion. This  is  the  work  of  one  skilled  in  the  analysis  of  psychological 
situations  and  is  of  quite  the  same  nature  as  the  psychoanalytic  treat- 
ment of  the  neuroses. 

It  seems  certain,  from  the  results  of  dealing  with  these  paranoid 
conditions,  that,  aside  from  any  definite  ability  to  modify  the  course 

1  A.  Meyer:  Treatment  of  Paranoia  in  Modern  Treatment,  Nerv.  and  Ment.  Dis., 
"VVliite  and  Jelliffe,  Lea  &  Febigpr,  1913;  Bjerre;  Psychoanalysis,  Boston,  1917. 


TREATMENT  863 

of  the  psychosis  or  to  produce  a  definite  curative  result,  the  psycho- 
analytic method  of  attack  may,  not  infrequently  at  least,  lead  to 
a  certain  amount  of  transfer,  that  the  physician  may  come  to  be 
highly  respected  and  affectionately  regarded  by  the  patient  to  such 
an  extent  at  least  that  he  may  very  largely  control  the  patient's 
activities.  This  has  been  known  to  happen  under  rather  extraordinary 
circumstances,  showing  a  very  high  degree  of  personal  influence  by 
the  physician  over  the  patient,  despite  the  fact  of  well  marked  and 
fixed  delusional  beliefs. 

In  dealing  with  paranoiacs  it  must  always  be  remembered  that  one 
is  dealing  with  a  class  of  patients  who  are  potentiallv  dangerou^. 
thatjhTjihis  group  belong_^perhaps  the  most  dangerous  of  the  so-called 
jnsane^ and  aside  from  matters  of  psychoanahsis,  or  in  fact  of  any 
questions  of  therapeutic  endeavor,  it  must  be  realized  that  where  it 
is  evident  that  the  welfare  of  the  individual  and  the  welfare  of  society 
cross,  the  welfare  of  the  individual  must  give  way  in  favor  of  that  of 
society.  If  the  paranoiac  is  actually  dangerous  it  is  necessary  to 
intern  him  in  some  institution  where  he  will  get  proper  care. 

The  question  as  to  whether  a  given  paranoiac  is  dangerous  or  not, 
in  the  absence  of  any  overt  acts,  is  often  an  extremely  difficult  one 
to  decide.  In  any  case  it  is  a  question  to  be  decided  by  a  study  of 
the  individual  case  and  it  alwaj's  includes  a  consideration  of  many 
factors.  Among  these  factors  the  following  may  be  mentioned:  It 
is  important  to  find  out  how  completely  the  mentality  of  the  patient 
is  permeated  by  the  delusional  system,  in  other  words,  how  much  or 
how  little  freedom  he  has  from  delusional  control,  whether  all  of  his 
mental  forces,  so  ■*;o  speak,  go  to  reinforce  the  delusion,  or  whether, 
on  the  other  hand,  he  is  left  reasonably  free  for  a  considerable  portion 
of  the  time,  in  contact  with  reality,  rather  than  plunged  into  the  depths 
of  his  unreal  world.  It  is  important,  too,  to  note  how  clearly  defined 
may  be  his  belief  in  the  activity  of  any  specific  individual  in  his 
delusional  system,  whether  he  believes  some  person  who  is  living, 
perhaps  nearby,  someone  whom  he  can  easily  come  in  contact  with, 
is  responsible,  at  the  bottom  of  his  persecutions.  It  is  important  to 
see  whether  the  patient,  in  the  consideration  of  his  delusional  ideas, 
is  at  all  subject  to  the  reality  motive,  whether  he  has  any  critique  left, 
or  whether  his  belief  is  shakable  in  any  degree  by  others,  whether  he 
can  be  influenced  materially  by  his  physician  when  it  comes  to  the 
question  of  his  delusional  beliefs,  or  whether  they  dominate  the  situa- 
tion absolutely.  It  is  important  to  judge  the  general  attitude  and 
mood  of  the  individual,  whether  he  is  entirely  shut  out  from  any 
consideration  of  others,  of  the  world  at  large,  whether  he  considers 
himself  quite  a  law  unto  himself,  whether  he,  for  example,  is  exalted, 
egotistic,  beyond  criticism,  self-sufficient,  and  believes  that  anything 
that  he  may  decide  to  do  is  justifiable.  It  is  important  to  know 
whether  the  patient  in  his  past  career  has  been  impulsive,  whether  he 
has  shown  tendencies  to  do  unusual,  bizarre,  or  grotesque  things,  or 
to  fly  into  passions,  or  to  be  uncontrollable  from  slight,  inadequate,  or 


864  THE  PARANOIA   GROUP 

unexpected  reasons.  Threats  have  to  be  evaluated  and  an  opinion 
reached  as  to  whether  the  patient  really  means  to  carry  them  out,  or 
whether  they  are  used  as  a  means  of  emotional  catharsis.  The  general 
education,  bringing  up,  and  ideals  of  the  individual  are  important  as 
indicating  what  he  is  liable  to  do.  A  person  who  was  brought  up 
originally  with  a  proper  regard  for  the  proprieties,  who  is  essentially 
a  gentleman  or  a  gentlewoman,  is  by  that  very  token  not  so  apt  to 
commit  some  vulgar,  rowdy,  indecent  act. 

And  finally,  it  may  be  said  that  while  the  general  attitude  toward 
paranoia  has  perhaps  not  materially  changed,  while  there  is  still 
pretty  good  ground  for  believing  that  a  certain  class  to  which  the  term 
paranoia  is  perhaps  alone  applicable,  is  inaccessible  to  therapeutic 
endeavor  and  are  doomed  to  suffer  from  their  psychosis  throughout 
their  lives,  gtill  even  if  this  is  so  there  is  no  absolute  way  of  deter- 
mining this  fact  except  by  a  consistent  and  sufficiently  prolonged 
efi'ort  to  modify  the  course  of  the  disease,  and  with  the  several  cases 
already  in  the  literature  which  indicate  that  conditions  that  might 
well  have  been  considered  chronic  and  irrecoverable  if  taken  at  their 
face  value  can  still  be  materially  benefited  and  perhaps  cured,  no  one 
is  in  a  position  to  pass  final  sentence  upon  any  patient  after  an  exami- 
nation or  two,  but  on  the  contrary,  liasthe  right  to  feel  that  there  is 
some  hope  for  all  of  them  and  that  at  least  hope^hpuld  noL-be  ahan- 
doneJ^l^til  consistent  therapeutic  efforts  have  been  applied  for  a 
reasonable  time . ^ 

1  Consult  Critical  Digest  of  the  Paranoia  Problem,  by  C.  R.  Payne,  Psychoanalytic 
Review,  vol.  ii. 


CHAPTER   XVIII. 
EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION. 

Epilepsy,  the  "falling  sickness,"  has  been  known  from  the  earliest 
times,  the  very  word  itself  carrying  in  its  history  (it  is  derived  from  a 
Greek  verb  meaning  "to  seize  upon")  evidences  of  the  animistic 
hypotheses  of  earlier  and  relatively  more  primitive  ways  of  thinking. 

The  word  epilepsy  is  used  as  a  symbol  under  which  are  grouped  a 
great  variety  of  conditions  which  in  general  are  characterized  by  sud- 
den and  relatively  transient  attacks  involving  for  the  most  part  dis- 
turbances of  consciousness  ("faints,"  "absences,"  "blanks,"  amnesias) 
and  convulsive  seizures  invoh'ing  the  voluntary  and  involuntary 
musculatures.  Such  attacks  are  the  outward  manifestations  of  a  wide 
variety  of  conditions  ranging  all  the  way  from  the  so-called  functional 
neuroses  and  psychoneuroses  (hysteria,  compulsion  neurosis),  the  more 
frank  psychoses  (dementia  precox),  toxemic  states  (uremia,  alcohol), 
many  organic  diseases  (paresis,  cerebral  syphilis,  abscess,  softening 
and  tumors)  to  the  grosser  defects  of  development  (idiocy). 

The  natural  evolution  of  the  concept  symbolized  by  epilepsy  in  the 
recognition  that  similar  "seizures"  may  result  from  such  a  multiplicity 
of  conditions  has  resulted  in  a  tendency  to  speak  of  "the  epilepsies" 
rather  than  of  "an"  epilepsy  and  makes  it  worth  while  to  consider  the 
attack  as  due  to  a  faulty  distribution  of  energy  which  may  be  brought 
about  in  many  ways  and  through  divers  mechanisms.  The  wide 
variety  of  conditions,  as  a  part  of  which  convulsi^'e  reactions  with 
associated  disturbances  of  consciousness  occur,  cannot  be  too  much 
emphasized.  The  toxic  states  (endogenous  or  exogenous)  are  usually 
transitory  and  depend  upon  the  continuance  of  the  toxemia,  but  in 
defective  parathyroid  functioning  with  disordered  calcium  metabolism 
the  convulsive  phenomena  continue  because  the  underlying  metabolic 
disorder  cannot  be  permanently  relieved.  Marked  organic  changes 
which  are  responsible  for  convulsive  attacks  are  usually  cerebral 
(tumor,  softening,  hemorrhage,  meningitis),  but  certain  organic 
conditions  resident  elsewhere,  notably  the  cardi()})athy  of  Stokes- 
Adams  disease  and  the  condition  resulting  in  animals  from  thymus 
extirpation,  appear  to  be  sufficient  causes,  while  still  more  obscure 
factors  determine  vagal  and  vasovagal,  gastric  and  intestinal  attacks 
which  appear  to  be  dependent  upon  elements  of  constitutional  make-up 
at  the  level  of  the  \egetativc  ner\-ous  system.  It  is  worth  while,  there- 
fore, to  attempt  to  get  a  viewpoint  of  all  these  conditions  dependent 
upon  their  common  element— faulty  energy  distribution. 

For  the  purpose  of  comprehending  the  epileptic  phenomena  then, 
55  (865) 


866       EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

the  nervous  system  may  be  viewed  in  a  very  simple  way.  From 
the  standpoint  of  striictm-e  it  may  be  thought  of  as  consisting  of 
receiving  organs,  designed  either  to  come  in  contact  with  the  external 
world  (exteroceptors)  or  with  other  parts  of  the  body  (proprioceptors) 
spoken  of  collectively  as  receptors.  The  combined  material  accumu- 
lated through  these  receptors  forms  the  basis  upon  w4iich  certain 
extensions  of  the  nervous  system  (effectors)  are  devised,  whereby  the 
reactions  of  the  body  are  conditioned  in  a  way  to  bring  about  that 
adaptation  essential  to  life  or  to  the  maintenance  of  the  social  structure. 
In  this  way  the  nervous  system  is  viewed  as  a  mass  of  interrelated 
reflexes  redistributing  the  energy  received,  for  the  purposes  of  the 
organism. 


Fig.  433. — Diffuse  sclerosis  of  cortex  with  atrophy  along  the  Rolandic  and  Sylvian 

fissures  (epileptic). 

As  the  incoming  stimuli  are  multitudinous,  so  the  outgoing  activities 
are  correspondingly  diverse,  and  a  healthy  organism  is  able,  by  reason 
of  its  nervous  mechanisms,  so  to  distribute  the  energy  received  as  to 
bring  about  a  series  of  harmoniously  adjusted  activities,  be  they 
physicochemical,  sensorimotor,  or  psychic.  This  viewpoint,  that 
energy  distribution  takes  place  at  all  of  these  levels,  should  not  be 
lost  sight  of,  as  there  is  a  tendency  to  think  of  the  problem  solely  in 
terms  of  muscular  work.  Atwood's  "man  in  the  box"  broke  up  more 
nitrogen  compounds  during  mental  than  during  mechanical  work. 
This  breaking  up  of  nitrogen  compounds  is,  however,  only  one  form 
of  registering  the  energy  output. 

That  which  characterizes  a  well  adjusted  nervous  mechanism  is  its 
ability  to  properly  and  in  an  orderly  manner  distribute  its  energy. 


EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION       867 

but  that  which  characterizes  the  pictures  of  the  disorders  included 
in  this  chapter  is  inefficiency  in  this  regard  which  ma>'  be  more  espe- 
cially emphasized  at  the  physicochemical,  the  vital,  or  the  psychic 
levels. 

An  explanation  for  epileptic  attacks  which  finds  its  ultimate  expres- 
sion under  such  s\Tnbols  as  eye-strain,  floating  kidney,  gliosis,  or  like 
specific  indictments  fails  to  realize  that  the  nervous  system  contains 
representations  of  all  of  the  organs  and  that  the  final  activity  of  the 
human  body  is  the  result  of  the  balance  which  has  bean  struck  among 


Fig.  434. — Gross  anatomical  lesions  associated  with  epilepsy.  An  asymmetrical 
internal  hydrocephalus  which  also  showed  a  .small  tumor  at  the  lateral  border  ot  the 
cerebellum. 


innumerable  tendencies.  The  part  that  any  })articular  organ  ])lays 
can  only  be  understood  when  taken  in  consideration  with  the  organism 
in  its  totality  and  realizing  the  specific  part  that  the  organ  in  question 
plays  in  the  whole  problem. 

Bearing  in  mind  this  view  of  the  nervous  system,  as  a  great  mass  of 
complexly  interrelated  reflexes,  and  further,  the  law  of  avalanche 
(Cajal)  which  insures  the  continuous  breaking  up  of  the  original 
sensory  stimulus  into  an  c\'er-increasing  number  of  a\'enues  of  dis- 
charge, it  will  be  seen  how  many  ways  are  open  to  interfere  with  the 


868       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 


orderly  procession  of  energy  through  this  compHcated  series  of  reflex 
The  nature  of  the  epileptic  discharge,  essentially  a  manifesta- 


arcs. 


tion  of  energy  at  greatly  reduced  adaptive  efficiency,  and  the  destruc- 
ti^'e  character  of  certain  pathological  lesions  which  initiate  it  (impaired 
metabolism,  gross  destructions,  psychic  imbalances)  indicates  that  the 
essential  defect  is  not  irritant  but  destructive,  the  blocking  or  closing 
of  many  paths  of  outlet  structurally  or  by  inhibition,  and  so  accu- 
mulating the  discharge  within  relati^•ely  narrow  confines.  Such  a 
conception  would  apply  equally  well  to  the  "idiopathic"  or  "genuine 


Fig.  435. — Gross  anatomical  lesions  associated  with  epilepsy.  Cerebral  asymmetry 
and  ventricular  dilatation  following  an  early  meningitis.  The  dura  over  the  atrophic 
hemisphere  was  of  the  consistence  of  an  egg-shell.    Section  of  preceding  brain. 


epilepsy"  with  Ammon's  horn  gliosis  and  to  the  epilepsy  associated 
with  marked  developmental  defects  (idiocy)  in  which  it  may  be  con- 
ceived that  the  wider  paths  for  avalanche  flischarge  have  not  been 
laid  down.  This  viewpoint  is  also  consistent  with  the  different  levels 
at  which  the  discharge  may  take  place — psychic,  physiological,  and 
physicochemical — the  character  of  the  attack  as  limited  to  certain 
levels,  the  psychic  (hysteria,  dementia  precox),  the  physiological 
(Jacksonian  types),  the  physicochemical  (tetany);  the  distribution  of 
the  discharge,  general  attacks  ("genuine  epilepsy"),  localized  attacks 


EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION        869 

(Jacksonian  t.^iJes),  and  as  beinj;  initiated  by  sensory,  motor,  or 
psychic  prodromes.^ 

Symptom  Groups. — Con^•ulsions  may  occur,  as  already  indicated,  in 
a  great  variety  of  conditions  while  distinctly  explosive  attacks  not 
convulsive  in  the  sense  of  muscular  spasms,  occur  under  still  wider 
conditions  in  states  not  definitely  epileptic  but,  with  reference  to  the 
more  essential  epilepsies,  in  what  may  be  referred  to  as  borderland 
conditions. 

The  essential  epilepsies  have  been  divided  into  the  late  epilepsies 
occurring  relatively  late  in  life  and  dependent  upon  toxemias  and 
gross  organic  changes  and  the  early  epilepsies  which  occur  relatively 
early  in  life,  generally  before  or  during  adolescence. 


Fig.  4.36. — Gross  anatomical  lesion  associated  with  epilciisy:     Tuiimr. 

Epilepsies  of  dross  Brain  Disease. — These  occur  in  paresis,  cerebral 
sj'phil's,  brain  cysts  (echinococcus,  etc.),  hy(lrocei)halus,  the  cerebral 
meningitides  (.syphilitic,  tuberculous,  .serous,  and  pachymeningitis), 
bony  tumor  of  skull,  traumatisms  (fractures,  insolation,  concussions, 
hemorrhages),  multiple  sclerosis,  cerebral  sclerosis  and  gliosis,  cerebral 
arteriosclerosis,  the  encephalitides  and  myoclonias. 

The  Epilepsies  of  Toxic  and  Infections  Origin. — The  toxemias  include 
those  of  endogenous  origin  (uremia,  diabetes),  of  exogenous  origin 
(metallic — such  as  lead  and  arsenic,  and  strychnine,  alcohol,  and  carbon 
monoxide) . 

1  For  literature  from  1900  ti)  1910  see  Referate  by  Gruhle.  Ztschr.  f.  d.  Kesamtc 
Neurol,  u.  Psych.,  Band  ii,  Heft  1.     Binswanger:  Epilepsie. 


870       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

The  infections  are  more  especially  the  exanthemata,  influenza, 
rabies,  malaria,  rheumatism,  syphilis,  etc.,  operating  either  through 
the  mechanism  of  an  overwhelming  toxemia  or  by  meningitides  or 
encephalitides. 

Anomalous  and  Borderland  Conditions. — Here  are  included  certain 
internal  secretory  imbalances,  particularly  diseases  of  the  thymus, 
thyroid,  and  parathyroids  and  degeneratio-adiposo-genitalis. 


Fig.  437. 


-Gross  anatomical  lesions  associated  with  epilepsy. 
Marked  feeble-mindedness. 


Internal  hydrocephalus. 


Certain  high  level  attacks  occur  in  hysteria,  compulsion  and  anxiety 
neuroses,  and  in  dementia  precox,  especially  the  catatonic  form. 

Here  also  should  be  included  the  vagaP  and  vasovagal  attacks,  slight 
passing  disturbances  of  consciousness  associated  with  vertigo  and 
sometimes  sensory  disorders  as  loss  of  sight,  possibly  certain  sensory 
disorders  of  fulminating  character  such  as  migraine,  and  some  disturb- 
ances of  sleep  (narcolepsy)  disturbances  of  consciousness  of  syncopal 
nature,  and  the  aflect  epilepsies  of  the  Bratz  type. 


'  Wm.  R.  Gowers:  The  Borderland  of  Epilepsy,  Philadelphia,  1907. 


EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION       871 


Fig.  438. 


-Epileptic,    showing 
ej'ebrows  from  falls. 


scars   over 


Convulsive  attacks  may  be  combined  with  a  number  of  conditions. 
The  association  of  myoclonia  is  one  of  the  most  intimate — myoclonus 
epilepsy.  This  combination  is 
familial,  several  children  of  the 
same  family  suffering  and  some- 
times successive  generations  be- 
ing affected.  Here  are  epilep- 
tiform attacks,  particularly  at 
night,  and  myoclonic  contrac- 
tions affecting  especially  the 
muscles  of  the  tongue,  pharynx, 
and  diaphragm.  The  relation  of 
the  two  symptom  groups  is  not 
clear. 

Pathological  Groups.  —  The 
preceding  clinical  grouping  gives 
a  fair  idea  of  the  pathological 
conditions  which  may  be  found. 
The  following  grouping  is  given 
by  Alzheimer^  as  a  result  of  the 
histological  examinations  of  63 
cases.  These  simply  indicate 
the  reasons  why  and  how  the 
structure  of  the  brain  is  modi- 
fied, thus  changing  its  functional  capacity  as  an  energy  distributor. 

A.  Cases  with  very  obscure  etiology  (genuine  epilepsy): 

1.  This  group  comprises  60  per  cent,  of  the  cases: 

(a)  With  sclerotic  changes  in  Amnion's  horn. 
•     (b)  With  superficial  gliosis  of  the  hemispheres. 

(c)  With  signs  of  an  acute  process  (status)  besides  a 
and  h. 

2,  This  group  comprises  4  per  cent,  of  the  cases:  The  process 

in  these  cases  can  be  called  an  atrophic  sclerosis.  There 
is  considerable  atrophy  of  different  regions ;  no  important 
changes  in  the  vessels;  deep  impairment  of  the  nervous 
structures,  especially  the  third  cell  layer  of  the  cortex. 

B.  Cases  due  to  external  poisons: 

1.  Alcohol:  Different  anatomical  changes,  as  in  chronic  alco- 

holism. Besides  these  sometimes  acute  changes,  as  in 
delirium. 

2.  Lead :  Different  changes.     Experimentally  lead  produces  a 

genuine  encephalitis. 

C.  General  diseases: 

1.  Syphilis:  Different  forms  of  brain  sj'philis,  especially  the 

endarteritis  of  the  finer  vessels  (Xissl,  Alzheimer). 

2.  Arteriosclerosis. 

*  Alzheimer    and    Vogt:  Die    Gruppierung    der    Epilepsie,    Jahresversamniluiig    des 
deutschen  Vereins  fur  Psychiatrie,  1907;  Ref.  AUg.  Ztschr.  f.  Psych.,  Bund  Ixiv,  1907. 


872       EPILEPSY  AXD  CONVULSIVE  TYPES  OF  REACTION 

J).  Focal  diseases:  Most  of  the  cases  in  this  group  are  cases  of 

epilepsy  with  idiocy  after  encephalitis. 
E.  Arrests  of  de^•elopment : 

1.  Stadium  verrucosum  (Rancke). 

2.  Sclerosis  tuberosa. 

This  survey  will  suffice  to  show  what  a  wide  variety  of  conditions 
have  been  included  under  the  term  epilepsy  and  also  to  point  out  the 
various  groups  that  are  being  at  present  split  off  and  separately  iden- 


FiG.   439. — Epileptic,  .showing  scar  from  burn  due  to  injury  during  con\'nlsion. 

tified.  What  has  been  said  about  the  distribution  of  energy  applies 
to  the  broad  group  of  convulsive  reactions.  The  disease  "genuine  epi- 
lepsy" will  now  be  briefly  described,  although  it  is  extremely  difficult 
to  do  this  at  all  accurately  as  it  must  be  borne  in  mind  that  hereto- 
fore all  matters  of  description  and  questions,  such  as  those  of  heredity, 
are  with  practical  uniformity  considered  with  reference  to  "epilepsy," 
without  effort  at  discrimination,  much  as  is  the  case  with  the  corre- 
sponding conglomerate  "insanity." 


CLASSICAL  EPILEPSY. 

This  roughly  corresponds  to  the  group  "genuine  epilepsy"  of 
Alzheimer  and  includes  those  cases  which  are  found  to  have  Ammon's 
horn  and  cortical  sclerosis  although  there  are  cases  of  "genuine  epi- 
lepsy" which  do  not  give  these  findings. 

Heredity. — The  genuine  epileptic  usually  comes  from  a  badly 
tainted  stock.  Epilepsy  may  not  appear  in  the  ancestors  but  they 
and  often  the  collaterals  show  evidences  of  ill-defined  nervous  disorders 


CLASSICAL  EPILEPSY  873 

(according  to  Davenport  and  Weeks, ^  migraine,  chorea,  paralysis,  and 
extreme  nervousness).  Epilepsy  and  feeble-minded ness  show  a  great 
similarity  in  their  hereditary  reactions  and  both  appear  to  be  due  to 
a  defect  of  the  germ  plasm,  that  is,  they  are  both  recessives.  As 
should  be  expected,  therefore,  the  two  conditions  are  frequently 
found  associated.  This  is  also  of  significance  in  relation  to  what 
has  already  been  said  about  the  developmental  failure  to  lay  down  the 
paths  for  the  higher  avenues  of  sensory  avalanche. 

The  Epileptic  Constitution. — The  classical  epileptic  is  apt  to  be  morose, 
irritable,  suspicious,  and  hypochrondriacal.  He  is  quite  character- 
istically uin-eliable  and  with  it  all  frequently  presents  a  very  aggressive 
form  of  sentimental,  shallow  religiosity.  This  type  of  epileptic,  in 
general  then,  is  very  sensitive,  irritable  and  insincere.  He  is  ego- 
centric to  a  very  considerable  degree,  paying  great  attention  to  him- 
self, his  own  feelings,  his  state  of  health,  his  physical  comforts,  and  his 
immediate  surroundings.  His  interests  are  variable  and  he  presents 
light  variations  of  mood  with  perhaps  headache  and  a  tendency 
generally  to  hypochondriacal  fixations.  His  interests  all  tend  to  be 
concentrated  in  this  egocentric  constellation.  His  reactions  of  irri- 
tability and  unreasonableness  present  infantile  characteristics.  ISIany 
epileptics  are  feeble-minded  or  more  profoundly  defective,  and  rather 
in  conformity  with  this  frequent  finding  the  word  associations  from 
epileptics  have  close  analogies  to  the  word  associations  of  the  imbecile. 
In  addition  to  these  traits  of  character  these  epileptics  are  usually 
lazy,  frequently  they  lie  openly,  present  an  attitude  based  on  high 
moral  standards  of  great  respect  and  consideration  to  one's  face  and 
quite  the  opposite  when  one's  back  is  turned.  Their  general  health 
is  apt  to  be  good  and  they  often  have  enormous  appetites,  and  are 
especially  fond  of  proteids.  While  good-natured,  even-tempered, 
well-disposed  epileptics  exist  they  are  more  apt  to  be  most  difficult 
problems  to  get  along  with,  and  as  a  class  in  the  hospital  they  are 
extremely  difficult  to  care  for.  Passing  attacks  of  mental  disturbance 
occur  in  the  interparoxysmal  period  without  apparent  relation  to 
seizures.  Attacks  of  transitory  iU-hnmor,  according  to  Aschaft'enberg, 
occur  in  7<S  per  cent,  of  cases.  This  is  a  condition  of  irritability, 
unreasonableness,  sometimes  associated  with  delusions  and  hallucina- 
tions. The  patient  is  in  a  "  touch-me-not"  state  and  very  apt  to  get 
into  quarrels  or  make  attacks.  Rarely  the  disturbance  is  expansive 
in  type  and  in  these  cases  may  be  associated  with  religious  fervor. 

A  study  of  the  sexual  characteristics  of  epileptics  has  been  made 
by  Maeder.2  As  a  result,  he  finds  the  sexuality  of  the  ei)ilc])tic 
still  largely  undeveloped,  still  only  little  removed  from  the  infantile 
stage.  The  sexual  feelings  are  very  prominent  and  are  aroused  in 
many  ways:  autogenically,  constituting  auto-erotism,  and  giving  rise 

>  A  First  Study  of  Inhcritanfc  in  Epilepsy,  Journal  of  Nervous  and  Mental  Disease, 
1911,  vol.  xxxviii,  No.  11. 

-  Sexualitiit  und  Epilepsie,  .Jalirl).  f.  psychoaiialytisehe  u.  psydiopatholoj^ische  For- 
schuHKon,    1909. 


874       EPILEPSY  AND  CONVULSIVE   TYPES  OF  REACTION 

to  such  phenomena  as  masturbation,  and  by  stimuli  from  without, 
constituting  aUo-erotism,  which  gives  rise  to  a  normal  libido  reaction 
(heterosexuality),  homosexuality,  exhibitionism,  etc.  Maeder  uses  the 
term  polyvalent  to  describe  that  characteristic  of  the  epileptic  sexuality 
which  permits  it  to  be  aroused  by  many  kinds  of  excitants. 

The  epileptic  state  leads  in  a  certain  proportion  of  cases,  if  it  has 
begun  in  early  life,  to  conditions  of  feeble-mindedness,  imbecility,  and 
idiocy,  or,  depending  upon  the  same  causes,  is  associated  with  these 
conditions.  Epilepsy  tends,  in  many  cases,  to  produce  a  general  mental 
deterioration  {epileptic  dementia)  which  may  become  very  profound.* 

A  study  of  the  personality  of  epileptics  by  Clark^  has  led  him  to  the 
conclusion  that  this  general  type  of  character  as  described  above  is  a 
result  of  the  disease  and  not  a  precedent  condition. 

Scripture  and  Clark^  have  described  the  epileptic  voice  sign  and  found 
it  in  75  per  cent,  of  cases.  The  voice  has  been  studied  by  the  "air 
puff"  method  of  recording  on  the  kymograph.  A  measure  of  the  wave 
gives  the  rates  of  vibration.  A  line  connecting  the  tops  of  the  ordinates 
produces  the  "melody  plot."  Normally  each  vowel  has  a  rising  and 
falling  melody.  In  epilepsy  the  vowels  run  along  on  an  even  tone — 
"plateau  speech."    This  is  very  characteristic  and  easily  recognized. 

Muskens,  in  his  study  of  the  muscular  phenomena,  has  found 
fatigability  and  weakness  of  single  muscles  or  muscle  groups,  startings, 
shocks  and  cramp-like  contractions  especially  just  before  or  after  going 
to  sleep.  All  sorts  of  physical  disturbances  may  be  associated  with 
the  epileptic  state  which,  like  the  character,  show  infantile  character- 
istics. Epileptics  get  along  best  under  very  carefully  protected 
circumstances  and  they  are  especially  prone  to  suffer  from  a  change  in 
the  accustomed  routine,  a  change  of  diet  being  accompanied  by  excesses 
and  a  subsequent  gastro-intestinal  upset.^  As  the  disease  progresses 
and  becomes  chronic  and  confirmed  one  will  note  many  evidences  of 
disturbance  at  the  vegetative  nervous  level. 

The  Seizure. — The  classical  major  epileptic  attack  (grand  mal)  is 
sudden  m  onset,  often  preceded  by  a  warning — aura.  The  patient 
falls  and  the  attack  immediately  develops  into  a  tonic  spasm  with 
unconsciousness.  The  tonicity  is  replaced  in  a  few  moments  by  clonic 
spasms  which  gradually  subside.  There  is  then  often  a  short  period 
of  automatic  activity  followed  by  a  gradual  return  to  full  conscious- 
ness, or  the  patient  sinks  at  once  into  a  deep  sleep  from  which  he 
awakes  complaining  of  lameness  and  weakness  in  the  muscles  that 
were  convulsed,  and  perhaps  headache. 

The  attack  has  been  described  by  authors  in  great  detail,  but  is 
really  different  in  practically  each  case,  although  the  type  tends  to 
remain  the  same  in  each  patient. 

1  A  Personality  Study  of  the  Epileptic  Constitution,  Am.  Jour.  Med.  Sc,  November, 
1914. 

^  Researches  on  the  Epileptic  Voice,  Proc.  New  York  Neurol.  Soc,  November  12,  1907. 

'  MacCurdy,  John  T.:  A  Clinical  Study  of  Epileptic  Deterioration,  Psychiatric 
Bulletin,  April,  1916,  ix.  No.  2, 


CLASSICAL  EPILEPSY  S75 

The  anra  may  be  sensory,  motor,  or  psychic.  The  sensory  warnmgs 
may  occur  m  any  of  the  sensory  fields:  the  visual  (flashes  of  light, 
hallucinations),  the  olfactory  (odors,  usually  bad — uncinate  fits),  the 
auditory  (simple  sounds  or  hallucinatory  voices),- etc.  The  epigastric 
aura  is  most  common  and  consists  of  a  wide  variety  of  disagreeable 
sensations  in  the  epigastrium. 

The  muscles  first  involved  vary  greatly,  as  does  also  the  order  in 
which  they  are  involved — march  of  the  con\Tjlsion.  The  patient  falls 
at  the  beginning  of  the  spasm,  the  direction  of  the  fall  being  dependent 
upon  the  muscles  first  aft'ected.  In  a  few  moments  all  of  the  voluntary 
muscles  are  convulsed,  includmg  the  muscles  of  respiration,  producing 
cyanosis,  and  the  jaw  muscles,  resulting  in  biting  the  tongue.  With 
the  beginning  of  the  clonic  stage  frothy,  bloody  saliva  issues  from  the 
mouth  and  the  cyanosis  gradually  disappears  with  the  resumption  of 
respiration.  Urine  and  feces  may  be  passed  during  the  attack — usually 
only  the  former.  In  the  partial  or  incomplete  seizures — petit  mal — the 
convulsive  phenomena  are  much  milder  and  may  even  escape  observa- 
tion altogether,  while  the  disturbance  of  consciousness  is  shorter  in 
duration  and  less  profound.  The  patient  may  blanch,  become  confused 
for  a  few  moments,  perhaps  falter  in  what  he  is  doing,  or  fumble  for  a 
few  moments  about  his  clothing  in  a  dazed  fashion  and  then  go  on 
about  his  affairs  as  if  nothing  had  happened.  These  attacks  are  also 
often  preceded  by  an  aura. 

The  psychic  disturbances  associated  with  the  attack,  before  and 
after,  or  replacing  it  are  many  and  varied. 

In  a  great  many  epileptics  there  is  a  marked  disturbance  preceding 
the  convulsion,  sometimes  of  several  days'  duration,  and  those  who  are 
accustomed  to  the  patient  can  tell  that  a  fit  is  impending.  This  change 
manifests  itself  in  increased  irritability,  complaining,  sometin^es  by 
depression  or  dulness,  and  there  may  be  associated  disturbances  of  the 
sensorium,  hypochondriacal  complaints  and  hallucinations.  All  these 
conditions  are  commonly  promptly  relieved  by  the  fit. 

Immediately  afier  the  convulsion  there  is  often  a  temporary  condi- 
tion of  confusion.  The  patient  rises  clumsily,  looks  about  him  in  a 
bewildered  manner  and  often  does  some  semi-automatic  acts,  such 
as  taking  oft*  his  clothes.  Also  following  the  attack  a  transitory 
exhaustion  paralysis  in  the  overacting  muscles  makes  itself  ap])arent. 

Just  before,  or  more  commonly  after,  the  convulsion  a  condition  of 
active  excitement  may  occur  which  may  reach  the  stage  of  frenzy. 
In  this  state  the  patient  is  a  veritable  wild  man — epileptic  furor.  He 
is  liable  to  kill  anyone  who  approaches  or  even  himself.  Fortunately 
his  efforts  are  diffuse  and  not  coherently  directed.  During  this  attack, 
which  is  usually  brief,  he  has  to  be  restrained  and  at  the  enfl  is  quite 
completely  exhausted. 

An  attack  of  mental  disturbance  may  take  the  ])lacc  of  the  convulsion 
and  thus  become  an  epileptic  equivalent.  These  attacks  of  })!<ijchic 
epilepsy  frequently  take  the  form  of  so-called  epileptic  automatism  or 
epileptic  dream  states.    In  these  conditions  the  ])atient  ma^•  do  almost 


876       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

aiiytliino;  and  when  he  comes  to  himself"  he  hus  absohitely  no  recollec- 
tion of  what  has  happened.  Usually  the  attacks  are  of  short  duration 
and  the  acts  rather  simple — more  simple  than  in  the  dream  states 
of  alcohol  or  hysteria.  However,  they  may  last  for  days,  all  sorts  of 
things  may  be  done,  crimes  may  even  be  committed,  so  that  the  con- 
dition often  becomes  of  great  medicolegal  importance.  The  crimes  of 
violence  are  often  noted  for  their  ferocity  and  brutality. 

It  must  not  be  forgotten  that  these  states  may  be  associated  with  a 
seizure  that  was  so  slight  as  not  to  have  been  noticed.  Evidences  of 
such  a  seizure,  especially  in  medicolegal  cases,  should  always  be 
looked  for. 

Transitory  conditions  of  depression,  excitement,  confusion,  delirium 
and  stupor  may  develop  and  quite  characteristically  a  condition  of 
ecstasy  with  hallucinations.  The  patient  sees  the  gates  of  Heaven  open 
and  as  the  heavenly  hosts  appear  he  hears  himself  addressed  by  the 
voice  of  God. 

The  transitory  states  of  ill-humor,  as  described  by  Aschaffenberg 
in  the  interparoxysmal  state,  might  also  be  considered  as  psychic 
equivalents;  these  are  frequently  associated  with  drinking. 

Besides  these  conditions,  paranoid  psychic  states  are  quite  common, 
while  of  the  more  transitory  psychic  manifestations  fugues  are  frequent 
and  certain  types  of  dipsomania  appear. 

In  addition  to  the  symptoms  thus  far  indicated  various  observers 
have  found  evidences  of  an  altered  blood  picture  such  as  leukocytosis 
and  hypo-eosinophilia,  while  disorders  of  metabolism  with  hyper- 
toxicity  of  the  secretions,  has  long  been  adduced  as  proof  that  the 
manifestations  WTre  dependent  upon  chemical  poisons  due  to  faulty 
metabolism. 

Meaning  of  the  Attack. — Bearing  in  mind  what  has  already  been  said 
about  the  distribution  of  energy,  it  will  be  of  advantage  to  pursue  this 
line  of  thought  somewhat  further. 

Energy  flow  may  be  blocked,  dammed  up,  and  break  through  in 
diffuse  discharge  at  any  level — psychic,  sensorimotor,  or  physico- 
chemical.  This  being  so  it  would  be  expected,  as  is  the  case,  as  already 
indicated,  to  find  disturbances  at  each  of  these  levels.  In  the  classical 
epileptic  attack  all  these  levels  are  involved,  but  what  is  of  equal  or 
greater  significance  to  the  general  hypothesis  is  that  there  are  attacks 
practically  limited  to  one  level,  and  a  study  of  the  several  types  of 
convulsive  reaction  will  show  a  series  of  cases  reaching  through  all 
of  the  intermediate  stages  from  the  highest  to  the  lowest  instinctive 
levels. 

The  hysterical  convulsion  offers  an  example  of  a  high  level  convul- 
sive type  of  reaction.  This  seizure  is  admittedly  psychogenic  in  origin 
and  presents  the  picture  of  a  conversion  of  psychological  into  physio- 
logical symbols.  In  other  words,  the  patient  escapes  from  his  painful 
ideas  by  converting  them  into  physical  symptoms.     (See  Hysteria.) 

The  disturbance  of  consciousness  in  these  hysterical  attacks  is 
relatively  slight,  much  less  than  it  outwardly  appears  to  be,  while  the 


CLASSICAL  EPILEPSY  877 

whole  situation  is  quite  near  the  surface  and  with  very  little  effort 
can  be  brought  to  conscious  control. 

Next  lower  in  the  scale  of  levels  are  the  psychasthenic  convulsions 
of  Oppenheim  (compulsion  neurosis  type)  which  are  expressions  of  a 
more  severe  grade  of  neurosis  but  still  within  strictly  psychological 
levels. 

Then  comes  the  very  interesting  affect  epilepsies  of  Bratz  and  Leu- 
buscher.  These  are  distinctly  epileptoid  t\'pes  of  reaction  conditioned 
by  purely  psychological  situations.  Here  the  outward  semblance  to 
a  deeper  level  epilepsy  is  much  greater  but  the  situation  is  still  a 
psychological  one.  The  reaction  of  the  patient  here  is  to  conditions 
that  are  absolutely  intolerable  and  to  which  no  adjustment  is  possible, 
such  a  situation,  for  example,  as  a  young  man  has  to  confront  when  the 
key  is  turned  upon  him  and  he  is  called  upon  to  realize  that  he  is  in 
prison  with  a  life  sentence  to  face.  Urder  these  circumstances  the 
patient  may  become  a  veritable  "wild  man,"  beat  his  clenched  fists 
against  the  bars,  rush  aimlessly  about  destroying  clothes  and  bedding, 
and  beat  his  head  against  the  walls  in  ineffectual  attempts  at  self- 
destruction.  Hallucinatory  disturbances  may  accompany  these  attacks 
and  amnesia  follow  them,  though  consciousness  during  the  attack  is 
not  entirely  lost.  That  these  patients  are  much  more  seriously  bur- 
dened constitutionally  and  more  nearly  allied  to  "genuine  epilepsy" 
than  the  psychasthenic  types  of  Oppenheim  is  indicated  by  the  fact 
that  they  give  a  history  of  "fits"  in  childhood  while  ther psychasthenic 
types  show  tics,  phobias,  and  compulsions.^ 

In  the  classical  epileptic  seizure  the  greater  severity  and  seriousness 
of  the  attack  is  indicated  by  the  complete  loss  of  consciousness  and  the 
still  further  reduction  in  the  purposeful  and  coordinated  adjustment 
of  the  muscular  reactions.  These  have  now  become  utterly  disorgan- 
ized. The  attack  has  involved  far  more  than  the  psychological  levels 
and  included  the  sensorimotor  and,  as  indicated  by  the  toxicity  of 
the  excretions,  the  biochemical. 

The  low  instinctive  level  to  which  the  epileptic  is  reduced  by  his 
seizure  can  be  appreciated  by  observing  his  activities  as  he  is  "coming 
out"  of  the  attack.  His  respiration  is  at  first  distinctly  abdominal 
(infantile  type),  he  makes  characteristic  sucking  movements  with  his 
lips,  and  his  movements,  from  the  complete  disorganization  into  which 
they  have  been  thrown,  assume  at  first  an  aimless  fumbling  with  his 
clothes,  a  tentative  feeling  about  as  he  instinctively  tries  to  readjust 
himself  to  reality,  to  "find  himself"  again.  In  this  tentative  "feeling 
about"  he  repeats  in  a  few  minutes  the  process  of  rt'lating  himself  to 
reality  which  is  a  normal  period  of  development  in  the  ciiild. 

Ferenczi-  has  endea^•ored  to  classify  the  neuroses  with  reference  to 
the  stage  of  development  they  represent  and  in  accordance  with  this 
scheme  suggests  that  epilepsy  belongs  to  the  period  of  wisli-fulfilment 

'  See  Galsworthy's  drama,  Justice. 

*  Entwickluugsstufen  des  WirklichkeitsHiimes,  Inteniatiunule  Zlsclir.  f.  iirztliche 
Psychoanalyse,  1913,  i. 


878       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

by  means  of  incoordinate  movements.  It  is  known  how  some  children 
when  thwarted  will  cry  out,  thrash  about  and  sometimes  straighten 
out  rigidly,  "  lose  their  breath"  and  become  blue.  Attacks  of  "  temper" 
the  mother  calls  them.  Later  on  the  child  will  kick  the  chairs  and  tear 
up  its  books  under  similar  circumstances  while  regression  to  approxi- 
mately the  same  level  is  shown  when  an  adult  stamps  his  foot,  clenches 
his  fists,  grinds  his  teeth,  and  otherwise  shows  reactions  of  anger  which 
are  quite  ineffectual  to  effect  any  change  whatever  in  conditions.  The 
meaning  of  it  all  is  an  absolute  inability  to  accept  or  to  adjust  and  an 
equally  determined  attitude  that  it  is  not  so  because  it  just  cannot  be 
so.  An  eft'ort  to  force  circumstances  to  be  different  by  a  supreme  effort 
of  thinking  them  different  which  when  it  fails  results  in  a  flight  from 
the  whole  thing  into  the  rigidity  and  unconsciousness  of  the  epileptic 
seizure. 

The  extreme  egocentricity  of  the  epileptic,  his  great  failure  to  project 
his  interests  into  the  outer  world,  his  tendency,  therefore,  to  retreat 
further  and  further  from  reality  and  to  revive  earlier  ways  of  finding 
pleasure  result  in  a  profound  regression,  which,  in  the  unconsciousness 
of  the  fit,  reproduces  the  helplessness  of  the  child  in  utero  and  demands 
the  same  degree  of  absolutely  complete  care.  Clark,  who  calls  this  a 
state  of  metro-erotism,  has  brought  forward  an  abundance  of  material^ 
to  demonstrate  that  the  fit  has  a  psychological  setting. 

The  seriousness  of  the  classical  epileptic  convulsive  reaction  as 
compared,  for  example,  with  the  hysterical  type  of  convulsion  is  indi- 
cated by  its  involvements  of  the  so-called  partial  tendencies.  As  has 
been  indicated  in  the  discussion  of  hysteria  the  split  there  is  massive. 
A  convulsion  then,  for  example,  may  be  found  to  re'produce  a  struggle 
against  a  sexual  assault.  In  epilepsy,  on  the  contrary,  the  split  is  much 
more  profound  and  as  seen  by  the  symptoms,  involves  more  particu- 
larly the  muscle  erotic  plus  perhaps  the  respiratory  erotic  as  more  or 
less  isolated  rather  than  as  adequately  integrated  tendencies.  A  well 
developed  musculature  is  quite  common  with  typical  epileptics  as  is 
also  a  feeling  of  muscular  well-being.  Equally  characteristic  of  certain 
epileptics  is  a  marked  cruelty  (sadistic)  tendency.  All  these  charac- 
teristics indicate  a  deep-seated  disorder  of  the  personality  and  so 
account  for  the  difficulty  that  the  problem  of  epilepsy  presents  from  a 
therapeutic  standpoint. 

As  bearing  upon  the  importance  of  the  psychic  element  it  is  surprising 
to  note  how,  as  a  result  of  acute  questioning  in  intelligent  subjects, 
it  may  very  frequently  be  demonstrated  that  a  particular  fit  served 
the  purpose  of  the  patient  in  some  way,  by  enabling  him  to  escape 
from  some  responsibility,  avoid  some  necessity  for  adaptation.  The 
way  in  which  this  is  done,  by  a  return  to  a  condition  demanding  the 
sort  of  care  which  a  mother  gives  .her  baby,  and  the  assumption  of  the 
characteristics  of  infancy,  Clark^  illustrates  by  abundant  material,  as 

>  Clark,  L.  P.:  Clinical  Studies  in  Epilepsy,  Psychiatric  Bulletin,  January  and  April, 
1916,  ix,  Nos.  1  and  2. 
2  Loc.  cit. 


CLASSICAL  EPILEPSY  879 

for  example:  talking  baby  talk,  assumption  of  the  fetal  position, 
covering  the  head  with  the  bedclothes,  passing  of  urine  during  the 
attack,  are  all  infantile  tA-pes  of  conduct. 

In  the  course  of  the  introversion  of  the  libido,  until  the  fit  occurs, 
Clark,  in  a  recent  chart  for  graphic  recording  of  the  epileptic  reactions 
suggests  that  the  degrees  of  regression  are  indicated  as  follows :  spon- 
taneous interest,  directed  interest,  lethargy,  irritation,  anger,  elation, 
psychic  phenomena,  petit  mal,  grand  mal. 

This  way  of  viewing  the  epileptic  attack  is  warranted  because  it 
follows  along  a  path  that  proceeds  from  the  known  to  the  unknown. 
The  mechanism  at  the  higher  psychological  levels  can  be  worked  out, 
and  although  those  at  lower  levels  cannot,  it  would  appear  that  the 
two  extremes  are  connected  by  a  regular  series  of  intermediate  stages 
as  represented  in  types  of  cases.  It  can  only  be  assumed  that  the 
disorder  in  "genuine  epilepsy"  has  to  do  with  adjustments  at  deep 
instinctive  levels,  adjustments  that  are  profoundly  biological  in  char- 
acter and  that  the  conflict  may  almost  threaten  life  itself  in  order  to 
understand  the  severity-  and  seriousness  of  the  attack  as  a  reaction  to 
failure.  A  study  of  the  aura  as  a  clue  to  the  point  at  which  blocking 
of  energy  commences,  and  a  detailed  analysis  of  all  the  elements  of 
the  attack  plus  an  analysis  of  the  make-up  of  the  individual  in  the 
interparoxysmal  period  would  seem  to  offer  the  mode  of  approach  to 
a  further  understanding  of  the  mechanisms  in  individual  cases.  The 
depth  to  which  such  an  analysis  would  have  to  go  and  the  severity  of 
the  constitutional  burden  in  the  classical  types  of  the  disease  is  indi- 
cated, for  example,  by  the  prevalence  of  the  epigastric  aura  the  char- 
acteristics of  which  indicate  the  possibility,  at  least,  that  the  disorder 
reached  as  deep  as  the  vegetative  nervous  system  level. 

It  can  be  seen  from  this  discussion  why  the  epileptic,  burdened  by 
deeply  instinctive  defects  of  biological  adjustment,  should  deteriorate. 
It  is  also  consistent  with  this  view  that,  in  general,  this  should  not  be 
true  of  symptomatic  epilepsy.  A  localized  lesion  of  the  cortex,  for 
example,  may  produce  convulsive  reactions  in  well-defined  groups  of 
muscles  only  without  loss  of  consciousness  (Jacksonian  type).  Here 
there  is  no  defect  of  biological  adjustment  involving  the  individual  as 
such.  A  group  of  muscles  only  has  been  cut  off  from  effective  associa- 
tional  relationship  with  higher  levels  and  so  becomes  reduced  in  its 
possibilities  of  reaction  to  relatively  incoordinate,  automatic  and 
purposeless  types.  A  portion  only  of  the  machinery  has  been  damaged , 
the  individual  remains  otherwise  intact.  The  disorder  is  confined  to 
the  sensorimotor  level. ^ 

From  this  point  of  view  it  seems  that  the  toxicity  of  the  blood  and 
urine  is  only  an  outward  evidence  of  the  depth  of  the  disorder  rather 
than  an  indication  of  its  cause.  It  is  true  that  certain  toxic  substances 
do  produce  convulsions,  but  they  do  it  by  damaging  the  machinery 

>  See  Clark,  L.  P.:  Nature  and  Pathogenesis  of  Epilepsy,  New  York  Med.  Jour., 
February,  1915,  el  seq.,  for  a  complete  statement  of  this  viewpoint  with  numerous  clinical 
proofs  of  its  value. 


880       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

like  the  cortical  lesions  just  referred  to  and  the  seizures  cease  with 
the  removal  of  the  poison.  This  is  seen  in  severe  forms  of  infection, 
febrile  states  in  children,  alcohol,  uremia,  etc. 

It  is  interesting  and  significant  to  note,  in  this  connection,  that  the 
form  of  dementia  precox  in  which  convulsive  seizures  are  most  prone 
to  occur  is  the  catatonic.  In  the  other  forms  types  of  compromise 
formation  take  place  while  the  catatonic  endeavors  to  cut  out,  to 
enucleate,  so  to  speak,  a  certain  portion  of  the  psyche,  a  condition 
much  more  favorable  to  blocking. 

Varieties  of  Convulsive  Attack. — The  Jacksonian  type  has  already 
been  referred  to  as  has  also  the  distinction  between  the  grand  and 
petit  mal  attacks  and  various  equivalents  (larvated  or  masked  epilepsy). 

In  addition  to  isolated  seizures,  there  are  serial  attacks  in  which 
several  seizures  follow  each  other  at  relatively  short  intervals  and 
finally  status  epilepticvs  in  which  large  numbers  of  attacks  accumu- 
late, following  each  other  at  short  intervals  until  unconsciousness 
becomes  continuous,  the  attacks  then  merging  into  one  another — 
overlapping.  The  temperature  rises  in  this  condition,  life  is  threat- 
ened and  indeed  it  is  the  typical  mode  of  death  of  the  epileptic  and 
his  ever-present  danger.  Serial  attacks  are  usually  grand  mal,  but 
may  be  petit  mal,  while  status,  even  if  it  begins  as  petit  mal,  soon 
takes  on  the  seriousness  of  the  major  variety.  Psychic  seizures  may 
also  be  serial  and  status  attacks  are  possible  without  or  with  very 
minor  convulsive  manifestations.  Status  may,  and  frequently  does, 
develop  in  the  symptomatic  epilepsies  due  to  gross  lesions  of  the  brain 
as  well  as  forming  a  frequent  termination  of  the  partial  or  incomplete 
seizures  of  the  Jacksonian  type. 

Myodomis  ejniepsy  appears  to  be  an  association  of  myoclonia  and 
epilepsy.  It  would  seem  that  the  myoclonic  shocks,  however,  gradu- 
ally eventuated  by  a  process  of  summation  into  an  epileptic  seizure. 
Some  epileptics  have  myoclonic  shocks  between  their  attacks  which 
appear  to  be  forerunners  of  the  seizure  rather  than  true  forms  of 
myoclonia. 

Continiious  Epilepsy. — Similar  to  the  myoclonic  varieties  are  the 
polyclonia  epileptoides  continua  of  Choroschko  and  the  epilepsia 
corticalis  continua  of  Koshewnikow.  This  is  a  condition  of  continuous 
myocloniform  shocks  in  single  muscle  groups,  usually  unilateral  and 
without  loss  of  consciousness. 

Course  and  Prognosis. — Epilepsy  is  not  a  unitary  concept.  Numerous 
conditions  are  covered  under  the  name,  anatomical,  etiological  and 
symptomatic.  It  is  better,  therefore,  here  also,  to  speak  of  the  reaction 
type  as  manifested  clinically.  The  epileptic  type  of  reaction  tends  to 
cripple  the  individual  more  and  more.  One  can  only  become  efficient 
in  dealing  with  reality  by  constantly  keeping  in  practice,  as  it  were. 
To  withdraw  from  difficult  situations  means  less  ability  to  meet  the 
next  ])r()blem  that  arises.  The  attacks  therefore  tend  to  become 
more  frequent  and,  in  a  considerable  number  of  cases  to  end  in  that 
permanent  renunciation  to  efficient  adjustment  to  realit}' — dementia. 


CLASSICAL  EPILEPSY  881 

Diagnosis. — From  what  has  been  said  it  can  be  seen  that  epilepsy 
is  not  an  entity,  that  the  term  includes  a  great  multitude  of  widely 
different  conditions  and  that  the  problem  of  diagnosis  is  therefore 
the  problem  of  differentiating  the  particular  one  of  these  several 
possibilities  in  an  individual  case.  Diagnosis,  however,  does  not  mean 
giving  a  name  to  a  thing,  but  understanding  it.  Every  individual 
epileptic  should  be  accepted  as  an  individual  problem  and  although  it 
may  often  be  quite  impossible  to  fathom  the  mechanisms  involved,  it 
is  only  by  such  a  method  of  approach  that  anything  worth  while  can  be 
hoped  for  when  the  question  of  treatment  comes  to  be  considered. 

The  possibility  of  exclusively  nocturnal  attacks — nochirnal  epilepsy 
— should  be  borne  in  mind.  It  is  suspicious  if  the  patient  awakes 
tired  and  lame,  as  if  his  muscles  had  been  beaten,  particularly  if  he 
shows  conjunctival  ecchymoses,  a  wounded  tongue  and  flecks  of 
blood  on  the  pillow.  A  localized  muscular  weakness  that  passes  off 
promptly  would  add  certainty  to  the  diagnosis. 

Treatment. — The  only  efficient  prophylaxis  is  not  to  transmit  the 
defective  germ  plasm.  It  would  seem  that  in  the  purely  symptomatic 
epilepsies,  such  as  those  due  to  cortical  traumatism,  that  the  germ 
plasm  might  escape  indictment,  but  this  does  not  necessarily  follow. 
A  certain  proportion  of  these  cases  will  be  found  to  have  had  con- 
vulsions in  infancy  so  that  they  might  have  been  considered  as  poten- 
tial epileptics  predisposed  to  react  by  convulsion-producing  mechan- 
isms. This  possibility  is  emphasized  by  the  frequency  with  which 
the  sjTnptomatic  epilepsies  develop  status  attacks. 

Treatment  of  the  Attach. — Once  the  attack  has  started  it  is  essential 
to  so  care  for  the  patient  during  his  period  of  helplessness  that  he  may 
not  be  injured  in  any  way.  If  he  has  fallen  in  a  safe  place  he  may  be 
allowed  to  remain  there,  perhaps  only  removing  him  from  proximity 
to  furniture  or  the  wall  against  which  his  limbs  might  be  injured  as 
they  are  in  the  throes  of  the  convulsive  seizure.  The  clothing  should 
be  loosened  about  the  neck  to  permit  free  breathing,  and  if  possible  a 
towel  end  rolled  up  and  pressed  between  the  teeth  to  prevent  injury 
to  the  tongue.  As  a  rule  he  should  be  permited  to  remain  on  his  back 
or  side,  according  to  the  position  the  contracted  muscles  force  upon 
him.  A  wound  received  in  falling  may  need  care  and  a  broken  limb 
needs  protection  from  the  severity  of  the  convulsive  contractions  to 
prevent  additional  injury  by  the  broken  ends.  Epileptics,  in  general, 
should  not  be  permitted  to  sleep  unobserved  or  alone  for  fear  they 
may  roll  over  and  smother  during  an  attack.  If  vomiting  occurs  the 
patient  should  be  rolled  on  his  side  and  care  should  be  exercised  to 
prevent  aspiration  of  the  vomitus.  In  the  automatic  period  following, 
watchful  care  is  needed  but  direct  efforts  at  control  should  be  avoided 
if  possible,  as  they  are  not  understood  and  may  only  excite  antagonism. 
The  patient  should  not  be  permitted  to  get  up  until  it  is  seen  that  no 
bones  are  broken.  In  this  automatic  state  an  attempt  to  walk  on  a 
broken  leg  might  easily  compound  the  fracture  for  consciousness  is 
so  reduced  that  pain  would  not  be  felt  or  reacted  to. 
56 


882       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

Treatment  of  Status. — Serial  attacks  indicate  possible  danger  of 
status  and  sedatives  should  be  used  to  control  their  frequency,  while 
sudden  withdrawal  of  bromides  should  be  a^'oided  as  tending  to 
precipitate  a  number  of  seizures.  Clark^  recommends  the  following 
as  an  emergency  prescription  when  status  is  threatened: 

B — Tr.  opii  deod. lUv 

Potas.  bromid gr.  xxv 

Chlor.  hyd gr.  xx 

Liq.  morph.  sulph.  (U.  S.) 3j — M. 

S. — One  dose:  repeat  in  two  hours  if  necessary. 

This  prescription  may  be  given  after  the  first  four  or  five  seizures, 
after  that  sedation  must  be  pushed  to  control  the  attacks  as  they  are 
themselves  a  source  of  serious  danger  to  life.  •  Chloroform  may  be 
given  in  emergency  by  inhalation,  but  the  most  valuable  of  the  drugs 
are  chloral  and  the  bromides.  While  it  is  necessary  to  push  them,  it 
must  be  constantly  kept  in  mind  that  these  very  drugs  are  contra- 
indicated  in  the  next,  the  stuporous  stage,  and  therefore  no  more  should 
be  used  than  is  actually  necessary  to  control  the  situation  as  the  fol- 
lowing coma  will  be  deepened  thereby.  Chloral  and  bromides  are, 
as  a  rule,  best  given  by  rectum.  The  heart  needs  watching  and  may 
need  stimulating,  especially  if  large  doses  of  chloral  are  administered. 

In  the  stuporous  stage  the  treatment  is  stimulating  and  supporting. 
Careful  nursing  and  feeding  and  protection  during  the  great  exhaustion. 

Treatment  heticeen  Attacks. — As  there  is  no  disease  entity  (epilepsy) 
there  is  no  treatment  that  applies  to  all  of  the  cases  included  under 
that  term.  Each  of  the  various  conditions  requires  treatment  suited 
to  it  as  does  each  individual  require  individual  consideration. 

The  various  surgical  conditions,  tumor,  cyst,  abscess,  trauma,  etc., 
require  appropriate  surgical  intervention.  In  tumor,  for  example,  as 
in  other  organic  conditions,  when  the  location  of  the  trouble  is  not 
evident,  a  study  of  the  attack  together  with  the  aura  may  give  valuable 
evidence  to  guide  the  surgeon.  It  is  desirable  to  have  all  such  cases 
reside  in  a  hospital  long  enough  for  their  attacks  to  be  accurately 
observed  before  operating. 

Conditions  of  infection  and  toxemia  require  no  special  mention  here. 
In  arteriosclerosis  with  softening  the  general  condition  overshadows  the 
special  manifestation  as  is  also  generally  the  case  following  hemor- 
rhage. Conditions  of  marked  arrest  of  development,  either  congenital, 
as  due  to  serious  birth  injuries  or  early  inflammations  naturally  offer 
little  prospect  for  improvement. 

Internal  secretion  imbalances  should  be  corrected  as  far  as  possible, 
but  for  the  most  part  little  more  than  palliation  can  be  expected, 
although  the  near  future  may  well  have  something  to  offer  in  this  realm. 

Middle-ear  disease  should  be  adequately  treated  before  meningeal 
sjTnptoms  and  lateral  sinus  thrombosis  take  place. 

The  syphilitic  meningitides,  whether  acquired  or  inherited,  offer  a 

1  Wm.  P.  Spratling:  Epilepsy  and  its  Treatment,  Philadelphia,  1904. 


CLASSICAL  EPILEPSY  883 

promising  field  for  relief  by  appropriate  antisyphilitic  treatment  which 
should  be  intensive  and  include  intravenous  injections  of  salvarsan. 

As  to  the  pharmacotherapy  of  "genuine  epilepsy"  it  can  be  inferred 
from  what  has  already  been  said  that  there  is  none  except  such  as  may 
be  called  upon  to  meet  or  prevent  emergencies  such  as  status.  Bro- 
mides have  been  so  constantly  used,  howe^•er,  that  they  need  to  be 
briefly  discussed. 

Bromide  acts  as  a  motor  depressant — it  raises  the  threshold  of 
motor  discharge  and  does  therefore  inhibit  the  convulsive  expression. 
That  it  does  this  is  sufficient  explanation  for  its  extensive  use.  Many 
popular  medicines  have  much  less  to  recommend  them.  When  we 
realize,  however,  that  the  convulsion  is  not  the  disease,  that  it  is  not 
the  cause  nor  a  first  expression  even  of  the  disease,  but  only  its  outward 
expression  and  the  end-result  at  that,  it  may  be  well  questioned  whether 
bromide  medication  is  rational.  Experience  seems  to  show  that  the 
fit  postponed  by  bromides  comes  to  pass  ultimately  any  way  and  that 
the  bromide  may,  in  fact,  operate  unfavorably  by  tending  to  produce 
a  summation  of  attacks  and  thus  increase  the  danger  of  status.  Added 
to  this  is  the  potentiality  for  disturbing  digestion  which  the  bromides 
possess  so  prominently,  so  it  may  be  said  that  they  had  best  not  be 
given  at  all  unless  under  most  carefully  regulated  conditions.  The  true 
function  of  the  bromides  is  to  control  the  convulsive  manifestations 
when  they,  as  such,  become  a  source  of  danger  as  in  serial  attacks  and 
threatened  status. 

Bearing  in  mind  the  theory  of  the  essential  epileptic  attacks  that 
has  been  elaborated,  the  rational  treatment  in  all  cases  where  the 
underlying  mechanism  cannot  be  unearthed  (as  in  the  symptomatic 
epilepsies),  is  to  assist  in  the  orderly  discharge  of  energy,  to  help  the 
process  of  sublimation.  This  is  best  effected  by  manual  training, 
steady  occupation  graded  to  suit  the  intellectual  level  and  other 
requirements  of  the  patient  and  preferably  conducted  under  institu- 
tional (colony)  supervision. 

In  many  individuals  much  is  to  be  expected  from  psychoanalysis. 
No  results  will  follow  from  short  treatment,  however.  The  most 
favorable  cases  need  from  twelve  to  eighteen  months.  The  treat- 
ment by  this  method  is  reeducational  and  consists  in  carefully  regu- 
lating the  entire  life  and  the  living  conditions  so  as  to  slowly  lead  the 
patient  into  reality  by  arousing  his  interests  in  things  outside  himself, 
a  gradual  leading  away  from  the  egocentric  fixation.  It  is  in  just 
such  cases  as  these  that  much  may  be  expected  in  the  future  from 
organotherapy  in  conjunction  with  a  psychoanalytic  handling  of  the 
personality  problems.  This  latter  must  needs  here  as  elsewhere  often 
include  a  reeducation  of  the  other  members  of  the  family  whose  atti- 
tude often  serves  to  help  to  keep  the  patient  ill. 

Surgery  of  the  colon  is  inefi'ective,  unintelligent,  and  must  be,  in 
most  cases  at  least,  actually  harmful.  It  is  but  another  one  of 
those  attempts  already  referred  to  (hysteria)  of  trying  to  cut  out  the 
psychosis, 


884       EPILEPSY  AND  CONVULSIVE  TYPES  OF  REACTION 

The  social  position  of  the  epileptic  is  most  pitiable,  often  so  impaired 
mentally  that  his  labor  is  at  a  discount,  he  loses  his  job  on  the  occasion 
of  the  first  fit,  even  though  he  may  have  succeeded  in  getting  one 
that  is  free  from  the  dangers  to  which  he  is  particularly  exposed  on 
account  of  his  infirmity.  He  is  thus  seriously  handicapped  in  the 
struggle  for  existence  and  barely  gets  to  earning  his  livelihood  in  one 
position  before  he  finds  himself  jobless  again,  without  recommenda- 
tion and  forced  to  begin  all  over  again.  He  is  thus  apt  to  be  poorly 
nourished,  poorly  clothed  and  the  subject  of  intense  social  repression 
operating  from  without  that  drives  him  back  upon  himself  and  aggra- 
vates greatly  his  trouble.  He  becomes  discouraged  and  depressed 
and  only  too  often  takes  to  alcohol,  the  very  worst  possible  thing  he 
could  do. 

In  the  colony  all  these  social  handicaps  are  removed.  Here  he  may 
have  a  fit  in  peace  and  comfort  without  feeling  that  he  is  disgraced  or 
in  imminent  danger  of  losing  his  means  of  livelihood.  This  relief  alone 
goes  a  long  way  toward  permitting  him  the  use  of  his  avenues  of  expres- 
sion and  in  bringing  about  a  relative  peace  and  quiet,  so  essential  as 
a  therapeutic  adjuvant.  If  in  addition  to  this  he  is  trained  in  some 
form  of  healthy  occupation,  preferably  outdoor,  that  is  interesting 
and  affords  an  added  means  of  expression,  the  best  possible  has  been 
done  for  him.  In  the  colony,  too,  he  is  provided  a  home,  congenial 
surroundings,  a  regulated  diet  and  is  under  that  careful  and  con- 
tinuous skilled  supervision  for  a  prolonged  time  which  is  so  essential 
to  the  best  results.  Under  colony  care  "Spratling  thinks  5  per  cent, 
of  cases  as  they  go  can  be  cured  and  that  this  percentage  could  be 
doubled  or  perhaps  trebled  if  all  the  cases  could  be  gotten  under 
treatment  early. 


CHAPTER   XIX. 
DEMENTIA  PRECOX   (SCHIZOPHRENIA)   GROUP. 

The  term  dementia  precox  has  been  the  occasion  of  a  great  deal 
of  discussion.  Coming  into  general  use  as  it  did  as  the  result  of  the 
studies  of  the  Kraepelinian  school  it  was  conceived  to  apply  to  a  group 
of  psychoses  belonging  to  the  period  of  adolescence  and  presenting 
dementia  as  a  fundamental  element  in  the  symptom  pictm-e.  When, 
however,  it  was  seen  that  what  appeared  to  be  the  same  disease  might 
occur  later  in  life,  even  after  thirty  years  of  age,  it  seemed  hardly 
proper  to  use  the  term  precox  as  applied  to  psychoses  of  early  life. 
It  was  therefore  proposed  that  the  term  precox  should  refer  not  to  the 
age  of  the  patient,  but  to  the  relatively  early  appearance  of  demen- 
tia in  the  course  of  the  disease.  The  term  dementia  was  here  used 
to  mean  a  permanent  mental  impairment,  and  when  it  was  realized 
that  man}'  cases  made  good  recoveries  without  any  apparent  or  at 
least  material  defect  remaining,  another  reason  was  evident  for  the 
inapplicability  of  the  term.  The  concept,  then,  might  be  formulated 
that  it  was  a  disease  in  which  dementia  was  a  relatively  early  symp- 
tom and  that  the  recoveries  occurred  only  when  the  disease  had  not 
progressed  to  any  extent.  This  also,  unfortunately,  does  not  meet 
thejacts.  because^an}^  cases  get  wgll^fter  prdlbii^edjiiid  appareirtly 
cHronic  courses!  Irr~the  absence^f  any  well  defined  crTterian5f 
'3ementia  it  was  impossible  to  predict  when  it  was  or  was  not  present, 
and  therefore  the  term  presents  very  many  undesirable  features. 
Although  it  is  somewhat  of  a  bootless  task  to  discuss  names,  and 
although  it  is  much  more  important  to  know  what  the  names  stand 
for  than  to  quibble  about  their  applicability,  still  it  is,  of  course, 
desirable  to  have  a  name  that  fairly  represents  the  thing  named.  To 
meet  this  demand  Bleuler  has  suggested  the  name  schizophrenia, 
implying  a  splitting  of  the  personality,  which  he  thinks  is  the  funda- 
mental symptom.  Although  this  term  as  used  by  Bleuler  includes  a 
number  of  conditions  that  many  psychiatrists  would  object  to  as 
being  included  in  the  dementia  precox  concept,  still  it  is  generally 
conceded  that  the  splitting  of  the  personality,  as  indicated  by  the 
name,  is  fundamental  in  this  group,  and  the  name  is  coming  into 
gradually  more  and  more  general  use. 

Dementia  precox  must  undoubtedly  have  always  existed  and 
have  been  observed  by  physicians,  and  in  particular  the  grotesque 
cases  of  catatonic  rigidity  and  peculiar  mannerisms  must  have  always 
attracted  attention.  In  the  early  history  of  psychiatry,  however, 
few  descriptions  of  cases  exist  that  could  be  unequivocally  said  to  be 

(885) 


886  DEMENTIA   PRECOX  GROUP 

cases  of  precox,  as  the  group  had  not  been  defined  from  other  groups 
superficially  resembhng  it,  as  for  example,  imbecility.  Willis,  the 
English  anatomist,  recognized  as  early  as  1672  that  many  young 
people  underwent  deterioration  and  Sydenham,  a  hundred  years 
later,  in  1772,  describes  similar  conditions  under  the  description  of 
stupidity,  while  later  on,  after  mania  and  melancholia  had  been 
more  or  less  defined,  many  of  the  excitements  and  depressions  that 
are  incident  to  the  course  of  dementia  precox  were  undoubtedly 
grouped  under  these  headings,  while  at  one  period,  only  a  few  years 
ago,  there  was  a  distinct  group  supposedly  representing  a  special 
disease  described  by  the  name  of  catalepsy,  where  also  undoubtedly 
a  certain  number  of  precox  cases  were  arranged. 

At  the_p££S£nt_time  three  pretty  welljdefined  groupj  of  cases  are 
incTu^Iedm  jthe_^nera  I  concept  of  dementiaT  precox,  namely,  the  hebe- 
phrenic, the  catatonic  and^  the  paranoid.  Kahlbaum  was  the  first 
to'describe  hebephrenia  as  a  disease  entity  in  1863  and  in  1871  his 
pupil,  Hecker,  published  some  excellent  descriptions  of  this  disease. 
In  1869  Kahlbaum  described  catatonia  under  the  term  Spannungsir- 
resein  or  vesania  catatonica,  of  which  he  gave  an  admirable  mono- 
graphic description  in  1874.  In  1896  Kraepelin,  in  the  fifth  edition 
of  his  Lehrbuch,  arranged  dementia  precox,  catatonia  and  dementia 
paranoides  as  disorders  of  metabolism.  Clouston,  the  Scotch  psy- 
chiatrist, had  already  described  what  he  termed  adolescent  insanity 
and  objected  to  the  term  dementia  precox  as  being  too  inclusive. 
KraepeHn,  however,  worked  over  his  material  with  great  thoroughness 
and  arrived  at  the  concept  that  includes  the  three  forms,  hebephrenic, 
catatonic  and  paranoid  by  tracing  the  life  histories  of  his  patients 
and  grouping  all  these  cases,  however  dissimilar  they  might  appear 
on  the  surface,  from  the  standpoint  of  prognosis.  They  were  cases 
that  had  a  fairly  definite  course  and  outcome,  eventuating  always 
in  a  certain  degree  of  dementia^    ,^-^ 

Etiology.- — ^The  question  oi(^heredity)  in  precox  has  been  studied, 
particularly  by  Wolfsohn,^  whocarefully  analyzed  the  material  from 
this  standpoint  at  the  Bergholzi  asylum  in  Zurich.  The  study  of 
2215  admissions  disclosed  647  cases  of  dementia  precox  of  whom  90 
per  cent,  showed  hereditary  taint.  Of  four  factors,  mental  disease 
was  the  most  frequent — about  64  per  cent. — ^followed  by  nervous 
diseases,  alcoholism  and  other  forms  of  hereditary  taint.  Heredity 
was  combined  in  34  per  cent.  The  most  frequent  combinations  were 
those  of  psychoses  and  alcoholism,  and  psychoses  and  nervous  disease. 
She  concluded  that  a  distinct  influence  of  heredity  could  not  be  proved 
in  the  cases  in  which  the  taint  was  alcoholism,  nervous  disease,  or 
other  forms.  The  catatonic  was  the  most  and  paranoid  the  least 
aft'ected  by  the  mental  taint,  while  the  influence  of  the  taint  had  no 
striking  effect  on  the  character  of  the  first  symptoms  of  the  disease. 

>  JeUiffe:  Dementia  Precox,  a  Historical  Summary,  New  York  Med.  Jour.,  1912. 
2  Die  Hereditat  bei  Dementia  Precox,  Allg.  Ztschr.  f.  Psych.,  1907,  Band  Ixiv,  Heft 
2  and  3. 


SYMPTOMS  8S7 

It  would  seem  that  there  is  a  certain  element  of  direct  heredity, 
inasmuch  as  families  are  found  in  which  se\-eral  cases  of  precox  occiu-, 
just  as  there  are  families  found  in  which  several  cases  of  the  manic- 
depressive  psychosis  occur. 

Of  the  exciting  causes  severe  shocks,  both  mental  and  physical,  are 
not  infrequently  found,  as  for  example,  severe  hemorrhages  and  infec- 
tions following  parturition.  In  the  latter  cases  precox  breakdowns 
occur  as  a  result  of  that  train  of  emotional  disturbances  which  follows 
upon  seduction  and  desertion. 

For  a  number  of  years  there  has  been  a  tendency  to  ascribe  the 
disease  to  distm-bances  of  metabolism  with  possible  toxic  factors  and 
to  suppose  that  its  origin  might  be  traced  to  disorders  of  the  glandular 
secretions,  more  particularly  of  late,  of  the  internal  secretions  of  the 
ductless  glands  and  inasmuch  as  the  disease  tends  to  focalize  about 
the  period  of  puberty  and  adolescence,  it  has  been  supposed  that 
perhaps  the  testicles  and  the  ovaries  might  be  the  offending  organs. 
This  theory  has  received  confirmation  recently  more  especially  by 
Fauser,  who,  working  with  the  Abderhalden  technic  has  isolated 
defensive  ferments  against  the  gonads  and  the  cortex.  More  will  be 
said  of  this  matter  later  when  the  discussion  of  the  nature  of  the 
disease  is  taken  up,  but  it  may  be  mentioned  here  that  whatever  its 
ultimate  nature  may  be  the  existence  of.  toxic  factors,  or  internal 
secretory  disturbances  is  largely  hypothetical  so  that  at  the  present 
time  it  is  more  useful  to  fo'-mulate  the  upsetting  factors  as  well  as  the 
general  symptomatology  in  psychological  terms  rather  than  in  terms 
descriptive  of  disturbances  at  physicochemical  levels.  In  any  case 
toxic  factors  may  as  well  be  effects  as  causes  or  perhaps  concomitant 
factors  in  a  vicious  circle. 

The  formulation  of  the  disease  in  terms  of  the  affects  or  of  complexes 
or,  in  accordance  with  Meyer,^  continued  unhealthy  biological  reac- 
tions, or  as  an  outgrowth  of  a  "shut  in"  character  is  after  all  niore 
of  an  effort  at  description  of  what  is  found.  Everyone  has  complexes, 
but  it  is  not  clear  why  in  certain  cases  they  lead  to  the  development 
of  a  precox  psychosis,  while  a  "shut  in"  character  might  itself  in 
certain  cases  at  least  be  considered  to  be  an  early  expression  of  the 
disease  process,  a  latent  precox  perhaps,  in  the  sense  of  Bleuler.^ 

Sjnnptoms, — Mental. — A  patient  from  time  to  time  Avrites  letters 
appealing  for  his  discharge  and  his  liberty  and  signing  himself  "The 
Emperor,"  It  is  this  incongruity,  this  lack  of  oneness  of  the  individual 
that  for  a  long  time  has  attracted  attention  in  the  svinptomatology 
of  this  disease.  I  low  is  it  possible  for  a  person  so  exalted  as  to  think 
himself  an  emperor  to  plead  in  quite  a  natural  way  for  his  discharge 
from  an  asylum?  The  two  positions  which  the  man  takes,  acknowl- 
edging himself  as  a  patient  and  pleading  for  his  discharge  while  still 
proclaiming  himself  an  emperor,  show  the  possibilit\'  of  maintaining 

*  Fundamental  Conceptions  of  Dementia  Precox,  Brit.  Med.  Jour.,  September  29,  1906. 
^Bleuler:  Die  Schizophrenic,  1911;  also  see  Bleulcr:  Lehrbuch  der  Psychiatric,  1917; 
KraepeUn:  Lehrbuch  der  Psychiatrie,  8  Edit. 


DEMENTIA  PRECOX  GROUP 

two  distinct  and  mutually  opposed  trends  of  thought  at  the  same 
time  without  the  one  apparently  interfering  or  serving  at  all  to  correct 
or  modify  the  other. 

This  possibility  is  dependent  upon  a  fundanientalassociational  dis- 
turbance  which  has  caused  Bleuler  to  see  su^spKtting  of  the  personalif^) 
as  the  foundation  symptom  of  the  disease  and  to  give  it  the  nameT 
therefore,  of  schizophrenia.  I'his  splitting  of  the  personality  has  been 
expressed  less  clearly  in  many  of  the  theories  that  have  been  advanced 
to  account  for  the  symptoms,  while  a  massive  splitting  of  the  person- 
ality has  long  been  described  as  a  fundamental  symptom  of  hysteria 
under  the  designation  of  dissociation.  The  symptoms  have  been  said 
to  be  dependent  upon  a  disintegration  of  the  personality,  a  disinte- 
gration of  consciousness  with  the  consequent  impairment  of  the  func- 
tion of  the  real.  Reality  is  unable  to  correct  or  adequately  modify 
the  delusional  ideaX^ith  fhe  result  that  such  bizarre,  ^trangp  aruT 
apparently^niips.ydToTogical  modifications  of  conduct  are  seen. 

These  unpsycJiological  appearances  are  dependent  upon  what 
Bleuler  calls  autistic  thinling,  that  is,  a  form  of  thinking  to  which  Jung 
would  apply  the  term  introverted,  in  which  the  individual's  interests 
are  w^ithdra^vn  from  reality,  and  he  occupies  himself  with  himself 
to  the  more  or  less  complete  exclusion  of  the  outside  world.  This 
is  the  field  of  dream  formation,  of  fantasies,  wherein  things  come 
true.  An  analysis  of  cases  of  dementia  precox  shows  that  the  delusion 
formations  are  based  upon  wish-fulfilling  mechanisms  which  result 
oftentimes  in  highly  symbolic,  and  to  the  observer,  non-understandable 
expressions  which  are  formulated  in  accordance  with  the  particular 
complexes  which  may  be  operative.  These  mechanisms  are  the  same 
as  those  observed  in  normal  people,  in  hysterics,  and  those  suffering 
from  the  various  neuroses,  but  it  is  impossible  for  these  individuals 
to  adequately  utilize  them;  they  therefore  result  in  impairment  of 
efficiency  and  withdrawal  from  the  world  of  realitv.  It  would  seem, 
too,  that  in  precox  the  regression  is  very  much  more  profound  than  in 
the  neuroses  and  the  psychoneuroses,  and  for  some  unknown  reason 
involves  a  serious  disintegration  of  the  personality  which  tends  to 
become  chronic  and  crippling.  From  the  standpoint  of  this  schizo- 
phrenic splitting  of  the  psyche,  based  upon  autistic  thinking,  many 
points  in  the  symptomatology  of  the  disease  become  understandable. 

The  peculiar  emotional  dulness  and  uncertainty  of  emotional 
response  of  the  precox  has  long  been  noticed,  and  Stransky^  has  par- 
ticularly designated  it  by  the  term  intrapsychic  ataxia,  by  which  he 
means  a  disturbance  of  the  coordination  between  the  intellectual 
and  the  affective  attributes  of  the  psyche,  which  are  respectively 
knoMii  as  the  noopsyche  and  the  thymopsyche.  This  noothymo- 
psychic  ataxia  gives  the  appearance  at  times  of  emotional  dulling  and 
at  other  times  of  a  senseless  emotional  reaction.    Thus  a  patient  who 

'  Ueber  die  Dementia  Precox,  Streifziige  durch  Klinik  und  Psychopathologie,  Verlag. 
von  T.   F.  Bergmann:  Wiesbaden,  1909. 


SYMPTOMS  889 

receives  news  of  the  death  of  a  near  relative  unmoved,  may  a  little 
later  on  laugh  heartily  at  apparently  nothing.  He  is  happy  when  he 
should  be  sad,  sad  when  he  should  be  happy,  angry  without  cause, 
experiencing  fear  without  reason,  for  the  most  part  cold  and  impas- 
sive, but  occasionally  showing  outbursts  of  marked  and  accentuated 
emotionalism.  This  is  the  "April  weather"  behavior  of  the  affects, 
in  the  language  of  Stransky. 

This  condition  of  affairs  is  understandable  upon  the  basis  of  the 
splitting  of  the  psyche.  The  emotional  reactions  occur  when  the 
complexes  have  been  touched,  which  the  individual  is  constantly  in 
an  attitude  of  trying  to  prevent.  This  method  of  dealing  with  the 
emotions  is  well  known,  particularly  in  the  formation  of  the  dream, 
in  which  the  mechanisvi  of  displacement,  by  removing  the  affect  from 
the  constellation  of  ideas  to  which  it  belongs  and  attaching  it  to  an 
indifferent  set  of  ideas  serves  to  hide  from  the  patient  the  realization 
of  the  actual  difficulty.  For  example,  a  patient  identifies  herself  with 
a  schoolmate  of  her's  and  then  accuses  the  schoolmate  of  being  "bad" 
and  pregnant.  It  will  be  easily  seen  that  in  such  a  case  the  patient 
is  protecting  herself  from  the  realization  that  she  has  thoughts  to 
which  the  term  "bad"  might  be  applied,  and  that  the  pregnancy  which 
might  result  from  being  bad  is  transferred  to  her  schoolmate.  Under 
such  circumstances,  she,  so  to  speak,  unloads  her  emotion  upon  this 
schoolmate  and  very  easily  may  produce  the  impression  of  indifference 
toward  herself  with  an  unmotived  affective  attitude  toward  the 
schoolmate. 

This  icithdraical  from  reality,  this  looking  within,  occupying  them- 
splvpswith  themselves,  no  longer  subject  to  the  corrective  influences 
ofThe  outside  world,  produces  many  surface  indications,  among  which 
are  failures  of  voluntary  attention,  lack  of  interest,  disturbances  of 
orientation,  disorders  of  memory.  The  disorders  of  attention,  lack  of 
interest,  failure  of  voluntary  attention  can  easily  be  seen  to  be  due  to 
the  turning  of  the  interests  within.  The  capacity  for  attention  may  be 
as  keen  as  ever,  but  the  patients  are  not  attending  to  the  things  going 
on  about  them,  but  rather  to  the  things  going  on  within,  and  so  they 
apparently  take  no  interest  in  the  people  or  the  events  of  their  environ- 
ment. They  may  even  express  themselves  as  perfectly  satisfied  with 
their  confinement  in  a  hospital,  and  be  so  manifestly  heedless  of  those 
about  them  that  it  is  practically  impossible  to  draw  them  into  con- 
versation. This  lack  of  interest  and  attention  naturally  produces 
what  appear  to  be  disturbances  of  memory  and  orientation.  The 
patient,  who  is  heedless  of  his  surroundings,  may  easily  not  know  the 
day  of  the  week  or  may  have  forgotten  the  events  that  only  recently 
took  place  about  him,  because  they  were  not  sufficiently  attended 
to  to  make  any  profound  impression,  while  a  i)atient  who  identifies 
himself  with  some  great  public  functionary  might  easily  not  give  the 
correct  date  of  his  own  birth.  Such  considerations  as  these  show  how 
necessary  it  is  to  penetrate  beneath  the  surface  indications  which 
the  patient  manifests  and  find  out  their  true  meaning.    They  also 


890  DEMENTIA   PRECOX  GROUP 

indicate  unequivocally  that  what  may  appear  c[uite  unpsychological 
is  really  perfectly  understandable. 

The  same  type  of  explanation  serves  to  render  clear  the  meaning 
of  such  surface  indications  as  the  shallowness  of  thinking  and  the 
apparent  dilapidaiion  of  thought.  The  interests  and  the  energies  are 
occupied  with  things  w^hich  are  not  accessible  to  casual  questioning. 
In  fact,  as  will  later  on  be  seen,  the  patient  may  be  wholly  inaccessible 
to  any  form  of  approach,  and  when  there  is  considerable  speech  pro- 
ductivity what  is  said  may  be  so  incoherent  upon  the  surface  as  to 
be  quite  non-understandable  and  constitute  what  has  been  called  a 
"word  salad." 

The  delusional  formation  of  precox  is  notoriously  grotesque  and 
partakes  of  this  characteristic  to  such  an  extent  in  harmony  with 
the  grotesqueness  of  the  thoughts  in  dreams  that  the  similarity  has 
not  been  overlooked.  The  mechanisms,  too,  are  probably  quite  the 
same,  although  much  more  difficult  to  fathom  because  of  the  inacessi- 
bility  and  lack  of  cooperation  of  the  patient.  If  the  eye  is  properly 
trained,  however,  to  see  meaning  in  the  apparently  meaningless, 
there  will  be  little  difficulty  in  seeing  how  certain  expressions  may 
be  interpreted,  even  though  in  an  individual  case  it  may  be  impossible 
to  verify  such  an  interpretation.  For  example,  an  old  precox  who 
talked  in  a  thoroughly  dilapidated  manner  but  was  able  with  some 
patience  to  give  a  fairly  good  account  of  himself,  injected  into  his 
series  of  replies  to  questions  that  the  President  was  confined  in  an 
almshouse  and  that  he  had  come  to  ^Yashington  to  be  President. 
He  also  stated  that  he  had  had  something  to  do  with  his  sister  when 
he  was  a  young  boy,  that  he  did  not  tell  the  priest  and  that  his  shadow 
was  very  heavy,  that  it  was  black  and  that  he  saw  the  Devil  in  it. 
Here  an  expression  of  grandiose  ideas  which,  speaking  generally,  may 
be  considered  as  compensatory,  is  seen.  In  his  autistic  thinking  he 
wishes  to  be  the  great  man  that  in  his  real  life  he  is  not.  Then  one 
sees  the  possibility  of  a  serious  moral  conflict,  the  result  of  incestuous 
relations  with,  or  perhaps  only  incestuous  thoughts  about,  his  sister, 
while  his  dark  and  heavy  shadow  in  which  he  sees  the  Devil  can  be 
easily  seen  to  be  a  symbolic  representation  of  the  destructive  effects 
which  his  moral  delinquencies  have  had  upon  him.  Here  one  also 
sees  that  the  hallucinatory  experiences,  the  disorders  of  the  sensorium 
express  themselves  as  symbolisms  of  the  conflict  and  receive  their 
interpretation  with  a  knowledge  of  the  nature  of  that  conflict. 

The  delusions  are  essentially  endogenous  in  origin,  that  is,  ulti- 
mately dependent  upon  factors  that  are  within  the  individual,  and 
they  tend  to  be  colored  and  determined  by  complexes  which  lie  at  the 
very  foundation  of  the  personality,  which  have  to  do  with  the  region 
of  the  psyche  which  has  long  since  been  forgotten  and  to  which  Freud 
gives  the  name  "unconscious."  It  is  largely  because  of  their  pro- 
foundly unconscious  origin  that  they  are  inaccessible  and  it  is  largely 
owing  to  this  also  that  they  produce  such  serious  disturbances  and 
such  marked  departures  from  conduct  that  is  at  all  eflficient. 


SYMPTOMS  891 

It  is,  too,  because  the  motivation  of  the  activities  of  the  patient  and 
the  origin  of  his  dekisions  are  in  the  unconscious  that  his  conduct  and 
productions  strike  us  as  unpsychological.  That  which  originates 
in  the  unconscious,  because  its  origin  is  unknown,  seems  strange  a'nd 
mysterious. 

Just  as  the  hallucinations  are  symbolisms  of  the  elements  in  the 
conflict  so  are  the  various  mannerisms,  stereotypies  and  neologisms. 
All  of  these  motor  disturbances  are  in  effect  symptom  activities,  complex 
indicators,  and  serve  in  some  way  to  portray  the  nature  of  the  conflict. 
In  a  recently  reported  case,  for  example,  an  old  precox  was  observed 
to  keep  pounding  one  hand  with  her  clenched  fist  in  a  rhythmic  stereo- 
typed fashion.  It  was  discovered  that  in  her  earlier  days  she  had 
been  jilted  by  a  shoemaker.  This  peculiar  action  could  be  seen,  in 
the  light  of  this  knowledge,  as  but  the  movements  of  the  shoemaker 
pounding  at  his  last.  Many  of  the  old  cases  of  precox  have  such 
stereotyped  activities  which  it  is  often,  in  fact  usually,  quite  impossible 
to  fathom,  but  in  the  light  of  such  a  case  as  this  it  will  be  seen  that 
they  must  all  be  looked  upon  as  having  meaning  and  that  for  a  com- 
plete reconstruction  of  the  psychosis  it  is  necessary  to  determine  what 
that  meaning  is. 

As  an  example  of  what  painstaking  analysis  may  disclose  may  be 
mentioned  a  patient  of  Jung's.  She  expressed  herself  in  stereotyped 
and  apparently  meaningless  phrases  interspersed  with  neologisms. 
One  of  her  statements  was :  "  I  affirm  a  million  Hufeland  to  the  left  on 
the  last  fragment  of  earth  on  the  hill  above."  A  detailed  analysis 
disclosed  this  sentence  to  mean,  approximately:  "For  the  bad  treat- 
ment of  the  physicians  which  I  have  to  endure  here  and  with  which 
T  am  tojtnreH  to  death  I  claim  a  high  indemnity." 
Cj^egativis^,  one  of  the  characteristic  symptoms  of  precox,  may  be 
expressed  passively  by  the  patient's  not  doing  what  is  expected  of 
him,  or  what  he  should  do,  or  by  actively  doing  the  exact  opposite 
of  what  is  requested.  The  passive  negativism  may  show  itself  in 
refusal  to  attend  to  the  promptings  of  normal  desires,  so  that  the 
bladder  and  the  rectum  are  permitted  to  become  overloaded  and  the 
saliva  to  collect  in  large  quantities  in  the  mouth,  or,  on  the  other 
hand,  active  negativism  may  show  itself  in  the  patient  doing  the 
exact  opposite  of  what  he  is  asked  to  do;  for  example,  if  he  is  asked 
to  shut  his  eyes  he  opens  them  wider  and  if  he  is  asked  to  open  them 
he  will  shut  them  tightly,  and  if  he  is  asked  to  put  out  the  tongue  he 
shuts  his  lips  tightly,  and  if  he  is  asked  to  shut  his  mouth  he  opens 
it,  etc.  This  peculiar  symptom  is  dependent  upon  what  Bleuler 
terms  th^^rnnfJtmlency  pfuteas^  by  which  he  means  that  every  idea  has 
connected  with  it  by  assfjutstion  its  exact  opposite  more  intimately 
than  other  ideas,  and  so  the  patient  who  is  witlulrawn  from  reality  and 
objects  to  being  invaded  by  the  world  of  real  things,  who  would,  in 
other  words,  live  within  himself,  finds  that  in  refusing  to  accord 
with  a  suggestion  from  the  outer  world  the  path  of  the  diametrically 
opposite  reaction,  is  more  patent,  more  accessible  than  any  other. 


892  DEMENTIA   PRECOX  GROUP 

This  is  a  type  of  reaction  which  one  sees  also  in  other  conditions,  as 
in  hysteria,  and  also  quite  naturally  in  children. 

The  suggestibility  of  the  precox  receives  a  similar  explanation. 
Some  patients  find  it  easier  to  follow  blindly  any  suggestion  which 
comes  to  them  from  any  source  than  to  actively  initiate  contact  with 
reality.  One  precox  patient,  for  example,  had  to  be  fed  by  placing  his 
food  before  him  and  a  spoon  in  his  hand  and  repeating  each  time  the 
command  to  take  another  mouthful.  After  having  responded  to  the 
command  he  remained  inert  until  it  was  repeated.  This  form  of 
activity  requires  no  initiative  on  the  part  of  the  patient,  no  actual 
effective  contact  with  reality  of  his  own  devising  and  permits  him  to . 
remain  with'"  hin^lf^  rnnph  as  does  the  negativistic  tendency. 

The  mtaiQiiic  rigidity)  oi  the  precox  is  a  still  more  active  shutting 
out  of  the  world  to  the  point  of  absolute  inattention  to  the  environ- 
ment, but  an  inattention  which  is  positive  and  active  rather  than 
passive,  as  in  catalepsy  and  command  automatism,  while  stupor  still 
more  effectively  shuts  out  the  world  of  real  things. 

There  is  a  reaction  type  in  dementia  precox  which  is  important  for 
an  understanding  of  the  nature  of  the  disease.  It  is  the  archaic  type 
of  reaction.  In  the  illustrations  that  have  been  given  it  is  seen  that 
when  the  individual  under  the  influence  of  mental  disease  regresses 
he  not  only  reaches  lower  levels  but  frequently  reaches  levels  corre- 
sponding with  his  early  infancy,  and  so  it  is  frequently  found  that  the 
delusions  and  other  morbid  manifestations  only  find  their  explanation 
when  traced  back  to  the  infantile  period.  In  the  same  way  regressions 
may  lead  back  to  lower  cultural  levels  so  that  patients  show  symptoms 
that  are  understandable  in  terms  of  the  psychology  of  primitive 
peoples.  The  materials  of  experience  are  used  by  the  patient  in  an 
archaic  way.  One  such  patient  with  a  very  complicated  delusional 
system  states  that  he  is  the  father  of  Adam,  that  he  has  lived  in  his 
present  human  body  thirty-five  years,  but  in  other  bodies  thirty 
million  years,  and  that  during  this  time  he  has  occupied  six  million 
different  bodies.  He  has  been  the  great  men  in  the  history  of  the 
development  of  the  human  race;  he  himself  created  the  human  race; 
it  took  him  three  hundred  million  years  to  perfect  the  first  fully 
developed  human  being;  he  is  both  male  and  female  and  identifies 
all  the  different  parts  of  the  universe  with  his  own  body;  Heaven, 
Plell  and  Purgatory  are  located  in  his  limbs,  the  stars  are  pieces  of 
his  body  which  have  been  torn  apart  by  torture  and  persecution  in 
various  ages  of  past  history ;  he  is  the  father  and  creator  of  the  various 
races  and  elements  of  the  human  organization,  etc.  Here  is  a  very 
primitive  type  of  thinking  in  which  the  patient  identifies  himself  with 
the  whole  universe  somewhat  as  the  baby  does  and  somewhat  as 
primitive  man  does.  He  is  quite  in  the  position  of  the  chief  of  some 
primitive  tribes  in  whom  all  the  forces  of  the  universe  center  and 
from  whom  radiate  all  of  these  forces  for  good  or  for  ill.  This  is  the 
archaic  type  of  reaction  which  shows  how  deep  the  split  of  the  person- 
ality may  be,  how  fundamental  it  may  be  and  gives  an  insight  into 
the  seriousness  of  the  disease  process. 


SYMPTOMS  893 

This  archaic  type  of  reaction  results  from  regression,  or  in  other 
words  an  introversion  of  the  libido  which  is  the  exact  opposite  of  what 
is  fmmrl  to  be  the  case  in  the  manic-depressive  psychosis"!  t)ementia 
precox  is  an  introversion  psychoshs} 

Physical. — A  considerable  number  of  precox  patients,  particularly 
the  catatonic  and  the  hebephrenic,  show  marked  physical  s^Tnptoms, 
and  not  infrequently  ha^-e  all  the  outward  appearances  of  being  quite 
ill.  They  often  emaciate  during  the  early  period  of  their  illness, 
suffer  from  anorexia  and  insomnia,  circulatory  disturbances,  disturbed 
cardiac  action,  cyanosis  of  the  extremities,  vasomotor  disorders  of 
which  dermographia  is  not  an  infrequent  manifestation.  The  deep 
reflexes  are  commonly  exaggerated,  while  the  pupils  in  this  class  of 
cases  are  characteristically  widely  dilated.  Convulsive  seizures  of  an 
epileptiform,  but  more  often  of  a  hysteriform,  variety  may  occur. 

In  the  very  early  stages  of  the  disease  physical  symptoms  which 
do  not  lead  to  the  suspicion  of  mental  disturbance  are  not  infrequent. 
Such  symptoms  as  headache  may  be  in  evidence  for  a  considerable 
time  as  may  also  vertigo,  and  Urstein-  has  called  special  attention  to 
the  occurrence  of  gastric  disturbances.  Other  physical  disturbances 
may  also  of  course  occur,  and  if  no  adequate  foundation  can  be  found 
for  them  a  mental  origin  should  be  thought  of. 

It  is  this  type  of  physical  illness  that  has  led  to  and  maintained  the 
belief  in  etiological  factors  at  the  biochemical  level. 

The  Abderhalden  method  of  research,  especially  as  applied  by 
Fauser,  has  indicted  the  adrenal,  the  thyroid  and  the  gonads.  A 
number  of  investigators  have  also  found  a  reaction  to  brain  tissue 
(cortex). 

Symptoms  of  disorder  of  the  various  viscera  not  infrequently  are 
noted  in  the  prodromal  period  of  the  disease  and  are  treated  for  some 
time  before  the  psychosis  is  sufficiently  in  evidence  to  force  recognition. 
Eppinger  and  Hess  f  nd  a  tendency  to  instability  of  both  parts  of  the 
vegetative  ner^•ous  system  and  many  of  the  gastro-intestinal,  cardiac, 
circulatory  and  cutaneous  conditions  are  dependent  upon  disturb- 
ances at  this  level.  Vagotonic  symptoms  are  not  infrequently  in 
evidence.  Laignel-Lavastine  records  pathological  changes  in  the 
sympathetic  ganglia,  notes  the  commonly  observed  fact  of  the  fre- 
quency of  death  from  pulmonary  tuberculosis  and  observes  that  the 
precox  often  has  a  small  heart.  Many  other  abnormal  conditions 
have  been  found  but  their  correlation  to  the  larger  concept  of  precox 
is  not  yet  clear. 

In  connection  with  this  whole  matter  of  defective  organs  Adlers' 
concept^  of  the  part  they  play  at  the  psychological  level  is  helpful.  The 
psyche  is  the  region  where  the  final  correlations  and  integrations  of  all 
the  reacting  levels  find  final  expression  so  that  a  badly  constructed  body 

1  Jung:  Psychology  of  Dementia  Precox,  Nervous  and  Mental  Disease  Monograph 
Series  No.  3. 

*  Die  Dementia  Precox  und  hre  Stellung  zum  manisch-depressiven  Irresein,  Berlin 
u.  Wien,  1909. 

»  Adler:  Organ  Inferiority,  Nerv.  and  Ment.  Dis.  Monograph  Series,  No.  24. 


894  DEMENTIA   PRECOX  GROUP 

must  express  itself  at  the  psychological  level  in  defects  of  adjustment. 
The  correlation  of  the  psychological  symptoms  with  the  several  organ 
defects  is  not  always  possible  in  the  present  state  of  our  knowledge. 
Evidence  is  accumulating,  however,  to  indicate  that  ultimately  we 
may  exj^ect  a  rich  symptomatology  at  the  vegetative  level  (endocrine 
glands  and  vegetative  nervous  system)  and  that  many  of  the  func- 
tional physiological  disturbances  and  even  organic  changes  (cortical 
aplasias)  will  find  their  explanation  in  anomalies  at  their  level.  We 
are  just  begiiming  to  glimpse  the  possibilities  of  correlating  all  these 
disparate  SAHiptoms  into  a  comprehensive  and  satisfying  concept. 

Mode  of  Onset. — The  early  manifestations  of  precox  vary  greatly. 
x\s^Jj£acly  indicated  the  disease  may  remain  latent  for  a  considerable. 
period,  manifesting  itself  only  in  slight  disturbances,  predominantly 
oTa  physical  nature,  sucii  as  headaches  and  gastro-mtestinal  disorders. 
In  quite  a  large  percentage  of  cases  a  "shut-m"  type  of  character  has 
been  found  to  have  existed  for  a  considerable  period  before  the  out- 
break of  the  symptoms,  in  fact  to  have  been  a  characteristic  of  the 
individual  before  the  breakdoA\Ti.  Whether  this  is  to  be  considered 
as  a  symptom  of  the  disorder  or  an  expression  of  the  type  of  individual 
in  whom  the  disorder  is  possible  cannot  be  answered  at  this  time. 

In  sharp  contrast  to  these  latent  periods  the  disorder  develops 
not  infrequently  with  great  suddenness.  After  some  emotional  shock 
the  patient  becomes  almost  immediately  greatly  confused  or  cata- 
tonic. 

It  is  quite  frequent  to  have  the  breakdown  be  a  slowly  progressive, 
developing  condition.  During  the  early  period  the  symptoms  may 
take  all  sorts  of  forms  and  may  easily  be  mistaken  for  tj^es  of  manic- 
depressive  psychosis,  compulsion  neuroses,  neurasthenia,  hysteria, 
hypochondria,  acute  confusion  and  paranoid  states.  Anything 
atypical  in  these  psychoses  should  make  one  think  of  the  possibility 
of  dementia  precox. 

Symptom  Groups. — The  varieties  of  dementia  precox  will  be  described 
under  five  heads :  I.  Dementia  Simplex;  II.  Hebephrenia:  III.  Cata- 
tonia]_JV^Faranoid  Forms;    V.  Mixed  and  AtypicajjVrms. 

I.  Demeutia  ISimplex. — in  this  group  of  cases^the  origin~is  usually 
insidious,  perhaps  manifesting  itself  only  by  slight  physical  disturl5- 
ances,  such  as  headache,  gastro-intestinal  attacks,  by  some  irritability, 
and  perhaps  a  tendency  to  withdraw  from  the  association  of  others. 
The  patient  may  suffer  from  dnsomiiia),  perhaps  disa^greeahlg  m^ea^l', 
and  there  may  be  passing  evidences  of  hallucinations  and  delusions. 
These  are  apt  to  be  expressed  only  at  times,  perhaps  under  the  influ- 
ence of  a  little  excitement,  and  show  little  tendency  to  organization 
or  progression. 

The  patient  emotionally  is  more  apt  to  be  somewhat  depressed,  or 
at  least  indifferent,  lacking  in  initiative  and  presenting  outwardly  an 
appearance  that  frequently  leads  to  the  diagnosis  of  a  "nervous  break- 
dowTi,"  "nervous  prostration,"  or  neurasthenia. 

Such  mild  attacks  as  this  may  be  pretty  well  recovered  from  but 


SYMPTOM  GROUPS  895 

may  recur  and  one  not  infrequently  finds  a  history  of  one  or  more 
such  attacks  having  preceded  a  more  severe  breakdowTi. 

It  is  this  group  of  cases  of  mild  abortive  forms,  formes  frustes  of 
the  French,  which  after  recovery  from  a  "flurry"  or  "excited  epi- 
sode," gives  one  the  impression  of  "peculiar  characters."  Many  such 
cases  are  found  in  the  ranks  of  the  criminals,  hoboes,  prostitutes, 
pseudogeniuses,  cranks  and  eccentrics,  and  accurate  analyses  of 
the  histories  in  these  cases  would  not  infrequently  show  a  precox 
episode  which  separated  a  period  of  relative  efficiency  in  their  lives 
from  the  period  following  of  relative  inefficiency,  in  which  latter  it 
might  be  possible  to  detect  certam  precox  residuals. 

An  analysis  of  the  life  histories  of  this  class  of  cases  shows  quite 
regularly  a  constant  tendency  to  slip  from  under  all  forms  of  responsi- 
bility, and  a  lack  of  capacity  for  any  kind  of  continuous  application 
and  inability  to  develop  the  habit  of  work.  They  characteristically 
resort  to  the  hobo  tj'pe  of  existence,  are  unable  to  adapt,  with  any 
degree  of  efficiency  at  all,  to  complex  conditions  of  life  and  wander 
from  place  to  place,  occupying  one  position  after  another  from  which 
they  are  either  dismissed  because  of  inefficiency  or  leave  voluntarily, 
giving  reasons  for  so  doing  which  are  totally  inadequate.  Such  cases 
as  these,  sometimes  by  a  steady  progress  become  very  greatly  dilapi- 
dated and  it  is  quite  surprising  at  times  to  find  the  amount  of  deteri- 
oration in  such  cases  after  their  admission  to  an  institution  and  to 
realize  how  long  they  have  gotten  on  in  the  outer  world  in  a  serious 
mentally  crippled  condition.  Of  course  their  continuance  in  the 
outer  world  was  made  possible  only  by  their  having  sunk  to  low  and 
relatively  simple  social  levels  that  made  little  or  no  demands  upon  them . 

When  such  patients  as  these  get  into  situations  which  require 
continuity  of  effort  and  constant  adjustment  and  from  which  they 
cannot  escape  they  not  infrequently  suffer  from  severe  and  more 
acute  breaks.  This  is  seen  in  the  military  service.  The  army  and 
the  na^y  naturally  attract  this  wandering  horde  of  inefficients  who 
see  in  the  military  service  only  the  glitter  of  brass  buttons  and  the 
opportunity  to  see  the  world.  After  enlistment,  however,  when  they 
are  required  to  take  up  the  grind  of  daily  work,  their  defects  soon 
come  into  the  foreground  and  manifest  themselves  either  by  dis- 
tinct psychotic  episodes  or  by  minor  uifractions  of  military  disci])line, 
such  as  staying  away  from  the  post  beyond  the  leave  granted,  or  per- 
haps more  serious  offences,  such  as  desertion.  Such  cases  when  they 
finally  come  under  observation  in  a  hospital  easily  show  their  defect. 

Many  women  of  this  type  marry,  have  children  and  although 
looked  upon  as  "unique,"  or  "queer,"  nevertheless  get  along  if  there 
are  no  serious  stresses. 

II.  Hebephrenia. — This  form  of  dementia  precox  is  more  severe 
than  the  preceding.  It,  however,  is  not  essentially  different,  bemg 
practically  the  same  condition  occurring  with  greater  severity,  more 
apt  to  be  somewhat  rrmr^'  nnit^^  ill  its  onset  and  manifesting  itself 
more  prominently  by  hallucinations  and  delusions. 


896 


DEMENTIA  PRECOX  GROUP 


The  onset  of  hebephrenia  not  infrequently  presents  the  outward 
appearance_"f  p,  flpprpssjrm  and  so  resembles  the  depressive  phase  of 
a  manic-depressive  psychosis,  or  perhaps  some  other  form  of  depres- 
sion. It  not  infrequently  happens  on  the  other  hand,  that  the  first 
appearances  of  the  disorder  are  those  rather  of  mental  confusion  than 
of  a  marked  depression.  These  early  manifestations  are  the  method 
of  reaction  of  the  individual  to  the  first  outbreak  of  the  conflict.  He 
may  become  either  greatly  depressed  or  may  be  quite  unable  to  react 
along  any  well  defined  line  and  become  quite  confused.  From  this 
acute  condition,  after  a  longer  or  shorter  duration,  he  settles  down 
into  a  more  stereotyped  expression  of  psychotic  symptoms. 

During  these  early  stages  the  productivity  is  not  infrequently  deli- 
rioid  in  character,  accusing  voices  are  heard  and  rather  ill-defined 
and  not  well  formulated^^jfas^fls^re  expressed  which  are  character- 
istically of  a  ^elf^ccTi^oryt^^^nd  in  harmony  with  the  depression. 
Attempts  at^uicid^notjn^e^^i^^  period. 

~Siter~the'^!ctive^  sympto'ms  of  the  onieTTiave^ubsid^^-^here  is  a 
settling  doT\Ti  into  a  more  stereot^^jed  delusional  expression,  but  not 
infrequently  with  a  more  or  less  incoherent  productivity  and  with 
expressions  that  sound  fantastic  and  silly.  One  patient  complains 
that  the  sheets  stick  to  his  feet,  another  that  he  is  the  "wandering 
planet."  Such  ideas  appear  to  have  no  adequate  reason  and  are 
expressed  quite  disconnectedly  from  the  general  train  of  thought  and 
little  or  no  attempt  is  made  to  support  them  by  logic.  One  patient, 
for  example,  says  that  his  enemies  are  following  him,  and  that  he 
has  been  killed  a  number  of  times.  Another  complains  that  other 
patients  are  trying  to  injure  him.  All  of  these  statements  are  made 
without  show  of  emotion  in  a  decidedly  matter-of-fact  way.  Such 
apjTearaTLces  astheseJiavjeJedjto^  descriptive  phrases  as  looseness 

ofthe  train  ofTfwught,  poverty  of  ideas,  emotional  deterior^jioiL  From 
tfe~preATmis~discTJSsion,  hjii^ever,  ot  the  m"eaning  of  s>^mptoms  it  is 
kno^Mi  that  theC^llucKations)are  expressions  of  the  conflict,  that  the 
delusions  are  compromise  formations,  and  that  the  apparent  inco- 
herency  of  the  speech  does  not  necessarily  imply  an  incoherency  in 
the  thought  content.  It  is  usually  not  especially  diflficult  to  find 
some  fairly  direct  connection  between  the  accusations  of  the  voices 
and  actual  occurrences  in  the  patient's  life.  Similarly  with  other 
disorders  of  the  sensorium.  One  patient,  for  example,  who  had 
seduced  a  girl,  who  bore  him  an  illegitimate  child,  saw  visions  of  his 
mother  and  heard  the  young  woman's  voice  telling  him  to  come  home, 
to  go  to  work  and  lead  a  decent  life. 

The  peculiar  emotional  reaction,  the  lack  of  interest  and  apparent 
emotional  dilapidation,  as  noted,  is  due  to  displacement,  and  so 
it  is  perfectly  understandable  that  when  an  effort  is  made  to  gain 
access  to  such  individuals  they  show  little  interest  and  character- 
istically reply  to  questions  addressed  to  discovering  the  reasons  for 
their  action  by  "I  don't  know."  It  is  really  quite  true  that  they 
do  not  know  the  reasons  for  their  actions  any  more  than  any  person 


SYMPTOM  GROU-PS  897 

understands  the  reasons  for  the  different  appearances  that  have  come 
to  him  in  a  dream.  And  then  again  these  patients  are  so  occupied 
with  themselves  that  they  do  not  care  often  to  be  interfered  with,  and 
the  "I  don't  know"  is  as  much  as  to  say  "leave  me  alone." 

The  general  conduct  of  the  patient  may  also  be  listless,  apathetic 
and  disinterested  in  character  and  in  harmony  with  his  emotional 
condition,  whereas  mild  alternations  of  depression  and  of  excitement 
with  the  development  of  little  peculiarities  of  conduct  and  speech 
show  the  alliance  between  this  group  of  cases  and  the  catatonics. 
One  patient,  for  example,  who  thought  that  he  was  ordained  to  preach 
and  that  bread  was  impure,  would  be  quiet  for  months,  and  then 
exhibit  a  certain  degree  of  restlessness  by  following  the  doctors  and 
nurses  about  the  ward  telling  them  his  troubles  for  a  few  days.  The 
alternation  may  be  much  more  marked  as  between  a  state  of  stupor 
and  a  state  of  excitement  in  which  the  patient  eats  paper,  strings, 
and  bedecks  himself  with  all  manner  of  trash  and  shows  outbreaks  of 
violent  anger.     This  condition  approaches  much  closer  to  catatonia. 

Many  of  the  cases,  however,  do  not  show  that  amount  of  dilapida- 
tion which  the  previous  description  would  imply,  while  any  degree 
of  incoherence  may  be  seen  up  to  the  production  of  a  veritable  "word 
salad."  On  the  other  hand,  the  patients  may  be  found  well  oriented, 
making  outwardly  a  natural  appearance,  being  able  to  give  a  very 
good  account  of  themsehes,  but  presenting  a  grotesque  delusional 
system,  supported  by  hallucinations  the  expression  of  which  stands 
in  rather  striking  contrast  to  the  outward  appearances.  These 
delusional  systems  represent  all  degrees  of  coherence  and  it  would 
seem  that  the  ability  on  the  part  of  the  patient  to  formulate  a  coherent 
and  consistent  delusional  workl  was  one  expression  at  least  of  his 
capacity  to  react  to  the  destructive  influences  of  the  conflict. 

One  such  patient  complained  of  illness  on  a  train  and  told  the 
conductor  that  she  had  had  a  hemorrhage.  Xo  sign  of  any  hemor- 
rhage was  in  evidence,  howe\'er,  and  when  she  reached  the  sanitarium 
to  which  she  was  removed  nothing  abnormal  was  found  on  examina- 
tion. When  her  mother  arrived  shortly  afterward  she  was  found 
delirious.  Later,  upon  her  admission  to  a  hospital  she  was  very 
hypochondriacal,  talked  at  length,  complained  of  attacks  of  hemor- 
rhage from  the  vagina,  which  hemorrhage  she  said  was  due  to  an 
assault  while  she  slept  either  by  a  man  or  some  sharp  instrument. 
Later  on  she  claimed  to  be  married  to  a  Mr.  O.  and  wrote  long  letters 
giving  an  account  of  her  troubles  and  giving  an  account  of  the  hemor- 
rhages and  the  supposed  ()j)erati()n,  showing  an  extensive  delusional 
sy.stem.  She  claimed  to  have  been  the  \ictini  of  a  criminal  operation 
performed  by  a  doctor.  She  later  said  that  she  had  been  engaged  to 
a  Hebrew  before  she  was  born  and  that  at  twent\'  she  broke  this 
engagement,  and  at  three  years  of  age  a  Catholic  priest  requested  one 
of  her  offspring  be  given  to  that  church  for  a  clergyman  and  that 
the  Hebrew  family  consented.  In  the  midst  of  a  great  mass  of  inco- 
iierent,  juml)led,  delusional  expressions  evidences  of  a  compensatory 
57 


898 


DEMENTIA  PRECOX  GROUP 


wish-fulfilling  delusional  system  are  found.  Wishing  for  a  child  she 
became  impregnated.  Being  a  virtuous  woman  this  has  to  be 
accounted  for.  She  therefore  has  the  delusion  that  she  is  married  to 
Mr.  O.  Inasmuch  as  no  child  appears,  a  delusion  that  shj  has  had 
an  abortion  performed  accounts  for  its  absence,  and  as  this  is  a  crim- 
inal operation  it  was  performed  without  her  knowledge  while  she  slept. 
These  delusions  are  mixed  up  with  all  sorts  of  ideas  about  the  dislo- 
cation of  her  various  viscera,  numerous  hemorrhages  from  the  vagina, 
and  a  fractured  skull,  etc.  It  is  loosely  organized  and  interrupted 
by  violent  outbreaks,  restlessness  and  irritability. 

She  has  been  unable  to  adjust  to  reality,  the  effort  at  compensation 
by  delusional  formation  has  also  failed,  with  the  resulting  progressive 
dilapidation  of  coherence  in  the  stream  of  thought. 


Fig.  440. — Appearance  of  patient  in  catatonic  stupor. 


lll.^^atatcmm. — This  variety  of  precox,  like  the  hebephrenic,  may 
come  oiisucldemy  with  symptoms  of  confusion  or  depression,  or  may 
be  of  somewhat  slower  onset.  It  is  more  apt  than  the  other  forms  to 
be  of  relatively  acute  onset,  in  which  case  it  sometimes  follows  a  sud- 
den shock  of  a  highly  emotional  character.  It  is  characterized  more 
especially  by  a  predominance  of  motility  disturbances  and  tends  to 
express  itself  in  alternating  conditions  of  catatonic  stupor  and  catatonic 
excitement. 

In  the  conditions  of  catatonic  stupor  negativism  reaches  a  very  high 
degree.  The  patients  are  perfectly  immobile,  sitting  off  in  corners  by 
themselves  or  lying  in  bed  without  paying  any  apparent  attention  to 
what  goes  on  about  them  and  are  quite  inaccessible,  fail  to  answer 
questions,  and  do  not  react  at  all  to  stimuli  from  the  outer  world. 
They  characteristically  often  refuse  to  speak  at  all.  This  mutism  is 
a  manifestation  of  the  negativism.  Besides  this  the  patients  often 
refuse  food,  pay  no  attention  to  the  promptings  of  the  bladder  and 
the  rectum  which  become  overloaded  with  urine  and  fecal  matter, 
often  to  a  serious  extent.  Saliva  may  be  permitted  to  accumulate  in 
the  mouth  where,  if  attention  is  not  paid  to  it,  it  may  undergo  putre- 
factive changes.     The  patients  quite  characteristically  show  peculiar 


SYMPTOM  GROUPS  899 

theatrical  attitudinizing,  make  grimaces,  occupy  peculiar  positions, 
and  if  they  speak  the  producti\-ity  is  often  incoherent  and  apparently 
senseless,  with  a  tendency  to  constant  repetition  of  the  same  phrases 
—perseveration— which  may  also  manifest  itself  in  the  movements  of 
the  body,  such  as  a  constant  swaying  movement  or  the  like. 

The  muscular  system  may  be  in  a  condition  of  waxy  flexihility, 
permitting  of  the  molding  of  the  limbs  into  any  position  where  they 
remain  indefinitely— cofo/^-ps?/.     When  this  is  present  the  patients 


FiLi.  -141. — Catalepsy;  flexibilitas  cerea. 

tend  to  show  a  more  or  less  high  degree  of  suggestibility  and  command 
automatism,  doing  mechanically  and  in  a  perfectly  automatic  manner 
that  which  they  are  commanded  to  do.  This  suggestil)ility  shows 
itself  further  in  echolalia,  the  repetition  of  words,  or  phrases  that  are 
addressed  to  them  or  that  they  hear  others  speak,  and  echopraxia, 
the  repetition  of  movements  which  are  made  in  their  presence.  On 
the  other  liand,  tiiere  may  be  a  marked  degree  of  muscular  tcnsiun, 
the  patient  maintaining  fixed  attitudes  with  the  muscles  thrown  into 


900  DEMENTIA   PRECOX  GROUP 

a  condition  of  rigid  contraction.  The  limbs  and  body  are  stiff  and 
immobile  and  resist  any  effort  at  bending.  Patients  in  this  condition 
are  positively  negativistic,  withdrawing  from  all  approaches,  refusing 
any  cooperation  with  the  nurse  in  attempting  to  dress  or  undress  or 
feed  them,  and  show  a  tendency  to  react  by  doing  the  opposite  of 
what  is  expected  of  them.  In  this  condition  of  stupor  the  patients 
may  appear  to  be  quite  disoriented  and  have  no  knowledge  of  what 
is  going  on  about  them.  If  they  are  watched,  however,  they  may  be 
seen  at  times  to  show  evidences  of  paying  attention  to  their  environ- 
ment, and  not  infrequently  when  the  stupor  passes  they  are  able  to 
give  a  fairly  good  account  of  the  things  that  happened  during  it,  but 
show  no  capacity  for  explaining  their  strani^jconduct. 

In  the  opposite  condition  of  (fat(^^^7xcitemeut\there  are  marked 
degrees  of  activity,  constant  taffiativeness  and"  noisiness,  sometimes 
destructive  and  impulsive  tendencies  manifested  by  breaking  windows 
or  attacking  those  about  them,  but  in  general  showing,  as  does  the 
speech,  a  marked  lack  of  coherence.  Both  the  productivity  and  the 
activities  of  the  patient  fail  to  show  any  clear  goal,  although  some 
patients  show  what  very  closely  approaches  to  the  flight  of  ideas  of 
the  manic.  Catatonic  excitement  may  reach  a  very  high  grade,  mani- 
festing itself  by  wildly  delirioid  reactions,  constant  motor  unrest  and 
sleeplessness,  a  rapid  failure  in  nutrition,  a  veritable  status  catatoni- 
cus.  Some  of  these  catatonic  cases  show  hysteriform  or  epileptiform 
seizures,  and  death  occasionally  results  in  these  higher  grades  of 
excitement. 

The  chronic  conditions  tend  to  show  well  defined  mannerisms,  such 
as  the  peculiar  attitudes  of  the  body,  especially  clumsy  ways  of  holding 
the  spoon  or  the  fork  in  eating,  meaningless  grimaces,  odd  ways  of 
walking,  such  as  sliding  the  foot  back  and  forth  two  or  three  times 
before  starting  oft'  and  other  ceremonials  for  initiating  movements. 
They  are  characteristically  stiff',  awkward,  clumsy  and  inaccessible, 
and  usually  indift'erent  to  their  surroundings  and  apparently  emotion- 
ally dull,  though  given  at  times  to  emotional  outbreaks  without 
apparent  cause.  All  of  these  psychical  symptoms  of  course  must  be 
considered  as  having  some  psychological  meaning.  In  harmony 
with  what  has  been  said  before  both  the  types  of  increased  suggesti- 
bility and  catatonic  rigidity  are  ways  of  shutting  out  the  world  of 
reality,  whereas  the  peculiar  automatisms  can  sometimes  be  traced  to 
their  meanings,  as  in  the  case  before  mentioned  of  the  young  woman 
who  kept  constantly  pounding  her  hand  with  her  clenched  fist. 

Physical  Symptovis. — The  general  appearance  of  the  catatonic 
forms  of  precox  indicate  more  than  in  the  other  varieties  the  presence 
of  definite^j(2Sl^tic  accompaniments.  Exaggeration  of  the  tendon 
reflexes,  lou'ermg  ot  cutaneous  sensibility,  vasomotor  disturbances, 
cold  and  cyanosed  extremities,  widely  dilated  pupils,  disturbances  of 
secretion,  and  loss  of  weight  are  frequently  observed.  It  is  this 
group  of  cases  that  have  led  most  distinctly  to  a  toxic  theory  of 
etiology  and  caused  the  precox  cases  to  be  thought  of  in  connection 


SYMPTOM  GROUPS 


901 


with  disturbances  of  metabolism  flue  to  changes  in  the  internal 
secretiongii,,, __^_^ 

l^^^ammM  — In  the  paranoid  cases  there  is  a  much  more 

efficient  efforTat  creating  a  coherent  and  logically  connected  series  of 
delusions  and  associated  nallucmations.  In  these  cases  there  is  much 
less  outward  evidence  ot  the  dilapidation  and  emotional  indifference 
that  have  led  to  the  belief  in  the  presence  of  a  permanent  mental 
impairment,  a  dementia. 

These  patients,  originally,  many  of  them  at  least,  were  grouped 
with  the  paranoias,  but  under  the  influence  of  Kraepelin  that  group 
of  paranoid  conditions  which  showed  a  tendencN-  toward  protrressive 
deterioration  were  included  in  the  dpTnpni-ia  pv^^r^v  r-i;^^mtipatinn, 
All  degrees  of  intervening  possibilities,  however,  occur,  so  that  one 


Fig.  442. — Dementia  precox;  mannerism. 


Fig.  44.3. — Dementia  precox ; 
mannerism. 


may  get  a  fairly  well  knit  delusional  system  in  a  patient  who  deterio- 
rates quite  rapidly,  whereas  other  patients  maintain  their  intellectual 
integrity  over  a  period  of  years.  The  delire  chronique  of  Magnan  is 
generally  considered  to  be  dementia  paranoides  and  this  group  of 
cases  in  general  has  been  recently  included  by  Kraepelin  under  the 
designation  of  jxirajihrenia.  Some  authors  today,  more  i)articularly 
Bleuler,  are  inclined  to  think  that  jx'rhaps  the  so-called  true  paranoias 
are  only  attenuated  forms  of  dementia  precox. 

The  delusional  system  in  these  cases  must  be  conceived  as  a  com- 
promise formation  and  as  essentially  wish-fulfilling  and  is  character- 
istically sexually  colored.  One  such  patient,  a  middle-aged  woman, 
was  persistently  persecuted  by  a  man  who  accused  her  of  leading  an 

'  Jelliffe:  Dementia  Precox  and  the  Vegetative  Nervous  System.  N'.  Y.  Med.  Jour., 
May,  1917, 


902  DEMENTIA   PRECOX  GROUP 

immoral  life  to  such  an  extent  that  she  finally  went  to  a  physician  to 
be  examined  to  prove  her  virginity.  All  sorts  of  vulgar  remarks  were 
constantly  made  about  her,  she  was  referred  to  by  the  ^'oices,  as  the 
widow  of  this  man.  The  patient  was  a  devout  Catholic  and  the  man 
was  a  Protestant,  a  perfectly  understandable  reason  for  a  severe 
emotional  conflict,  which  in  her  delusional  system  unloads  itself  upon 
the  man  rather  than  acknowledge  its  true  origin.  This  whole  con- 
flict arose  at  the  time  of  the  death  of  her  brother  and  it  is  significant 
that  in  her  delusions  she  believed  that  she  had  received  letters  accus- 
ing her  of  incestuous  relations  with  her  brothers,  of  having  become 
pregnant  by  them  and  of  destroying  the  pregnancy.  These  letters  were 
addressed  to  her  as  the  wife  of  her  several  brothers.  Here  one  sees 
an  infantile  determiner  for  the  delusional  system  in  the  love  which 
she  entertained  for  her  brothers  and  which  later  was  transformed, 
under  the  influence  of  the  conflict,  into  delusions  of  an  incestuous 
nature.  Finally,  there  was  a  whole  crowd  of  people  who  were  conspir- 
ing against  her  and  whose  object  it  was  to  kill  her.  Such  delusional 
systems  can  without  much  difficulty  be  seen  to  be  expressions  in  dis- 
torted form  of  the  conflict.  She  has  never  been  able  to  get  away 
from  her  infantile  attachment  to  the  members  of  her  family  and 
establish  herself  upon  her  o^n  feet,  and  these  attachments  hold  her 
back,  produce  a  withdrawal  from  the  outer  world,  a  regression  into 
the  world  of  fantasy,  with  a  tendency  toward  what  may  be  termed 
psychic  death,  or  an  absolute  lack  of  efficient  reaction  to  reality,  and 
this  is  symbolized_b}'  the  gang  of  persecutors  who  are  bent  upon  her 
destruction. 

Y.(j^ixed  and  Atypical  States^ — All  of  these  forms  of  dementia 
precox  so  far  described  are  sniiply  variants,  in  accordance  with  the 
present  viewpoint,  of  one  disease  trend,  and  so  it  is  not  strange  that 
it  should  be  found  that  there  are  no  hard  and  fast  lines  separating  one 
from  the  other.  In  consequence  it  is  not  infrequent  to  see  transitory 
or  mild  motility  disturbances  in  cases  that  are  essentially  hebe- 
phrenic or  to  see  fairly  well  defined  paranoid  delusional  formations 
in  either  the  hebephrenic  or  the  catatonic  varieties. 

As  to  the  atypical  forms,  there  have  been  mentioned  and  described 
under  dementia  simplex  the  larvated  and  abortive  types,  the  "formes 
frustes."  These  are  probably  much  more  frequent  than  ordinarily 
supposed.  For  example,  Wilmanns,  in  a  study  of  127  vagabonds, 
found  6()  cases  of  dementia  precox,  while  undoubtedly  it  is  not  infre- 
quent for  these  abortive  forms  to  be  considered  as  cases  of  constitu- 
tional defect.  This  difficulty  becomes  very  much  more  aggravated 
when  it  is  remembered  that  attacks  of  dementia  precox  may  occur 
in  childhood,  according  to  Vogt  as  early  as  five  years  of  age,  while  a 
little  later,  nearer  to  the  period  of  puberty,  they  are  not  so  uncommon. 
In  these  cases  of  course  mental  development  is  interfered  with  and 
remaining  upon  a  lower  level  gives  the  impression  of  imbecility.  The 
same  difficulty  arises  in  a  somewhat  aggravated  form  when  dementia 
precox   develops   in  young  persons   already  of  defective   make-up. 


NATURE  OF  DEMENTIA   PRECOX  903 

Here,  of  course,  the  differentiation  is  no  longer  possible,  because  it 
must  be  realized  that  both  conditions  of  defect  and  precox  are  present 
in  the  same  individual. 

Pathology. — ^There  is  a  considerable  pathology  of  precox  which, 
however,  is  not  always  cleat-jy  correlated  with  the  clinical  picture. 
It  is  probable  that  dm-mg  the  life  of  the  individual  disturbances  that 
are  shown  in  the  general  physical  manifestations  of  the  disease  occur 
at  the  biochemical  level.  Beginning  degenerative  changes  in  the 
vessels  are  sometimes  found,  and  tuberculosis  is  quite  frequent,  but  is 
easily  understandable  as  being  dependent  upon  the  generally  lowered 
physical  condition  and  inactivity  of  these  patients,  coupled  with  their 
bad  habits  and  the  necessity  of  their  rather  close  confinement.  Such- 
cjiangps  as  arc  found  in  the  nerve  cells  are  of  a  degenerative  character, 
with  evidences  of  neuronophagia  and  with  perhaps  ameboid  glia  cells. 
The  acute  cases  of  catatonic  excitement  which  end  fatally,  the  so-called 
catatonic  "Hirntod,"  show  a  certain  amount  of  evidence  of  degenera- 
tion, more  particularly,  however,  evidences  of  a  chemical  nature  in 
the  form  of  certain  lipoid  elements  in  the  cortex,  which  seem  to  be 
products  of  disintegration. 

Southard  has  found  certain  anomalies  in  precox  brains  which 
appear  to  be  of  the  nature  of  aplasias  or  ageneses.  These  agenetic 
or  aplastic  areas  appear  to  be  grouped  more  or  less  in  correspondence 
with  the  three  main  tjpes  of  the  disease.  The  frontal  region  is  char- 
acteristically involved,  while  he  has  described  a  cerebellar  group  cor- 
responding to  the  catatonic  variety,  while  the  profound  emotional 
disturbances  he  thinks  are  due  to  lesions  in  the  deep  layers  of  the 
cortex  which  have  no  direct  motor,  sensory  or  perhaps  associational 
relations.  These  changes  and  perhaps  many  others  will  probably 
be  illuminated  by  an  understanding  of  the  mechanisms  at  the  vegeta- 
tive nervous  system  level. 

Nature  of  Dementia  Precox. — From  the  description  of  dementia 
precox  up  to  this  point  it  will  be  seen  that  it  presents  correlations  on 
the  one  hand  to  the  more  distinctly  so-called  psychogenic  types  of 
disorders,  such  as  the  psychoneuroses,  and  on  the  other  hand  to  the 
more  distinctly  somatic  diseases.  It  has  been  seen  that  it  was  possible 
to  formulate  all  of  the  mental  symptoms  in  the  same  way  that  they 
are  formulated  in  the  psychoneuroses.  In  other  words,  the  mental 
symptoms  are  capable  of  interpretation  solely  at  the  psychological 
level.  On  the  other  hand,  for  an  understanding  of  the  whole  disease 
process  it  must  not  be  lost  sight  of  that  recent  inyestij^ations  are  tend- 
ing to  sh"ow"more  and  more  that  there  are  distuK-tbiocTicn^^  clis:;^ 
Turbances  during  life  and  pathological  changes  arcT)cing  foumUifteT 
death!  In  any  case  the  acute  cases  that  lead  to  death  must  be  coii- 
ceived'  as  having  profound  bodily  changes  correlated  with  psychic 
sjTnptoms.^ 

1  Jung:  Psychologj'  of  Dementia  Precox;  Spielrein:  Analysis  of  a  Case  of  Dementia 
Precox;  Binswanger:  Dementia  Precox.  See  Abstracts  in  Psychoanalytic  Review,  vols, 
ii,  iii,  iv. 


904  DEMENTIA   PRECOX  GROUP 

The  foniuilation  of  the  disease  from  either  staiKlpoint  alone  must 
of  necessity  be  unsatisfactory.  For  example,  the  theory  which  makes 
the  etiology  toxic  fails  utterly  to  give  a  comprehensive  idea  of  why 
the  mental  manifestations  take  the  particular  form  they  do,  why  for 
example,  in  the  case  already  cited,  the  hallucinatory  voice  should  be 
that  of  a  young  woman  that  the  patient  had  seduced  telling  him  to 
lead  a  decent  life.  This  is  surely  the  patient's  self-critique  appearing 
in  hallucinatory  form. 

It  Avould  seem,  especially  in  view  of  the  type  of  archaic  reactions 
which  have  been  described  that  when  the  type  of  splitting  of  the 
personality  is  very  severe  then  the  term  dementia  precox  or  schizo- 
phrenia applies.  It  is  possible  that  the  somatic  processes,  whatever 
they  may  be,  aid  in  this  deeper  regression  to  the  infantile  and  the 
archaic  in  the  individual  and  which  therefore  have  been  less  efficiently 
sublimated  and  thus  permit  the  older  phylogenetic  thought  symbols 
to  appear  in  conduct. 

At  any  rate,  it  seems  quite  evident  that  the  great  difficulty  of 
conception  here  is  due  in  part  to  the  quite  arbitrary  separation  of  the 
individual  into  two  distinct  and  mutually  exclusive  parts,  namely, 
m  ind  and  body.  The  dualistic  hypothesis  that  conceives  of  the  mental 
and  the  physical  going  side  by  side  without  mutual  interference  or 
interaction  is  responsible  for  such  a  conception.  From  the  medical 
standpoint  such  a  concept  seems  entirely  unnecessary,  for  one  is 
met  at  every  turn  with  the  intimate  relations  constantly  maintained 
between  the  two,  and  therefore  comes  naturally  to  consider  the  human 
being  as  a  biological  unit  presenting  types  of  reactions  that  at  one 
extreme  are  predominantly  psychic,  w^hile  at  the  other  they  are  pre- 


EXPLANATION  OF  PLATE  XII. 

The  drawing  was  made  with  an  Edinger  drawing  apparatus  from  colored  photomicro- 
graphs of  the  original  sections.  The  fatty  material  is  stained  red  by  Scharlach  R  and 
Ehrlich's  hematoxylin  is  used  as  a  counterstain  for  the  cell  body  and  nucleus. 

ax.  axis-cylinder  of  cell. 
nl.  nucleolus. 
gl.  neuroglia. 

Fig.  1. — Three  ganglion  cells  from  the  3d  layer  of  the  cerebral  cortex  of  the  frontal 
lobe,  from  a  case  of  dementia  precox  twenty-seven  years  of  age  (all  three  of  these  cells 
were  found  in  one  fluid  f.,  oil  immersion),  a,  Entire  cell  Ijody  filled  with  fat,  only  nucleus 
and  nucleolus  free.  b  and  c,  Characteristic  fatty  deposit  in  base  of  cell  and  in  axis- 
cyUnder  which  is  much  swollen  and  affected  to  the  point  of  myelinization. 

Fig.  2. — A  medium-sized  ganglion  cell  from  third  layer  of  cortex  in  another  case  of 
dementia  precox,  aged  thirty  years,  with  fatty  axis-cylinder  and  base  of  cell  filled  with 
fat  In  normal  brains  of  this  age  practically  no  fat  is  found  in  cells  of  this  type,  shown  in 
Figs.  1  and  2. 

Fig.  3.— Giant  Betz  cell  from  the  motor  cortex  in  a  case  of  senile  dementia,  aged 
sixty-nine  years.  A  considerable  amount  of  fat  in  the  base  of  the  cell.  In  normal  brains 
these  large  motor  cells  contain  some  fat,  but  never  as  much  as  shown  in  this  cell. 

Fig.  4. — Section  trom  the  superficial  layer  of  the  cortex,  showing  neuroglia  cells  filled 
with  fat,  from  a  case  of^dementia  precox,  aged  thirty  years.  Normal  brains  at  this  age 
show  absolutely  no  fat  in  the  superficial  neuroglia.  The  amount  of  fat  is  only  found  in 
senile  dementia. 


PLATE   XII 


I 


ctX 


7         • 


# 


4 


¥ 


4. 


NATURE  OF  DEMENTIA   PRECOX 


905 


dominaiitly  physical,  hut  which  present  every  grade  of  intermediary 
type.  The  se\'ere  mental  disturbance  of  the  nature  of  anxiet\-  that 
goes  with  certain  cardiac  and  pulmonary  diseases  is  well  known,  as 
are  also  the  physical  upsets,  particularly  the  gastro-intestinal,  that 
are  associated  with  certain  mental  conditions  such  as  worry.  The 
interrelations  between  the  mental  and  the  physical  are  a  matter  of 
daily  observation.  Occasionally,  how- 
ever, they  are  very  pronouncedly  empha- 
sized. A  chronic  patient  who  had  been 
for  many  years  in  a  hospital,  working 
daily  at  outdoor  labor,  was  suddenly 
seized  with  a  violent  impulse  in  which 
he  attacked  all  about  him.  He  seized  a 
heavy  iron  bar,  killed  two  people  and  in- 
jured another  and  ran  headlong  and  wildly 
wnthout  direction  into  the  woods.  He 
was  finally  cornered,  and  in  the  process 
of  securing  him  he  was  shot  by  a  farmer 
with  a  load  of  buckshot,  none  of  which, 
however,  penetrated  farther  than  through 
the  skin  and  produced  no  serious  wounds. 
He  was  brought  back  to  the  hospital, 
incoherent,  mumbling  and  trembling, 
showing  all  the  evidences  of  a  tremendous 
emotional  upset.  The  shot  were  picked 
out  of  his  skin,  the  wounds  were  dressed 
and  he  was  put  to  bed.  Up  to  that  time 
he  had  been  a  strong,  physically  healthy 
negro.  He  never  left  his  bed  again  and 
approximately  a  year  afterward  he  died, 
having  de\-eloped  an  acute  tuberculosis. 
Such  cases  as  these  demonstrate  the 
necessity  of  considering  the  human  being 
as  a  unit  and  not  endeavoring  to  draw 
hard  and  fast  lines  of  distincticm  between 
the  mind  on  the  one  hand  an<l  the  body 
on  the  other. 

In  the  present  state  of  knowledge,  how- 
ever, one  is  often  unable  to  make  any 
specific  correlation  between  the  physical 
findings  and  the  mental  symptoms,  while 
on  the   other  hand   it   is   quite  possible 

to  express  the  symptomatology  of  the  disease,  to  describe  it,  to,  so  to 
speak,  reconstruct  the  psychosis  ])urely  in  i)sychological  terms.  For 
the  present,  therefore,  the  disease  must  be  described  i)sychologicall>'. 
and  the  explanation  of  the  mental  sym])toms  must  be  sought  psycho- 
genetically,  without,  however,  forgetting  that  there  are  certain  somatic 
changes  which  are  pretty  generally  attached  to  the  sym])t<)inat()l()gy 


Fici.  444. — Faiita.-itic  i):ir:uioia. 


906 


DEMENTIA   PRECOX  GROUP 


of  the  disease  process  and  which  must  ultimately  be  made  to  fit  into 
the  general  rubric  before  a  complete  understanding  of  the  entire 
situation  is  had. 

On  the  psychologicalside^tilien,  dementia  precox  is  seen  to  be  a 
certain  type  of  reactioTPtcTarmental  conflict,  resultnig  m  aTspTitHng 
dTtHepsyche  and  the  outcrop  of~unconscious  mental  trends__to_tbe 
surface7)f  the  mental  life.  The  patient  is  confronted  with  a  situation 
"to  whiclihe  cannot  adequately  adjust,  which  is  absolutely  inaccept- 
able  and  impossible,  and  he  is  therefore  driven  away  by  his  incapacity 
to  assimilate  it  and  cast  back  upon  himself.  The  battle  of  the  oppos- 
ing forces  produces  the  disease  picture  which  is  the  outward  evidence 
of  the  effort  on  the  part  of  the  individual  to  reach  a  solution  of  the 
difficulty.  The  symptoms  are  the  results  of  the  appearance  of  the 
unconscious  trends  distorted  and  disguised  as   they  are    in  dream 

formations.  Jung  has  especially 
noted  this  similarity  to  the  dream 
state  and  would  consider  precox  as 
a  sort  of  waking  dream  or  dream 
from  which  the  patient  does  not 
awake,  the  dream  picture  being 
fixed,  as  it  w^ere,  by  another  element 
in  the  situation,  the  toxin,  which 
for  the  sake  of  analogy  solely,  may 
be  said  to  act  like  the  fixing  agent 
in  the  photographic  process.  It  is 
seen  that  the  conception  of  the 
disease  as  being  from  the  beginning 
and  fundamentally  a  dementia  must 
be  modified,  if  by  dementia  is  meant 
a  permanent  mental  impairment,  a 
mental  loss,  which  is  thought  of  in 
the  same  terms  as  a  loss  of  tissue 
from  a  wound.  It  w^ould  appear 
from  the  description  of  the  mechan- 
isms that  have  been  given  that  at  least  in  the  early  stages  of  the  disease 
there  is  nothing  at  all  corresponding  to  such  a  permanent  loss;  that 
what  has  happened  has  been  a  disintegration,  a  falling  apart  of  the 
component  parts  of  the  psyche,  a  shifting  of  emphasis  to  the  uncon- 
scious factors  in  the  conflict,  more  particularly  a  shifting,  or  to  use 
the  technical  term,  a  displacement  of  the  emotional  content  of  certain 
idea  constellations.  The  dementia,  therefore,  which  has  been 
described  as  such,  is  at  least  to  this  extent  a  misnomer.  From 
the  point  of  view,  however,  that  this  disintegration  and  resulting 
impairment  is  either  permanent  or  tends  to  be,  it  has  a  meaning. 

The  psychological  side  of  the  situation,  however,  as  may  be  seen, 
is  not  all.  There  are  certain  physical  changes  in  the  course  of  the 
disease  and  certain  pathological  findings.  So  far  as  the  observation 
goes,  however,  the  etiological  factors  lie  almost,  if  not  quite  entirely, 


Fig.  445.— The  fetal  attitude  assumed 
in  severe  grades  of  regression.  This  at- 
titude was  maintained  most  of  the  time 
for  years,  the  patient  seeking  the  darkest 
corner  of  the  ward. 


NATURE  OF  DEMENTIA   PRECOX  907 

in  the  mental  sphere  and  one  must  therefore  conceive  of  the  physical 
changes  as  superadded.  This  is  a  possibility  which  was  well  illus- 
trated by  the  case  already  quoted  of  the  man  who  died  after  a  tre- 
mendous emotional  explosion  during  which  he  killed  two  people. 
When  the  psychic  splitting  is  profound  and  when  it  is  of  considerable 
duration  it  is  quite  understandable  that  it  should  unloose  bits  of 
physiological  mechanism  and  thus  produce  the  physical  changes 
found.  From  the  descriptions  of  the  mechanisms  in  the  psycho- 
neuroses,  taken  in  connection  with  the  discussion  of  the  vegetative 
nervous  system,  it  may  be  seen  that  constantly  operatiA-e  psychic 
disturbances  are  capable  of  producing  the  physical  changes .  Compare 
Crile's  study  of  the  emotions  and  Cannon's  work  on  the  relation  of 
anger  and  fear  to  the  gastro-intestinal  functions  where  it  may  be  seen 
that  both  surgeon  and  physiologist  are  forced  to  put  the  psychic 
factor  in  the  foreground. 

As  already  indicated,  dementia  precox  is  an  introversion  psvchosis(. 
that  is,  instead  of  running  awav  from  his  conflict  into  realitv  as  does^ 


the  manic-depressive,  the  patient  turns  bg^'lc  and  att^^ipts  to  retxace 
his  footsteps  upon  the  path  of  development.  Introversion,  however,  is 
a  chnTacterisLic  uf  many  Lypey  of  mental  illness,  some  of  the  neuroses, 
for  example,  and  occurs  in  the  course  of  other  illnesses  such  as  paresis. 

Introversion  to  some  degree  is  also  a  quite  common  mechanism  in 
otherwise  healthy  people,  especially  when  confronted  by  difficulties, 
while  whole  races,  the  Orientals,  seem  to  be  characterized  by  an 
emphasis  on  the  introversion  side  of  their  mental  mechanisms. 

To  describe  precox  then,  as  an  introversion  psychosis  merely  desig- 
nates its  most  characteristic  quality,  it  does  no  more  than  this.  Why 
does  introversion  become  so  malignant  in  these  cases?  That  is  the 
question  to  be  answered.  A  number  of  suggestions  only  can  be 
offered,  any  one  or  all  of  several  factors  may  be  active.  The  depth 
of  the  introversion  is  probably  important  but  the  stratigraphy  of  the 
psyche  is  not  well  enough  known  to  decide  this  question.  ]\Iany 
patients  do,  however,  get  worse  in  proportion  to  the  distance  on  the 
regressive  pathway  that  their  difficulties  force  them.  Then  again 
the  strength  of  the  fixation  is  of  undoubted  importance  but  perhaps 
of  greater  importance  to  prognosis  for  many  patients  with  profound 
regression  get  well,  often  rapidly,  which  would  inflicate  that  the 
strength  of  the  fixation  was  not  great.  The  factors  which  make 
for  possibility  of  rebound  are  not  kno^vn  though,  in  general,  when 
the  etiological  factors  are  largely  exogenous  and  can  be  removed 
recovery  is  more  to  be  expected  as  in  the  war  neuroses  and  the  prison 
psychoses.  Finally  I  have  indicated  that  regression  may  involve 
bits  of  physiological  mechanism  and  so  tend  to  fix  the  regression  and 
prevent  the  recovery.  This  latter  element  is  bound  up  in  the  con- 
ception of  inferior  organs.  It  is  often  impossible  to  say  whether  an 
organic  difficulty  has  been  produced  by  the  regression  or  whether  it  con- 
ditioned the  regression  in  the  first  instance.  In  other  words,  whether 
the  etiology  is  primarily  psychogenic,  or  organic,  or  whether,  on  the 


908  DEMENTIA   PRECOX  GROUP 

other  hand,  organic  and  psychological  manifestations  are  not  both  the 
outward  manifestations  of  an  inherent  de^'elopmental  anomaly. 

The  general  tendency  at  present  is  to  think  of  the  psychological 
conflict  as  primary  and  the  various  symptoms  of  disordered  function 
at  the  physiological  level  as  secondary.  Circulatory  and  glandular 
imbalances  and  functional  disturbances  of  various  organs  are  thus 
explained  as  results  of  the  primary  emotional  disturbances  and  the 
organic  changes  as  results  when  such  functional  disorders  are  main- 
tained for  a  long  period  and  are  severe  in  character.  In  many  instances 
primary  defects  of  the  personality  undoubtedly  found  in  organic 
defects  and  such  defects  as  well  as  those  that  are  more  obviously 
secondary,  would  appear  to  be  mediated  through  the  vegetative 
nervous  system.  They  would  include  glandular  (gonadal,  etc.)  defi- 
ciencies and  imbalances,  gastro-intestinal,  cardiovascular  and  other 
organic  inadequacies,  and  organic  deficiencies  such  as  the  aplasias 
of  the  central  nervous  system.  There  is  a  wide  field  here  for  correlat- 
ing generalizations  which  will  undoubtedly  show  precox  to  be  the 
result  of  relatively  severe  processes  which  in  less  severity  occur  in 
prognostically  less  serious  conditions  such  as  certain  of  the  neuroses. 

From  their  point  of  view  then  precox  is  an  especially  severe  mani- 
festation of  the  individual's  inadequacy  to  meet  the  biological  demands 
made  upon  him  to  fulfil  his  biological  destiny.  The  theory  of  a 
toxemia  does  not  help,  as  such,  to  understand  the  results  but  on  the 
other  hand  the  toxemia  can  be  understood,  if  it  exists,  as  resulting  from 
the  general  functional  disturbances  incidental  to  his  unsuccessful 
struggle  with  his  problems.  Only  under  some  such  comprehensive 
formula  as  here  indicated  is  it  possible  to  arrange  any  of  the  multi- 
tudinous manifestations  of  this  type  of  mental  illness. 

A  study  of  the  dominating  visceral  tonicities  and  postural  tensions 
in  individual  cases  will  demonstrate  the  nature  of  the  psychological 
conflict  because  it  will  show  the  organs  that  are  in  a  state  of  pre- 
paredness for  carrying  out  unconscious  trends  which,  however,  are 
not  permitted  to  express  themselves  but  are  kept  inoperative  by  the 
force  of  repression. 

Ways  of  Getting  Well. — With  the  concept  of  the  disease  process 
which  has  been  previously  elaborated,  what  is  the  significance  of  the 
three  main  types  of  precox  which  are  found  clinically?  Bertschinger^ 
has  recently  made  an  admirable  study  of  the  process  of  recovery  in 
precox  and  his  discussion  of  the  nature  of  the  conflict  and  the  ways 
of  adjusting  is  particularly  illuminating. 

The  three  clinical  tA^^es  of  the  disease  are  the  expression  of  the 
interplay  of  the  two  factors,  the  conflict  and  the  reaction,  the  severity 
of  the  former  and  the  efficiency  of  the  latter  determining  the  outcome 
in  the  individual  case.  The  degree  of  confusion  in  the  acute  onset 
would  then  be  an  expression  of  the  completeness  with  which  the 
patient  was  driven  back  from  reality  and  the  dominance  of  the  uncon- 

1  Heilungsvorgange  bei  Schizophrenen,  AUg.  Ztschr.  Psychiat.,  Band  Ixviii,  Heft  2. 
Translated  in  Psychoanalytic  Review,  April,  1916,  iii,  No.  2. 


WAYS  OF  GETTING  WELL  909 

scious  trends.  Conditions  of  moderate  confusion  with  capacity  for 
adequate  reaction  to  reality  at  times,  or  under  the  special  stimuli  of, 
for  example,  questions,  show  that  the  patient  still  has  a  certain  grip 
upon  the  real  world  and  is  making  an  effort  at  least  to  retain  it. 
Certain  other  cases  of  quite  clear  consciousness  with  complete  orien- 
tation show  a  very  adequate  grasp  upon  reality  and  these  patients,  to 
the  casual  observer,  often  seem  quite  natural.  In  such  patients, 
however,  one  will  notice  interferences  of  thought,  hesitations  in  the 
course  of  conversations,  stutterings  and  stammerings  over  certain 
points,  the  evidences  in  other  words,  of  complex  interferences  and  the 
patients  will  complain  that  from  time  to  time  their  minds  seem  to  be 
absolutely  blank.  These  saltatory  associations  and  thought  depriva- 
tions are  the  expressions  of  reactions  to  buried  complexes,  so  that  in 
these  patients  there  is  a  fairly  adequate  grasp  upon  reality  for  ordinary 
purposes  at  least,  with  only  spasmodic  influences  from  relatively 
restricted  areas. 

In  general,  then,  the  hebephrenic  type  may  be  seen  to  be  a  reaction 
to  the  conflict  which  is  essentially  inadequate  and  inefficient.  In  the 
acute  stages  the  patient  may  be  absolutely  overwhelmed  by  the 
conflict,  disoriented  and  confused.  Later  on  the  progressive  disinte- 
gration and  dilapidation  of  thought  indicates  the  slowly  progressive 
conquering  of  the  capacity  for  adjustment  to  reality  by  the  invasion 
into  consciousness  of  the  unconscious  trends. 

The  catatonic  tApe  represents  a  somewhat  different  form  of  reaction. 
Here  the  patient  is  oftentimes  suddenly  overwhelmed  by  the  conflict, 
as  under  circumstances  of  accident  or  sudden  and  severe  shock.  Xo 
attempt  at  adjustment  is  made  at  first,  but  the  whole  situation  is 
actively  and  definitely  shut  out.  Here  there  is  an  active  effort  on 
the  part  of  the  individual  to  exclude  the  offending  tendencies,  and 
when  this  succeeds  recovery  takes  place  as  the  result,  so  to  sj)eak,  of 
the  encapsulation  of  the  objectionable  material  and  its  exclusion  from 
consciousness.  This  form  of  the  disorder  is  the  most  acute  and  the 
recovery  is  equally  most  apt  to  be  prompt,  and  it  will  be  seen  from 
this  explanation  why  this  is  so. 

In  the  paranoid  form  of  the  disorder  the  reaction  is  much  more 
efficient  than  in  the  hebephrenic  variety  and  in  some  respects  less 
efficient  than  in  the  catatonic.  Here  the  individual  takes  a  flight 
into  a  psychosis  and  the  delusions  are  the  expression  of  a  compromise 
between  the  opposing  psychic  trends.  Unable  to  live  in  the  real  world, 
the  patient  succeeds  in  inventing  a  world  in  which  he  can  live,  and 
having  invented  it  he  succeeds  in  getting  along  fairly  well  without 
noticeable  deterioration.  The  conflict  in  these  cases  tends  to  become 
stationary  after  the  devel()i)incnt  of  the  delusional  world.  The 
mechanism  here  is  the  same  as  in  ])araii()ia.     (See  Paranoia.) 

Hertschinger  has  more  es])ecially  defined  the  ways  of  getting  well 
by  pointing  out  that  the  i)atient  in  recovering  may,  as  the  result  of 
his  conflict  to  which  he  cainiot  adjust,  find  a  compromise  h\  changing 
him.self  and  interj)reting  the  world  of  reality  in  terms  of  his  morbid 


910  DEMENTIA  PRECOX  GROUP 

fantasy,  or  by  translating  the  world  of  his  fantasy  into  terms  of 
external  experience.  And  so  one  would  find  on  the  one  hand  delusions 
of  grandeur  which  are  a  compromise  formation  and  serve  to  change 
the  individual  so  that  he  may  be  better  satisfied  with  life  and  on 
the  other  hand  one  finds  delusions  of  persecution,  the  delusions  of 
influence  from  the  outer  world,  that  serve  to  change  the  outer  world 
in  conformity  with  the  patient's  complexes.  These  outside  influences 
are  but  the  reflections  back  upon  the  patient  of  his  failures  to  get  from 
the  world  what  he  wants  and  they  are  consequently  felt  as  malign 
and  destructive  influences.  Another  method  of  getting  well  is  that 
already  described  of  the  catatonic,  the  shutting  out  and  encapsulation 
of  the  conflict  in  a  circumscribed  amnesia. 

In  many  cases  conversions  into  bodily  symptoms,  such  as  are  found 
in  hysteria,  are  found.  Another  method  of  getting  well  is  by  living 
through  a  series  of  imaginary  experiences,  which  brings  the  complex 
to  a  logical  conclusion.  For  example:  A  young  Japanese  woman 
was  overwhelmed  by  the  sad  news  that  five  members  of  her  family 
had  been  killed  in  battle.  She  passed  instantly  into  a  dreamy  state 
of  consciousness,  went  on  with  the  work  of  the  household  just  as  if  all 
five  were  members  of  it,  made  their  beds,  set  their  places  at  table  and 
acted  in  every  way  as  if  they  were  alive  and  present.  Finally  she, 
so  to  speak,  let  one  of  them  die  and  then  another  and  another  until 
finally  she  had  compassed  the  death  of  all  five,  after  which  she  awoke 
from  her  dream-like  state  and  was  well.  She  had  succeeded  in  an 
efficient  reaction  to  the  situation  by  its  attenuation,  extending  it 
over  a  considerable  period  of  time.  Finally,  a  certain  number  of 
patients  get  well  by  the  final  domination  of  the  reality  motive,  with  a 
resulting  correction  of  their  delusional  fantasies. 

Course  and  Progress. — As  will  be  seen  from  the  description  of  the 
disease  the  catatonic  form  is  more  apt  to  be  acute  in  onset  and  it 
Itqc-  il^f.  Kocf  pv-^gnosis.  while  the  hebephrenic  form  and  the  simple 
flenientin,o-  varieties  tend  to  |TrnPTPSSivp  flptpriorfltioii  .illd  llii  |»i.i-m.- 
noid  form  tpiids  to  remain  station^  wilhuul  i'lTaterial  deterioration.. 
A  few  of  the  hebephrenicsget  well,  moTh  uf  llit  catatonin^^cover, 
but  all  of  these  cases  are  liable  to  recurrent  attacks.  According  to 
a  study  of  Zablocka^  of  515  cases,  60  per  cent,  proceeded  to  light, 
18  per  cent,  to  medium  ariid  22  per  cent,  to  severe  deterioration. 

In  the  catatonic  cases  that  recover  there  is  usually  an  appreciable 
change  in  the  individual.  The  encapsulation  of  the  conflict  means 
that  the  recovery  has  taken  place  by  a  sacrifice  of  a  certain  portion  of 
the  personality.  The  portion  of  the  personality  in  which  the  conflict 
is  resident,  so  to  speak,  has  had  to  be  cut  out  or  walled  oft',  and  the 
energies  from  this  region  are  no  longer  available  by  the  individual. 
This  loss  is  quite  characteristically  shown  in  certain  changes  of  char- 
acter, indicating  that  the  individual  has  gotten  well,  but  has  paid  a 
certain  price  for  that  result. 

'  Zur  Prognosestellung  bei  der  Dementia  Precox,  Allg.  Ztschr.  f.  Psychiat.,  Band  Ixv. 


COURSE  AND  PROGRESS  911 

The  cases  that  do  not  get  well  tend  to  regress  to  lower  levels,  and 
quite  characteristically,  in  institutions  at  least,  they  finally  reach  a 
level  on  which  they  can  maintain  themselves  without  sinking  further. 
In  this  condition  they  remain  stationary  for  long  periods  of  time. 
Quite  often  patients  brought  to  an  institution  in  a  very  much  disturbed 
condition  settle  do^n  quite  promptly  mider  the  simpler  conditions 
of  institution  life,  while  in  the  outer  world  the  precox  cases  tend  to 
gravitate  into  the  ranks  of  the  hobo,  the  prostitute,  and  the  petty 
criminal.    Here  they  finally  find  their  level  and  get  along  after  a  fashion. 

Acute  and  severe  grades  of  regression,  in  which  the  splitting  goes 
to  the  vers'  foundation  of  the  personality,  often  express  themselves 
by  suicidal  attempts  and  sometimes  by  homicidal  attempts.  The 
preparation  for  such  attempts  may  sometimes  be  foreseen  in  the 
dreams. 

Remissions  are  quite  the  rule  and  come  about  under  circumstances 
which  reanimate  the  conflict.  Often  patients  get  along  very  well  in 
an  institution,  but  become  upset  shortly  after  going  back  to  the 
conditions  under  which  the  conflict  developed.  This  is  especially  true, 
Bertschinger  has  shown,  if  the  conditions  to  which  the  patient  is 
returned  have  materially  changed  during  his  internment.  The  patient 
does  better  if  returned  to  a  home  that  has  remained  comparatively 
unchanged.  If,  on  the  contrary,  the  family  has  moved  into  a  new 
neighborhood,  acquired  new  associations,  or  if  a  parent  has  died  mean- 
time, conditions  requiring  new  adjustments  on  the  part  of  the  patient, 
he  may  not  be  able  to  meet  the  demands  and  so  relapses. 

Malignancy  of  the  Reaction. — The  ways  of  getting  well  show  that, 
for  the  most  part,  the  method  of  dealing  with  the  conflict  is  the  opposite 
of  that  in  the  extraversion  t\'pe  of  psychosis.  It  is  the  method  of 
introversion  that  demands  that  the  individual  change  in  conformity 
with  the  demands  of  the  complex,  the  delusional  system .  This  method, 
because  it  involves  a  flight  away  from  rather  than  into  reality  is  a 
determining  factor  in  the  malignancy  of  the  precox  t}j)e  of  reaction. 
It  is  because  the  patient  cannot  escape  from  the  mechanism  of  intro- 
version, because  he  cannot  project  his  interests  into  the  world  of 
reality  (extraversion)  that  he  so  frequently  fails  to  make  those  adjust- 
ments that  spell  recovery.  He  is  incapable  of  socializing  his  instincts 
and  remains  shut  in,  infantile,  solitary,  asocial. 

The  course  of  the  disease  is  irregular  and  prediction  is  cjuite  impos- 
sible. In  a  general  way,  however,  one  gets  clues  from  keejnng  in 
mind  the  fact  that  the  conflict  is  between  the  reality  motive  and  the 
fantasy  formation,  and  by  watching  the  interplay  of  these  two 
motives  one  gets  an  idea  as  to  whether  the  reality  motive  is  gaining 
or  losing  ground. 

The  question  of  a  recovery  is  a  mooted  one,  but  in  general  it  is 
conceded  that  the  patients  that  recover  do  present  to  careful  analysis 
certain  residuals.  Of  course,  however,  these  residuals  may  be  of  any 
degree  and  maybe  so  slight  as  not  to  necessarily  imp'-'ir  the  individuals 
in  the  ])osition  in  life  which  they  may  occupy.    It  is  possible  that  true 


912  DEMENTIA   PRECOX  GROUP 

recovery  may  take  place  with  a  resolution  of  all  the  symptoms,  but  if 
this  is  so  it  is  probably  the  exception. 

Diagnosis. — The  diagnosis  of  dementia  precox,  while  comp?ratively 
easy  in  the  well  defined  and  the  advanced  cases,  becomes  a  matter  of 
great  difficulty  during  the  early  history  of  the  illness  or  in  cases  in 
which  the  symptoms  are  mild. 

The  manic-depressive  psychosis  prpsp1^^^g  mi^  nf  iht^  n1inn*»otf>rirtin 
diff^cuTfies.  In  this  psychosis  there  is  usually  a  history  of  repeated 
attacks  without  deterioracion.  it  must  not  be  forgotten,  however, 
that  precox  itself  frequently  presents  a  similar  history  and  that  if 
deterioration  is  present  it  may  not  be  prominently  in  evidence.  The 
depression  which  so  frequently  ushers  in  a  precox  attack  may  easily 
be  mistaken  for  a  depression  of  the  manic-depressive  psychosis.  The 
manic-depressive  retardation  is  similar  in  its  outward  appearances 
to  the  indifference  and  perhaps  lighter  degrees  of  negativism,  particu- 
larly the  inaccessibility  of  the  precox.  The  manic-depressive  is  more 
apt  to  havp  rlpjusions  of  a  self-accusatory  type  than  the  pi;;ecoj^aii"d 
the  latter 's  delusions  are  jjiore  apt  to  be  grotesque.  They  more 
frequently  are  evident  distortions  and"  symbolisms"  TKe  pressure  of 
activity  of  the  manic  resembles  the  activity  of  the  catatonic.  In  the 
former,  however,  the  activity,  although  rapidly  changing  as  to  its 
object,  is  characteristically  addressed  to  some  purpose,  while  in  the 
catatonic  the  activity  is  more  diffuse  and  has  less  direction.  It  is 
more  incoherent.  The  distinction  here  between  the  extraversion  of  the 
manic  and  the  introversion  of  the  precox  will  aid  in  the  differentiation. 

It  is  still  a  mooted  question  whether  there  are  not  conditions  inter- 
mediate between  precox  and  manic-depressive  psychosis  which  partake 
somewhat  freely  of  the  characteristics  of  both.  There  are  a  con- 
siderable number  of  cases  in  which  the  difficulties  of  diagnosis  are  very 
great.  Flight  of  ideas  may  be  quite  typical  in  precox,  for  example, 
while  the  depression  of  the  precox  may  resemble  very  closely  that 
of  the  manic-depressive.  The  difficulties  are  greatest  with  that  class 
of  manic-depressive  psychoses  known  as  the  mixed  states. 

If  we  will  consider  the  individual  and  not  the  disease  we  can  under- 
stand how  in  the  same  patient  extraversion  and  introversion  mechan- 
isms may  replace  one  another.  In  precox,  therefore,  we  may  expect  to 
see  temporary  eft'orts  to  escape  from  the  conflict  by  a  flight  into 
reality — thus  producing  manic  s;\inptoms  which,  however,  cannot  be 
kept  up  for  long  and  then  the  patient  lapses  into  regression.  In  this 
way  the  characteristics  of  the  two  psychoses  may  be  present  in  the 
same  illness  and  only  represent  efforts  at  dealing  with  the  conflict. 
Such  facts  indicate  the  danger  of  attempting  to  adhere  to  a  cut-and- 
dried  scheme  of  diagnosis. 

In  the  early  stages  the  mild  depression  of  the  precox  may  simulate 
that  of  a  neurasthenic,  or  the  agitated  depression  may  simulate  that 
of  an  anxiety  neurosis.  In  both  instances  the  precox  is  more  apt  to 
show  grotesque  ftelusions  and  conduct  disorders  of  a  bizarre  nature, 
such  as  tearing  his  clothes,  self-mutilation,  or,  on  the  other  hand, 


DIAGNOSIS  913 

characteristic  negativistic  s^niptoms,  such  as  retaining  the  saliva  or 
the  urine,  withdrawing  from  efforts  addressed  to  assist  him,  refusmg 
to  cooperate  in  changing  his  clothes,  the  refusal  of  food  and  the  like. 
It  must  not  be  forgotten,  however,  that  the  etiological  factors  of  the 
actual  neuroses  may  operate  in  the  same  person  who  breaks  doAMi  as  a 
precox,  and  that  therefore  neurasthenic  and  anxiety  symptoms  may 
be  present  as  expressions  of  these  etiological  factors.  It  is  important 
to  keep  such  a  possibility  as  this  in  mind  when  it  comes  to  the  matter 
of  treatment. 

Epileptiform  and  hysteriform  episodes  may  lead  to  a  diagnosis 
oFepilepsy  or  hysteria.  It  must  not  be  forgotten,  however,  that  it  is 
possible  to  have  precox  complicated  with  epilepsy  and  that  in  dementia 
precox  all  of  the  sjTnptoms  and  characteristic  mechanisms  that  are 
found  in  hysteria  may  be  found. 

In  the  infection  and  exhaustion  psychoses  the  differentiation  may 
be  very  difficult  and  it  is  necessary  in  such  patients  often  to  wajLJOILa- 
considerable  time  until  th£.^iibsidence  of  the  infection  and  then  see 
whetherthe  case  clears  up,  as  it  usually  does  if  it  is  a  simple  infection 
psychosis.  One  has  to  be  very  careful  in  making  a  "prognosis  in  cases 
of  this  character.  Not  infrequently  cases  of  so-called  puerperal 
insanity  are  really  cases  of  precox  which  have  been  precipitated  by  the 
circumstances  of  the  puerperal  period,  loss  of  blood,  prolonged  labor, 
infection,  or  the  mental  stress  incident  to  an  illegitimate  pregnancy. 

Fronfpafesi^  the  differentiation  can  now  be  made  by  the  laboratory 
methodSTctrOur  disposal.  It  must  not  be  overlooked  that  the  precox 
may  have  syphilis  and  therefore  a  positive  Wassermann  in  the  blood 
serum.  In  fact,  not  a  few  do  show  this  reaction,  and  it  is  quite  readily 
conceivable  that  the  presence  of  an  uncured  syphilis  may  well  be  a 
precipitatmg  factor  in  the  outbreak  of  the  psychosis  in  a  certain  pro- 
portion of  cases.    

From  thevtoxic  psychos|^,  particularly  from  alcoholic  deterioration 
the  differentiation  is  otten  quite  difficult.  It  mut  be  borne  in  mind 
in  this  connection  that  the  relatively  normal  man  deteriorates  very 
slowly  from  the  use  of  alcohol,  while  one  finds  in  the  records  of  precox 
cases  who  have  indulged  in  alcohol  that  the  deterioration  has  come 
very  much  earlier.  In  addition  to  this  it  will  be  found  that  the  amount 
of  deterioration  in  the  precox  case  is  very  much  greater  than  could 
reasonably  be  explained  by  the  alcoholic  indulgence  of  the  patient. 
When  this  discrepancy  in  the  histo  -y  is  found  one  is  justified  in  suspect- 
ing that  one  is  dealing  with  a  fundamentally  more  serious  condition 
than  mere  alcoholism.  These  two  types  of  cases  show  a  character- 
istic type  of  defence  which  serves  to  separate  them,  the  one  from  the 
other.  In  both  instances  the  patients  tend  to  minimize  the  true 
etiological  factor  and  to  exaggerate  the  unimportant  one.  For  example, 
the  precox  case  will  always  exaggerate  the  amount  of  alcohol  he  has 
been  taking,  while  the  alcoholic  will  always  minimize  it.  This  is  of 
course  only  roughly  true  but  it  may  be  helpful  in  siting  up  patients. 

The  whole  question  of  alcoholic  deterioration  is  by  no  means  settled. 
58 


914  DEMENTIA   PRECOX  GROUP 

Bleuler^  is  inclined  to  include  the  alcoholic  hallucinoses  in  the  dementia 
precox  group  and  at  least  it  must  be  acknowledged  that  many  alcoholic 
patients  after  repeated  attacks  of  acute  alcoholic  psychoses  undergo  a 
deterioration  which  is  precox  in  character. 

The  difficulty  of  differentiating  between  defect  due  to  precox  and 
congenital  defect  has  already  been  mentioned  in  discuss' ng  precox 
in  children,  and  precox  on  a  defective  basis.  A  material  help  in  making 
this  differentiation  is  an  inquiry  into  the  school  knowledge.  The  school 
knowledge  will  be  reasonably  well  retained  in  the  precox,  while  it  will 
not  have  been  acquired  in  the  defective. 

As  was  insisted  upon  in  the  beginning,  the  hebephrenic  and  simplex 
types  frequently  complain  of  numerous  minor  ailments.  These  are 
frequently  treated  by  the  general  practitioner  or  the  specialist  on  the 
basis  of  their  physical  rather  than  their  psychical  character.  Such 
patients  repeatedly  go  through  complicated  systems  of  treatment  until 
the  true  character  of  their  illness  has  become  evident,  when  often 
much  valuable  time  has  been  lost. 

Treatment.— Dementia  precox  has  generally  been  considered  to  be  a 
hopeless  condition  for  which  little  or  nothing  could  be  done.  This  is 
at  least  not  an  attitude  with  which  to  approach  a  patient,  and  when  we 
bear  in  mind  the  considerable  number  of  recoveries  that  take  place 
in  the  disease  it  is  hardly  an  attitude  that  is  warranted.  The  treat- 
ment, however,  must  of  necessity  be  very  difficult,  because  conditions 
that  have  to  be  met  are  multitudinous  and  range  all  the  way  from 
disturbances  at  the  lower  physiological  levels  through,  distinctly 
psychological  problems  to  the  relation  of  the  individual  to  his  social 
milieu.    These  matters  may  be  taken  up  in  their  order. 

Treatment  of  Physical  Conditions. — Here  the  treatment  must  be 
practically  entirely  symptomatic.  The  bladder  and  rectum  must  be 
carefully  watched  where  there  is  negativistic  retention.  The  mouth 
must  be  kept  clean,  swabbed  out  with  listerine  or  some  other  antiseptic 
mouth  wash,  if  there  is  retention  of  saliva  or  food  particles,  otherwise 
putrefactive  changes  may  occur,  infections  of  the  gum  with  ulcerations 
may  result,  with  possible  complications,  such  as  pneumonia  and  death. 
Other  such  conditions  as  these  have  to  be  met  in  a  practical  and 
common  sense  way  and  need  little  special  comment;  for  instance, 
surgical  injuries,  such  as  self-mutilation,  bruises,  and  the  like  have  to 
be  met  in  the  usual  way,  while  it  is  especially  important  to  get  these 
patients  out  of  doors  and  not  permit  their  seclusive  tendencies  to 
further  the  development  of  tubercular  disease. 

the^present  time  a  good  deal  of  attention  is  being  paid  to  the 
rternal  seCTetioi)s.    These  may  be  investigated  in  the  individual  case, 
but  as  yet  their  beneficial  action  is  unproved. 

1  Dementia  Precox  oder  Gruppe  der  Schizophrenien.  Franz  Deuticke,  Leipzig  u.  Wien, 
1911.  This  work  is  by  far  the  most  comprehensive  and  complete  treatment  of  the  whole 
dementia  precox  problem  extant.  An  excellent  review  of  the  work  by  August  Hoch 
will  be  found  in  the  Review  of  Neurology  and  Psychiatry,  June,  1912. 


TREATMENT  915 

Treatment  of  the  Mental  Condition. —The  treatment  of  the  mental 
condition  resolves  itself  into  the  treatment  of  disturbances  at  lower 
and  higher  levels.  In  the  disturbances  of  the  lower  levels  we  have 
characteristically  the  excitements  and  the  stupors.  In  general  the 
treatment  of  the  excitements  should  be  b^^ygrotheraE^  the  con- 
tinuous^bath  or  the  pack,  according  to  the  iimiviclual  experience  of  the 
phTsiriflrTor  thp  p^n^-PT1^pnfp  Q£_the_hospital.  Restraint,  either  physical 
or  chemical  should  be  avoidedif  possible,  resorting  to  drugs  only  in 
so  far  as  may  be  necessary  to  produce  a  sufficient  amount  of  sleep. 
It  must  be  borne  in  mind  that  all  of  the  drugs  that  are  used  for  chemical 
restraint,  more  especially  those  belonging  to  the  belladonna  group,  are 
delirium-producing  and  therefore  tend  to  interfere  with  the  adjustment 
of  the  patient  to  reality.  Small  doses  of  atropine  are  not  contra-. 
indicated  to  obtain  vegetative  nervous  system  control  of  certain 
annoying  somatic  symptoms. 

In  conditions  of  stupor  the  general  health  has  to  be  carefully  looked 
after.  The  patient  must  often  be  tube-fed,  bowels  and  bladder  require 
special  attention,  and  the  position  of  the  body  should  be  changed 
sufficiently  so  as  to  prevent  pressure  upon  any  portion  of  the  skin 
surfaces  or  a  tendency  to  hypostatic  congestion  of  the  lungs  in  weak- 
ened patients.  C'leanliness  and  regular  and  sufficient  feeding  are  the 
essential  things,  and  it  is  very  desirable  in  addition  to  wheel  the 
patient's  bed  out  upon  the  open  porch  where  he  can  have  abundance 
of  fresh  air,  if  this  is  possible. 

Treatment  at  the  higher  psj'chological  levels  has  the  same  thing 
to  be  said  for  it  as  psychotherapy  has  for  the  psychoneuroses.  While 
it  cannot  be  expected  that  patients  can  be  cured  by  psychoanalytic 
treatment  as  they  are  when  suffering  from  the  psychoneuroses,  still 
it  must  be  remembered  that  all  of  the  symptoms  of  a  precox  case  are 
"^ot  necessarily  at  the  same  level,  and  while  the  patieiitniay  not  be 
susceptible  of  a  cure,  still  many  of  the,_symptoms  may  be~lafgety~ 
r'elieved,  if  not  dispersed  altogether.  Q^sychoanalysis  therefore  sTiould 
bemused  and  even  where  the  therapeuticTtfect-mav'  not-oe  especially 
great,  still  it  furnishes  that  sort  of  information  about  the  patient  which 
it  is  essential  to  have  in  order  to  deal  with  him  intelh'gently.  One  must 
know  something  about  the  factors  that  produced  the  breakdown,  one 
must  know  something  of  the  nature  of  the  complexes,  in  order  to  go  at 
the  problem  of  regulating  the  life  of  the  patient,  not  only  intelligently 
but  with  any  possibility  of  accomplishing  anything.  To  approach 
the  problem  in  any  other  way  is  to  approach  it  blindly.' 

In  the  old  cases  of  precox  that  have  become  considerably  dilapi- 
dated, that  are  relatively  inaccessible,  it  would  seem  that  the  best 
method  of  approach  was  through  the  agency  of  industrial  training. 
If  an  effort  be  made  in  this  directi()n  intelligently  after  a  sufficiently 
careful  analysis,  so  far  as  possible,  of  the  particular  condition  so  that  it 

1  Jelliffe;  Treatment  of  Dementia  Precox,  International  Clinics,  1917.  Campbell: 
Modern  Treatment  of  Nervous  and  Mental  Diseases,  White  and  Jelliffe,  Lea  &  Febiger, 
1913. 


916^  DEMENTIA   PRECOX  GROUP 

will  appear  what  is  the  best  method  of  approach  in  order  to  arouse  the 
interest  and  fix  the  attention  of  the  patient,  a  great  deal  can  be  accom- 
plished in  making  this  class  of  patients  generally  useful  about  the 
hospital  and  in  limiting  their  destructive  and  filth}^  tendencies  and 
adapting  them  to  a  much  healthier  series  of  adjustments  than  if  they 
are  merely  left  to  themselves. 

The  Treatment  of  the  Social  Relations. — The  endeavor  to  modify 
the  environment  of  the  patient  must  be  guided  by  what  has  been 
found  as  the  result  of  psychoanalysis.  The  precox  splitting  goes  back 
to  the  early  infantile  situations,  the  time  when  the  love  of  the  child 
was  given  out  in  its  entirety  to  the  immediate  members  of  the  house- 
hold, father  and  mother,  brothers  and  sisters.  Later  on  if  the  child  is 
to  become  an  efficient  adult  he  must  emancipate  himself  from  the 
thraldom  of  this  affection.  This,  however,  is  what  the  precox  has 
not  been  able  to  do,  and  the  affection  which  binds  him  to  his  infantile 
loves  is  quite  truly  felt  as  a  destructive  force  that  prevents  his  onward 
progress  in  the  world.  There  frequently  results,  therefox'e,  all  sorts 
of  antagonisms  addressed  to  the  members  of  the  immediate  household 
which  are  variously  expressed  and  variously  symbolized  and  distorted. 
The  love  of  the  little  child  for  the  parent  or  the  brother  or  the  sistcx 
when  it  breaks  through  from  the  unconscious  into  the  conscious  life 
of  the  adult,  is  not  understood  at  its  true  value,  but  becomes  a  hateful 
thing,  and  so  characteristically  there  arise  all  sorts  of  incest  fan- 
tasies. Recently  one  such  patient  in  the  hospital  struck  and  stunned 
another  patient  and  when  called  to  task  about  it  he  instantly  protected 
himself  by  the  statement  that  "they"  were  accusing  him  of  incestuous 
relations  with  his  sister.  These  are  the  commonest  of  ideas  among 
precox  cases.  Psychoanalysis  will  orient  the  physician  with  relation 
to  these  ideas  and  enable  him  to  adjust  the  patient  intelligently. 
Such  ideas  frequently  require  the  removal  of  the  patient  to  an  insti- 
tution. 

Although  a  wholly  pessimistic  attitude  is  not  warranted  in  approach- 
ing the  problem  of  precox  in  an  individual  case,  still  it  must  be  realized 
that  after  all  one  can  hardly  expect  a  complete  recovery.  One  can 
only  hope  to  readjust  the  situation  so  that  the  patient  may  get  along 
comfortably  and  perhaps  lead  a  useful  life,  probably  upon  a  slightly 
lower  level.  The  tendency  of  the  disease  is  essentially  to  limit  the 
individual  in  the  mental  sphere,  and  this  limitation  means  of  necessity 
that  life  has  to  be  led  at  a  correspondingly  lower  and  simpler  plane 
of  adjustment.  This  is  exactly  what  the  institution  provides  for  the 
patient,  but  there  is  no  reason,  in  the  absence  of  dangerous  tendencies 
and  if  it  is  desirable  from  other  standpoints,  why  an  attempt  should 
not  be  made  in  this  direction  outside  of  an  institution.  We  know  this 
can  be  done,  for  we  see  not  infrequently  patients  getting  along  very 
well  under  the  solicitous  care  of  some  relative,  for  example,  and  when 
this  relative  dies  and  the  care  is  removed  and  their  world  is  no  longer 
carefully  arranged  for  them,  then  they  find  their  way  into  the  hospital. 

A  careful  dealing  with  all  of  the  conditions  surrounding  the  patient, 


PROPHYLAXIS  917 

which  may  have  to  include  the  other  members  of  the  family,  more 
particularly  dealing  with  them  intelligently  as  the  result  of  such  psy- 
choanalysis as  can  be  made,  will  enable  the  physician,  in  a  very  con- 
siderable proportion  of  cases,  if  the  means  are  at  his  disposal,  which 
of  course  they  frequently  are  not,  to  so  adjust  the  situation  as  to  bring 
about  a  state  of  relative  calm  and  quiescence,  with  the  preservation 
of  a  considerable  degree  of  eflSciency. 

Prophylaxis. — ^The  prophylaxis  of  dementia  precox  is  a  most  difficult 
problem,  and  in  the  first  instance  of  course  should  be  met  from  the 
eugenic  standpoint.  Marriage  should  be  very  carefully  supervised 
where  the  individual  comes  from  badly  tainted  stock.  Such  general 
principles  may  be  borne  in  mind,  as  for  example,  the  liability  to  mental 
disease  in  children  from  tainted  stock  is  greatest  among  the  earlier 
born  and  falls  oft'  rapidly,  as  Heron  has  shown,  particularly  after  the 
fourth  child,  while  jNIott  in  working  out  his  Law  of  Anticipatioirhas 
shown  that  if  thBrndivrdtiar  passes  the  twenty-fifth  year  the  liability 
to  a  mental  breakdown  is  very  materially  lessened. 

The  possibilities  of  prophylaxis  before  the  outbreak  of  the  psychosis 
are  not  known,  yet  it  would  seem  that  it  would  be  rational  to  endeavor 
to  deal  with  those  character  anomalies  that  we  know  favor  this  type 
of  disorder.  The  method  of  approach  will  of  course  resolve  itself 
into  an  attempt  to  define  the  lines  along  which  frank,  open  reactions 
do  not  seem  possible  to  the  individual,  particularly  along  lines  of 
definite  sex  conflicts.  In  this  particular  the  whole  matter  of  sexual 
education  has  to  be  gone  over  and  its  value  as  a  prophylactic  measure 
determined.^ 

1  See  Jelliffe:  Predementia  Precox,  Am.  Jour.  Med.  Sc,  August,  1907,  for  a  study  of 
these  early  features  which  antedate  the  appearance  of  the  disease,  also  chapters  on  the 
treatment  of.  Paranoid  States  and  of  Dementia  Precox,  by  Adolf  Meyer  and  Macfie 
Campbell,  Modern  Treatment  of  Nervous  and  Mental  Diseases,  White  and  Jelliffe, 
Lea  &  Febiger,  1913,  vol.  i. 


CHAP(TER  XX. 
INFECTION-EXHAUSTION  PSYCHOSES. 

This  group  of  infection-exhaustion  psychoses  is  somewhat  of  a 
heterogeneous  group,  including  all  of  the  mental  disturbances  depend- 
ent upon  the  various  infections,  as  well  as  certain  conditions  which  are 
tentatively  supposed  to  be  dependent  upon  exhaustion  and  which 
give  similar  clinical  pictures.  For  the  most  part  it  contains  the 
psychoses  dependent  upon  all  the  febrile  diseases,  for  up  to  the  present 
time  at  least  these  different  diseases  cannot  be  differentiated  by  means 
of  the  mental  picture  alone. 

In  connection  with  the  similarity  of  the  manifestations  in  this 
group,  although  the  ultimate  etiological  factors  are  widely  different, 
it  is  worth  while  to  bear  a  few  considerations  in  mind.  In  the  first 
place  it  has  been  suggested  that  after  the  manner  of  thinking  regarding 
the  syphilitic  manifestations  and  the  alcoholic  psychoses,  as  already 
outlined,  that  the  mental  picture  is  not  dependent  upon  the  immpdiatp 
infecting  agent  or  uponthe  toxin  directly  elaborated  by  the  infecting 
microqrganism,  but  on~tEe~CQntrary  is  due  to  a  general  disturbance^ 
inHEhe  jnetabolism,  the  result  of  the  infectionj_  in  other  words,  that 
there  is  an  intermediate"ageiicy  at  work,  a  metatoxin.  On  the  other 
hand,  it  has  been  suggested  that  the  gamut  of  symptomatology  which 
the  neuron  may  produce  in  the  process  of  its  destruction  is  necessarily 
confined  within  certain  relatively  narrow  limits  and  that  therefore 
inimical  agencies  that  act  at  the  biochemical  level  can  only  produce 
relatively  few  groups  of  symptom-complexes.  This  is  undoubtedly 
true.  On  the  other  hand,  while  it  will  be  found  that  if  the  individual 
cases  are  carefully  studied  the  general  course  of  the  malady  and  the 
mechanisms  involved  are  quite  similar  in  the  different  cases;  in 
other  words,  that  the  patients  fall  into  one  of  very  few  groups;  still 
the  content  of  the  delirious  or  delusional  experiences  and  the  minor 
variations  in  the  manifestations  in  the  malady  must  have  another 
explanation.  This  explanation  is  naturally  the  make-up  of  the  indi- 
vidual. A  destructive  agency  at  work  in  tearing  down  can  only  tear 
down  what  has  before  been  built  up.  It  is  constrained,  in  other 
words,  to  deal  with  the  materal  at  hand,  and  therefore  personal 
variations  must  be  expected;  for  example,  the  delirious  patient  will 
weave  expressions  into  his  productions  that  refer  directly  to  his 
own  experiences. 

Prefebrile,  Febrile,  and  Postfebrile  Psychoses. — The  same  thing 
may  be  said  regarding  fever  and  infection  which  is  said  regarding 
alcohol,  namely,  that  it  is  a  measure  of  the  mental  stability  of  the  indi- 
(918) 


PREFEBRILE,  FEBRILE,  AND  POSTFEBRILE  PSYCHOSES     919 

vidiial.  While  some  persons  may  remain  mentally  clear  with  a  tem- 
perature of  a  106°  F.,  others  may  become  delirious  with  hardly  more 
than  a  degree  of  temperature.  This  difference  is  a  well  known  one, 
and  in  a  disease  like  typhoid  fever,  for  example,  the  general  consensus 
of  opinion  is  that  the  prognosis  is  most  serious  in  those  cases  that 
become  delirious  early  and  show  from  the  beginning  marked  and 
exaggerated  nervous  symptoms. 

Infection  and  Initial  Delirium. — Under  this  head  are  included  the 
mental  disturbances  which  develop  early  in  the  infectious  diseases 
before  there  has  been  any  rise  in  temperature.  In  fact,  the  delirium 
in  these  cases  may  disappear  when  the  fever  is  fully  developed,  although 
this  is  not  the  rule,  the  usual  course  being  for  the  delirium  of  this 
period  to  go  over  into  a  fever  delirium.  This  infection  delirium  is 
also  found  in  conditions  which  are  essentially  afebrile,  as  for  example 
rabies,  and  is  there  due  of  course  to  an  overwhelming  of  the  body 
with  toxins,  or  perhaps  to  a  bacteremia.  This  type  of  mental  dis- 
turbance occurs  characteristically  with  the  onset  of  t\'phus,  in  the 
period  previous  to  the  eruption  in  smallpox,  and  has  been  observed 
in  connection  with  influenza,  acute  chorea,  especially  the  chorea  of 
pregnancy,  and  in  malaria. 

Symptoms. — The  symptoms  of  infection  delirium  are  the  usual 
sjTnptoms  of  delirium,  which  may  present  any  degree  of  severity,  from 
mild  confusion  to  delirium  acutum,  or  collapse  delirium,  ending  in 
death.  The  diagnosis  of  initial  delirium  previous  to  the  appearance 
of  the  typical  signs  of  the  disease  of  which  it  is  a  symptom  is  of  course 
practically  impossible. 

Fever  Delirium. — Fever  delirium  is  the  psychosis  which  accompanies 
febrile  movement  and  which  in  general  varies  in  severity  hand  in  hand 
with  the  variation  in  the  severity  of  the  fever. 

It  may  be  described  in  four  stages  according  to  the  degree  of  its 
severity.  In  the  first  stage  headache,  irritability,  sensitiveness  to 
noises  and  light,  restlessness,  and  disturbing  dreams;  in  the  second 
stage  hallucinations  appear,  especially  in  the  visual  field;  the  halluci- 
nations are  of  a  dream-like  character,  and  the  patient  may  still  be  made 
to  react  clearly;  in  the  third  stage  the  motor  disturbance  is  greater 
and  takes  on  the  character  of  jactitation;  in  the  fourth  stage  there  is 
profound  dulling  of  consciousness,  uncertain  and  ataxic  movements, 
ending  in  coma  and  death.  Of  course  this  regular  progress  of  the 
delirium  may  be  interrupted  at  any  point  by  an  improvement  in 
the  s;y'mptoms  and  recovery. 

The  onset  and  the  severity  of  the  delirium,  as  already  set  forth, 
show  to  an  extent  the  mental  stability  of  the  patient.  The  delirium 
develops  or  becomes  severe  much  more  readily  in  the  unstable  and 
poorly  organized  than  in  the  stable.  The  course  of  the  delirium  may 
be  interrupted  by  an  acute  excitement  followed  by  a  stuporous  con- 
dition, which  Bonhoeffer^  describes  as  an  epileptiform  excitement,  which 

>  Die  symptomatischen  Psychosen. 


920  INFECTION-EXHAUSTION  PSYCHOSES 

is  followed  by  a  dream  state,  for  which  latter  he  retains  the  name  of 
Ziehen,  infectious  dream  state.  With  these  conditions  confusing 
symptoms  may  arise  which  makes  the  diagnosis  difficult  for  a  time. 
Thus,  along  with  the  disorientation  there  may  be  flexibilitas  cerea 
confabulation,  perseveration. 

In  certain  cases  the  orientation  is  less  disturbed,  while  the  hallucina- 
tions are  more  prominent,  and  there  is  an  outward  semblance  of  a 
hallucinosis. 

Here,  as  elsewhere  in  psychiatry,  the  various  forms  of  the  psychosis 
are  frequently  designated  by  the  prevailing  symptoms.  Thus,  the 
symptoms  found  may  be  epileptiform  excitement,  dream  states,  stu- 
porous conditions,  hallucinosis,  catatonic  and  confusional  states,  and 
the  delirium  may  be  designated  by  using  any  one  of  these  descriptive 
terms. 

Postfebrile  Psychoses. — ^These  conditions  either  develop  as  a  result  of 
the  passing  over  of  the  delirium  of  the  febrile  state  into  the  period  of 
convalescence,  or  they  may  take  their  origin  from  the  first,  during  the 
postfebrile  period.  In  the  latter  case  they  would  be  considered  as 
belonging  more  properly  to  the  exhaustion  psychoses,  and  yet  it  must 
be  understood  that  the  term  exhaustion  is  a  very  vague  one  and  that 
in  all  probability  it  implies  at  least  the  accumulation  of  toxins. 

The  characteristic  picture  of  this  psychosis  is  associated  with  the 
great  physical  debility  which  follows  the  subsidence  of  the  fever  in  an 
infectious  disease.  The  patient  is  weak,  tremulous,  exhausted,  and 
complains  of  being  tired.  The  mental  state  is  fundamentally  one  of 
weakness.  There  is  no  confusion,  but  the  capacity  for  attention  is 
very  much  reduced,  and  in  that  way  there  come  about  apparent  mem- 
ory disturbances.  The  patient  is  unobservant  of  his  surroundings, 
usually  somewhat  depressed,  and  may  be  very  much  occupied  with  his 
own  bodily  feelings  to  the  extent  of  having  h^'pochondriacal  ideas,  and 
fleeting  hallucinations  are  not  infrequent.  In  more  severe  cases  there 
may  be  more  evidence  of  mild  delirium  or  confusion,  the  mood  may  be 
more  definitely  anxious  and  fearful  and  there  may  be  delusions  char- 
acteristically of  the  persecutory  type.  The  patient  is  apt  to  be  irritable, 
cross,  and  complaining.  Occasionally  there  may  be  considerable 
motor  excitement  of  an  epileptiform  character,  and  sometimes  actual 
delirious  dream  states.  Occasionally  the  picture  of  a  mild  expansive- 
ness  is  found.  A  certain  number  of  cases  present  a  well  marked 
Korsakow  syndrome. 

Exhaustion  Psychoses. — ^The  term  exhaustion  in  this  connection  is 
not  intended  to  be  used  in  other  than  a  tentative  sense.  The  whole 
question  of  fatigue,  except  with  reference  to  very  specific  problems, 
such  for  example  as  muscle  fatigue,  is  still  little  understood.  In  general, 
however,  it  may  be  said  that  there  are  two  factors  in  the  problem, 
the  negative  and  the  positive.  The  former  is  the  result  of  the  actual 
wearing  out  of  a  substance  in  the  body,  as  for  example  muscle,  while 
the  second  is  the  result  of  the  formation  of  certain  poisonous  substances 
which  result  from  the  breaking  down  of  tissue.     In  the  conditions 


EXHAUSTION  PSYCHOSES  921 

which  ordinarily  are  observed  it  would  seem  evident  that  both  of 
these  factors,  the  positive  and  the  negative,  are  in  evidence,  and 
exhaustion  is  considered  where  they  appear  to  be  the  predominating 
factors.  For  example,  exhaustion  is  spoken  of  when  coming  on  acutely 
as  the  result  of  a  sudden  loss  of  a  considerable  quantity  of  blood,  of 
coming  on  more  slowly  as  a  result  of  the  debilitating  effects  of  a  chronic 
disease  such  as  carcinoma.  In  this  latter  case,  however,  it  will  be 
easily  seen  that  in  all  probability  the  toxic  element  must  enter.  Simi- 
larly in  convalescence  from  acute  illness  where  the  fever  has  been 
very  high  and  the  illness  has  been  prolonged,  conditions  accompanied 
by  great  physical  prostration  are  seen  in  which  it  seems  fair  to  assume 
that  the  element  of  direct  exhaustion  is  responsible  very  largely  for 
the  mental  picture.  Under  the  head  of  exhaustion  psychoses  two 
main  types  will  be  described — collapse  delirium  and  acute  hallucinatory 
confusion  (amentia) — but  it  must  be  understood  that  these  two  psy- 
choses are  not  necessarily  peculiar  but  only  conditions  in  which  the 
exhaustion  element  appears  to  predominate.  Either  or  both  of  them 
may  be  found  during  the  period  of  acute  infection  in  the  febrile  diseases, 
and  conditions  that  begin  with  infection  and  fever  and  give  the  picture 
of  an  infection  or  of  a  fever  delirium,  may  go  over  into  the  severer  type 
as  the  patient's  general  condition  becomes  worse  and  the  organism  is 
overwhelmed  with  the  poisoning. 

Collapse  Delirium. — ^Ihis  is  the  delirium  grave,  or  the  acute  delirious 
mania  of  the  older  authors. 

The  disease  may  present  a  prodromal  period  of  restlessness,  irri- 
tability, and  insomnia,  after  which  a  condition  of  mild  confusion 
may  develop  with  only  a  slight  degree  of  perplexity  and  perhaps  fleet- 
ing hallucinations,  slight  clouding  of  consciousness,  disorientation  and 
dreamy  delusions;  psychomotor  excitement  is  common  at  this  time, 
the  patient  being  active  and  perhaps  inclined  to  actual  violence  and 
destructiveness.  Often  associated  in  the  early  symptoms  are  accesses 
of  anxiety,  amounting  at  times  to  actual  terror.  This  condition 
gradually  becomes  worse,  and  finally  the  degree  of  excitement  becomes 
very  great,  exceeding  anything  that  we  usually  see  in  the  other  psy- 
choses. When  this  extreme  form  of  excitement  is  in  evidence  the 
outcome  is  usually  apt  to  be  serious,  and  the  older  aat iters  believed 
it  to  be  uniformly  fatal. 

In  these  severe  cases  the  incoherence  becomes  absolute,  the  disori- 
entation complete,  the  clouding  of  consciousness  profound,  the  temper- 
ature generally  runs  high,  perhaps  as  high  as  10G°  F.,  gastro-intesti- 
nal  symptoms  are  common,  there  is  usually  almost  complete  anorexia, 
coated  tongue,  offensive  diarrhea,  a  high  grade  of  indicanuria,  and 
rapid  emaciation  which  results  in  a  high  grade  of  exhaustion,  with 
typhoid  symptoms.  There  may  be  a  certain  amount  of  catalepsy, 
with  stereotyped  movements,  grimacing,  and  echolalia,  and  stuporous 
conditions;  coma  and  death  not  infrequently  result. 

This  condition  occurs  with  all  degrees  of  severity,  and  while  the  most 
severe  cases  die,  the  milder  ones  make  good  recoveries. 


922  INFECTION-EXHAUSTION  PSYCHOSES 

It  may  often  be  quite  impossible  to  make  a  diagnosis  of  the  physical 
condition  of  the  patients  while  in  their  excitement.  It  must  be  remem- 
bered, especially  if  the  patient  has  temperature,  that  these  conditions 
are  pretty  apt  to  be  dependent  upon  some  acute  physical  illness, 
particularly  an  infection,  and  that  not  infrequently  a  deep-seated 
pneumonia,  concealed  from  the  usual  approach  by  percussion,  and 
auscultation,  is  at  the  basis  of  the  difficulty,  while  an  infection  such  as 
grip,  rheumatism,  or  the  like  may  also  be  an  etiological  factor. 

That  the  element  of  exhaustion  is  after  all  not  the  only  element, 
and  perhaps  not  the  most  important  element,  will  be  appreciated  if 
one  stops  to  consider  that  the  great  majority  of  patients  do  not  react 
in  this  exaggerated  way  to  acute  toxemias  or  infections.  In  all  proba- 
bility the  fundamental  factor  at  the  bottom  of  such  a  reaction  is  the 
individual  make-up,  just  as  an  ounce  of  whisky  will  disorganize  one 
individual  as  much  as  a  pint  will  another.  The  personal  factor  is  the 
important  one. 

Acute  Hallucinatory  Confusion  (Amentia)  .^ — ^This  psychosis  is  less 
acute  in  its  characteristics  than  the  former.  The  symptoms  are  those 
of  a  mild  confusion  with  incoherence  and  a  considerable  degree  of 
perplexity.  There  are  usually  fleeting  hallucinations  in  the  various 
sensory  areas,  sometimes  delusions,  which,  however,  are  not  char- 
acteristically fixed,  with  a  more  or  less  changeable  emotional  attitude 
varying  with  the  content  of  the  delusions.  The  patient  is  character- 
istically in  a  condition  of  mild  motor  unrest. 

The  duration  of  the  illness  is  relatively  long.  It  may  be  prolonged 
over  several  weeks,  is  usually  from  one  to  three  months  in  duration,  and 
may  be  considerably  longer.  The  course  of  the  disease  is  not  infre- 
quently interrupted  by  lucid  intervals,  during  which  the  patient  is 
quite  clear.  These  may  last  anywhere  from  a  few  minutes  to  a  day 
or  two,  and  then  the  patient  will  lapse  back  into  his  previous  condition 
of  confusion.    This  is  an  important  point  to  bear  in  mind. 

A  very  marked  degree  of  perplexity  is  rather  characteristic  with 
these  patients.  There  is  considerable  disturbance  in  their  perception 
of  their  environment  which  they  do  not  seem  to  understand,  things 
about  them  appear  to  be  changed,  they  appear  to  be  in  some  strange 
place,  things  are  not  right,  they  do  not  understand  the  meanings  of 
things,  they  get  mixed  up,  get  the  wrong  bed,  and  act  in  similarly 
stupid  ways. 

Bonhoeffer^  describes  hallucinatory  and  psychomotor  catatonic  forms, 
in  the  latter  of  which  flight  of  ideas  and  incoherence  predominate. 

In  addition  to  the  previously  described  psychoses,  Bonhoeffer^ 
describes,  as  occurring  late  in  the  course  of  the  infectious  diseases, 
and  therefore  it  would  seem  dependent  to  a  certain  extent  at  least 
upon  exhaustion,  a  hyperesthetic  emotional  state  of  mental  weakness, 
associated  w^ith  physical  symptoms,  such  as  severe  headache,  pares- 
thesias, and  pains  in  the  joints,  great  prostration,  oversensitiveness 

1  Op.  cit.  2  Op.  cit. 


TYPHOID  FEVER  92^ 

to  noise  and  light,  easily  frightened,  troublous  dreams.  He  also 
describes  an  amnesic  variety  resembling  Korsakow's  psychosis.  Acute 
delirium  may  also  develop  and  one  may  see  a  meningitic  form  with 
very  severe  delirious  reaction. 

The  possibilities  of  local  injuries  to  the  brain,  such  as  brain  abscess 
and  local  meningitis  must  be  held  in  mind,  ^^^len  these  develop  in 
children  they  not  infrequently  leave  sequel*,  such  as  mental  defect 
and  epilepsy. 

Treatment.— The  treatment  of  all  of  these  conditions  is,  of  course 
in  the  main  the  treatment  of  the  underlying  disease.  In  general 
however,  it  may  be  said  that  for  the  excitement  the  continuous  bath  or 
the  wet  pack,  with  perhaps  the  occasional  exhibition  of  a  h^^pnotic 
should  be  chosen  rather  than  the  constant  drugging  of  the  patient,  so 
frequently  employed. 

When  there  is  marked  lack  of  desire  for  food  and  the  condition  is 
serious  and  approaching  one  of  profound  exhaustion  there  should  be 
no  delay  in  resorting  to  artificial  feeding.  The  rectum,  of  course,  may 
be  used  if  the  stomach  is  very  irritable,  but  should  be  a  last  resort. 
It  is  preferable  to  give  small  quantities  by  the  stomach  frequently, 
and  endeavor  in  that  way  to  deal  with  the  situation  than  to  give  rectal 
feeding,  with  the  usual  result  of  being  satisfied  with  the  giving  of  an 
amount  of  food  which  is  really  very  inadequate. 

In  the  extreme  exhaustion  of  the  later  stages,  especially  when  there  is 
marked  dehydration,  hypodennoclysis  often  gives  most  excellent  results. 

Tjrphoid  Fever. — An  initial  delirium  in  typhoid  fever  is  generally 
conceded  to  be  of  most  ominous  import,  while  an  early  delirium  which 
is  continuous  and  severe  is  also  of  bad  prognostic  significance.  With 
this  disease  the  good  effects  of  the  bath  treatment,  as  used  in  the 
Brand  method,  is  especially  well  seen.  Cold  baths  for  the  reduction  of 
temperature  have  as  one  of  their  most  important  results  their  sedative 
and  calming  effect  upon  the  nervous  symptoms. 

The  possibility,  in  all  such  diseases  as  this,  of  the  localization  of  the 
infection  in  the  meninges  should  be  thought  of,  and  in  cases  of  marked 
delirium,  therefore  the  possibility  of  a  meningitis  should  be  borne  in 
mind.  Lumbar  puncture  may  be  valuable  under  these  circumstances, 
both  for  diagnostic  purposes  and  for  the  relief  of  ])ressure. 

In  the  late  stages,  during  couAalescence,  special  efforts  should  be 
made  to  make  the  dietary  as  full  and  liberal  as  possible. 

Erysipelas,  the  various  exanthems,  and  the  several  types  of  malaria 
are  also  not  infrequently  complicated  by  mental  symptoms,  while 
influenza  often  })roduces  profound  depressions  associated  with  great 
physical  exhaustion  hanging  over  during  a  long  convalescence.  We 
must  remember  in  all  of  these  conditions,  particularly  in  the  exanthems, 
the  possibility  of  meningeal  focalization.  Influenzal  infections  some- 
times also  produce  meningeal  hiflammation. 

Ande  articular  rheumatism  is  especially  important  in  this  connec- 
tion because  of  its  relation  to  acute  chorea  and  the  so-called  chorea 
insaniens. 


924  INFECTION-EXHAUSTION  PSYCHOSES 

Pneumonia  is  frequently  associated  with  mental  symptoms.  Very 
many  of  the  cases  of  delirium  tremens  owe  their  severity  to  a  pneu- 
monia, usually  a  masked  form  of  this  disease,  either  a  central  pneu- 
monia or  one  located  in  the  upper  lobes,  and  therefore  not  so  readily 
diagnosed. 

In  connection  with  all  this  class  of  diseases  the  many  complications 
must  be  borne  in  mind  which  may  arise  in  their  course  and  upon 
which  the  mental  symptoms  may  depend  other  than  the  meningitides, 
as  for  example,  the  acute  types  of  nephritis  in  connection  with  scarlet 
fever,  the  middle-ear  complications  of  measles,  and  the  endocardial 
complications  of  rheumatism. 


CHAPTER  XXI. 
THE  TOXIC  PSYCHOSES. 

Alcoholism. — It  is  generally  conceded  that  alcohol  is  a  powerful 
poison  and  as  such  if  taken  in  large  quantities  or  over  a  long  period 
of  time  produces  serious  damage  to  the  individual.  There  is,  unfor- 
tunately, associated  with  this  view  the  opposite  view  that  alcohol  is  a 
A'aluable  medicine  in  certain  conditions,  particularly  that  it  is  a  valuable 
stimulant,  while  among  certain  peoples  it  is  generally  accepted  as  an 
essential  article  in  the  daily  dietary. 

As  a  matter  of  fact  the  toxic  properties  of  alcohol  far  outweigh 
any  possible  beneficent  effects  that  it  may  have.  In  fact,  it  is  question- 
able whether  alcohol  should  be  considered  in  any  other  sense  than  as  a 
poison.  It  has  no  special  medicinal  properties  that  are  of  value,  it  is 
not  a  stimulant,  and  at  most  might  be  considered  as  a  hypnotic, 
especially  in  old  people  with  some  arteriosclerosis.  Other  drugs 
can  be  used,  however,  quite  as  well  and  more  safely.  It  is  probable 
that  the  widespread  belief  in  its  efficacy  for  all  sorts  of  conditions  is 
based  upon  an  effort  at  justification  for  its  use. 

The  part  that  alcohol  plays  in  the  production  of  mental  disorders 
is  extremely  difficult  to  express  in  definite  terms.  It  has  been  claimed 
that  12  to  15  per  cent,  of  the  psychoses  are  dependent  upon  alcohol 
as  the  principal  etiological  factor,  and  yet  any  such  figure  as  this  is 
extremely  misleading,  for  it  is  really  not  known  how  alcohol  brings 
about  its  results,  and  especially  it  is  not  known  whether  it  is  primarily 
the  alcohol  which  is  to  blame  or  whether  secondarily  the  metabolism 
disturbances  which  are  produced  by  its  continuous  use.  It  is  of  the 
highest  significance  that  of  the  cases  that  come  to  autopsy  a  very 
considerable  number  of  them  in  the  general  hospitals  and  poor-houses 
are  found  to  sufi'er  from  cirrhosis  of  the  liver,  while  in  the  hospitals  for 
the  insane  this  condition  is  of  rare  occurrence.  The  indication  is  very 
clear  that  the  psychoses  are  dependent  upon  some  peculiarity  of  make- 
up of  the  individual  which  is  afl'ected  in  an  exaggerated  way  by  alcohol 
or  of  which  alcoholism  is  the  expression.  If  this  is  true,  then  the 
alcoholism  is  only  a  surface  indication,  and  the  true  etiological  factors 
lie  deeper. 

Psychology. — There  are  many  types  of  persons  who  drink  and  there 
are  many  reasons  for  drinking.  The  usual  reasons  given  refer  to  social 
conditions.  One  is  rather  taught  to  believe  that  the  social  demands 
are  of  such  a  nature  as  to  require  a  certain  amount  of  drinking,  and 
that  this  drinking  once  started  tends  to  perpetuate  itself  and  to  finally 
become  a  fixed  habit.    This  way  of  looking  at  the  situation,  like  the 

(925) 


926  THE   TOXIC  PSYCHOSES 

belief  in  the  beneficent  effect  of  alcohol,  is  very  largely  at  least  an  effort 
at  justification.  The  social  conditions  cannot  be  changed.  They  have 
to  be  submitted  to,  and  therefore  the  drinking  is  inevitable  and  not  the 
fault  of  the  individual.  This  really  offers  no  adequate  explanation, 
and  in  all  probability  there  is  very  little  truth  in  the  statement.  People 
do  not  drink  simply  and  solely  because  they  have  acquired  a  habit 
of  drinking.  The  habit  element  is  the  least  important  in  the  whole 
situation,  and  if  that  were  the  only  thing  to  be  dealt  with  the  problem 
of  alcoholism  and  of  other  habituations  would  be  relatively  simple. 
People  drink  because  of  definite  returns  which  they  get  from  drinking. 
A  given  number  of  persons  all  placed  under  the  same  conditions,  social, 
etc.,  do  not  all  drink.  It  is  only  some  of  them  who  drink,  and  those 
who  drink  do  so  not  only  because  they  get  definite  desirable  results, 
but  because  those  results  are  practically  necessary  for  them.  In  other 
words,  far  more  important  than  the  question  of  habit  formation,  is 
the  question  of  the  individual  psychology.  Here,  again,  as  has  been 
seen  already  in  dealing  with  the  psychoses,  the  question  of  mental 
conflicts  is  most  important.  When  the  individual  is  confronted  by 
situations  to  which  he  cannot  adjust  adequately,  when  the  world  of 
reality  makes  demands  which  are  too  great  for  him  to  meet,  one  of  the 
ways  in  which  the  individual  reacts  to  such  a  condition  is  by  narcotizing 
himself  and  so  withdrawing  from  the  whole  situation.  Alcohol  then 
becomes  an  agent  which  helps  the  patient  to  get  away  from  the  conflicts 
thrust  upon  him  by  reality,  it  helps  him  to  withdraw  within  himself, 
helps  him  to  live  in  the  world  of  fantasy  where  things  come  true  as 
he  wishes  them.  Under  these  circumstances  it  can  be  seen  why  what 
appears  to  be  a  habit  is  formed.  The  moment  the  individual,  harassed 
by  the  absolutely  inacceptable  demands  of  the  world,  finds  an  avenue 
of  escape  in  which  he  can  rest  from  their  harassings,  finds  the  possi- 
bility of  peace,  of  repose,  he  finds  it  equally  impossible  to  resist  the 
temptation  to  avail  himself  of  it  and  of  course  he  usually  continues 
to  avail  himself  of  it.  He  is  rendered  more  and  more  incapable 
of  meeting  the  conflict  efficiently.  Therefore  a  vicious  circle  is  estab- 
lished and  the  individual  is  hopelessly  involved.^ 

Aside  from  the  class  of  individuals  described  above,  it  must  be 
constantly  borne  in  mind  that  indulgence  in  alcohol  is  oftentimes 
the  expression  of  a  neurosis  or  a  psychosis.  For  example,  the  recurrent 
attacks  of  manic-depressive  psychosis  may  be  ushered  in  by  alcoholic 
indulgence,  and  if  one  is  not  keenly  observant  he  may  easily  suspect 
that  he  is  dealing  with  an  alcoholic  psychosis  rather  than  with  a  manic- 
depressive.  The  same  thing  of  course  may  be  said  of  dementia  precox 
which  is  oftdn  found  associated  with  alcohol,  and  with  paresis,  which  not 
infrequently  has  a  history  of  alcoholism  in  its  early  stages.  Then  it 
must  be  remembered  that  in  certain  post-traumatic  conditions,  that  is, 
cases  following,  especially,  head  injury  and  sunstroke,  and  in  arterio- 
sclerotic and  senile  conditions  the  patient  may  react  in  an  exaggerated 

1  See  Jelliffe:  The  Mentality  of  the  Alcoholic,  N.  Y.  Med.  Jour.,  April  7,  1917,  for 
discussion  of  psychoanalytic  priuciples  coijcerniog  the  deeper  psychology  of  alcohoHsm. 


CHRONIC  ALCOHOLISM  927 

way  to  alcohol  and  that  very  small  doses  may  produce  very  pronounced 
effects.  This  is  true  also  in  connection  with  certain  other  conditions, 
particularly  imbecility  and  epilepsy.  Here  pronounced  reactions  to 
alcohol  are  found,  and  it  is  important  to  evaluate  the  importance  of 
the  alcohol  in  the  entire  situation. 

Finally,  there  are  certain  psychoses  which  appear  to  be  essentially 
alcoholic;  to  depend  upon  prolonged  indulgence  in  alcohol.  Of  the 
acute  conditions  due  to  alcohol  drunkenness  is  the  most  typical, 
while  of  the  chronic  conditions  dependent  upon  alcohol,  delirium 
tremens,  alcoholic  hallucinosis,  and  Korsakow's  psychosis  are  the 
most  important.  These  latter  because  they  appear  to  be  dependent 
upon  something  other  than  simple  alcohol,  as  they  never  occur  as  the 
result  of  single  large  doses,  but  can  only  occur  in  a  person  chronically 
addicted  it  has  been  proposed  to  call,  after  the  manner  of  the  psychoses 
due  to  syphilis,  the  meta-alcoholic  psychoses.  With  this  introduction 
a  short  description  of  each  of  the  mental  pictures  dependent  directly 
or  indirectly  upon  alcohol  will  be  gi\'en. 

Drunkenness. — The  general  phenomena  of  drunkenness  are  too 
well  known  to  require  detailed  description,  except  that  perhaps  the 
less  evident  manifestations  are  not  generally  thought  of.  The  pro- 
gressive disturbance  of  coordination  of  the  motor  centers,  the  higher 
being  thrown  out  of  adjustment  first,  and  the  progressive  disturbance 
of  the  sensory  apparatus  in  the  same  way  produce  disturbances,  the 
latter  of  which  are  entirely  subjective,  and  so  are  not  generally  observed. 
The  mood  of  the  drunken  man  is  variable.  Each  man  reacts  to  alcohol 
in  a  way  peculiar  to  himself,  but  in  a  general  way  there  may  be  con- 
sidered to  be  two  classes  into  which  cases  of  drunkenness  may  be 
divided,  the  exalted  and  the  depressed,  the  former  closely  resembling 
the  manic  phase  of  the  manic-depressive  in  his  extreme  loquaciousness 
and  hyperactivity,  while  in  the  latter  the  patient  withdraws  within 
himself,  being  sullen,  morose,  and  disinclined  to  associate  with  others. 

One  of  the  important  considerations  under  this  head  is  the  degree  of 
ease  with  which  the  individual  is  seen  to  react  to  alcohol.  Alcohol, 
like  fever,  may  be  used  to  express  the  measure  of  cerebral  resistance; 
unstable  and  defecti\'e  individuals  reacting  to  both  in  an  exaggerated 
way. 

Pathologiral  Drujikenness. — Drunkenness  that  exhibits  unusual 
features,  which  leads  the  individual  to  perform  strange  acts  or  acts  of 
violence  or  which  produces  serious  physical  sym])toms  is  known  as 
pathological  drunkenness.  Persons  in  this  condition  may  develop 
well  marked  hallucinations  or  delusions  to  which  they  tend  to  react, 
while  convulsive  attacks  are  not  infrequent,  and  the  whole  period  is 
often  obliterated  from  the  memory  by  an  amnesia. 

Chronic  Alcoholism. — Uncomplicated  habitual  drunkenness,  that  is, 
inebriety  not  dependent  upon  some  definite  underlying  condition,  such 
as  manic-depressive  psychosis  or  paresis,  has  the  earmarks  of  a  psycho- 
neurosis  and  is  therefore  dependent  upon  the  character  make-up  of 
the  individual  for  its  cause.     These    earmarks,  which  the  alcoholic 


928  THE  TOXIC  PSYCHOSES 

well  shows,  are  the  feeling  of  inefficiency  as  a  result  of  which  the  alcohol 
is  taken  as  an  effort  'at  finding  safety — it  is  the  means  of  a  flight  from 
reality. 

It  must  be  recalled  that  many  psychoneurotics  whose  compulsive 
flights  are  alcoholic — and  such  is  usually  the  flight  from  an  uncon- 
scious homosexual  conflict — are  very  superior  types  of  people. 

The  life  history  of  many  an  alcoholic  shows  him  to  be  an  inefficient 
individual.  He  is  incapable  of  meeting  reality  efficiently  every  day. 
He  may  be  able  to  deal  with  the  problem  oP  reality  for  a  greater  or  lesser 
length  of  time,  but  continuity  of  effort,  day  in  and  day  out,  is  foreign 
to  the  alcoholic  character.  He  can  stand  the  strain  only  about  so 
long,  longer  in  some  cases  than  in  others,  but  the  principle  is  the  same. 
This  is  the  inefficiency  Adler  believes  is  dependent  upon  organ  inferior- 
ity, or  to  use  an  older  and  more  tried  expression,  it  is  constitutional. 
The  reaction  to  such  a  feeling  of  inferiority  drives  the  inefficient 
individual  to  find  some  way  of  escape  from  the  horrid  facts,  the  over- 
burdening oppressions  of  reality.  This  he  finds  in  alcohol  which  dulls 
his  perception  of  reality  and  permits  the  world  of  fantasy  to  reign 
supreme.  In  this  fool's  paradise  the  alcoholic  finds  temporary  surcease 
from  the  burdens  he  is  but  poorly  equipped  to  bear. 

The  eft'ects  of  alcoholic  poison  may  be  exhibited  in  any  organ  of  the 
body,  more  particularly  the  central  nervous  system,  the  stomach,  the 
liver,  the  kidneys,  and  the  bloodvessels.  The  effects  on  the  nervous 
system  are  shown  in  various  disturbances  of  sensation,  such  as  the 
paresthesias,  amblyopia,  amaurosis,  dulness  of  hearing,  of  touch,  etc., 
while  in  the  motor  realm  we  find  tremor  and  epileptiform  attacks,  with 
general  motor  enfeeblement  characteristically.  The  mental  changes 
are  gradual  and  progressive,  the  intelligence  is  blunted,  the  judgment 
is  impaired,  the  moral  sense  dulled,  while  actual  delusions  not  infre- 
quently develop. 

While  all  of  these  changes  may  occur  in  chronic  alcoholism,  it  is 
usual  to  see  in  individual  cases  one  organ  more  especially  selected  out 
by  the  alcohol  for  its  destructive  effects.  With  the  beer  drinkers 
who  absorb  several  liters  of  beer  each  day,  cardiac  hj^Dertrophy  is 
quite  common.  Some  patients  develop  serious  kidney  complications, 
while  others  are  able  to  drink  over  long  periods  of  years  without  any 
material  impairment  of  the  kidneys.  The  same  remark  applies  to  the 
liver,  while  the  effects  upon  the  nervous  system  and  the  bloodvessels 
are  equally  varied.  It  is  characteristic,  too,  that  the  individual  as  a 
whole  varies  in  the  destructive  effect  which  alcohol  has  upon  him, 
some  individuals  apparently  being  able  to  use  large  quantities  over 
considerable  periods  of  time  without  noticeable  impairment.  The 
general  outward  appearance  of  health,  however,  which  many  indulgers 
in  alcohol  show  is  sometimes  rudely  dissipated  when  they  are  attacked 
by  an  acute  illness,  for  however  well  they  may  have  been  able  to  go 
on  with  the  routine  day's  work,  their  weakness  is  shown  when  they  are 
subjected  to  the  special  stress  of,  for  example,  a  pneumonia,  and  the 
death-rate  in  this  class  of  persons  is  much  higher  than  in  abstainers. 


DELIRIUM  TREMENS  929 

Delirium  Tremens. — Delirium  tremens,  whUe  an  acute  manifesta- 
tion of  alcoholism,  can  only  occur  in  a  person  suffering  from  the  effects 
of  chronic  alcoholic  poisoaing.  It  may  manifest  itself  sometimes  as 
the  result  of  a  prolonged  debauch,  sometimes  as  the  result  of  an  illness 
or  injury  in  a  chronic  alcoholic.  It  has  often  been  maintained,  and  is 
still  believed  by  many,  that  the  delirium  is  not  infrequently  the  result 
of  a  sudden  withdrawal  of  alcohol,  as  for  example  when  a  patient  is 
taken  to  the  hospital  for  an  injury  nothing  is  thought  of  his  alcoholic 
tendencies  until  he  develops  a  delirium.  In  these  cases  the  delirium 
is  presumed  to  be  due  to  the  fact  that  the  patient  did  not  get  his  usual 
supply  of  alcohol.  There  is  no  good  reason  for  this  opinion,  and  it 
must  be  remembered  that  in  the  prodromal  period  of  delirium  tremens 
not  infrequently  the  patient  has  experienced  a  disgust  of  liquor  for  a 
number  of  days.  A  pretty  effective  negation  of  the  theory  of  an 
abstinence  delirium  is  derived  from  the  English  prison  statistics,  which 
show  that  in  the  year  1907  there  were  G3,000  inebriates  who  were 
suddenly  deprived  of  alcohol  by  confinement,  and  in  this  number  only 
246  developed  delirium  tremens,  less  than  one-half  of  1  per  cent. 

Symptoms. — ^The  s3^nptoms  of  delirium  tremens  may  come  on  slowly 
being  preceded  by  several  days  of  general  physical  upset,  with  transient 
sensory  falsifications  and  perhaps  delusional  interpretation,  which 
show  a  special  tendency  to  come  on  at  night  or  under  conditions  in 
which  accurate  perception  is  interfered  with.  On  the  other  hand, 
the  delirium  may  come  on  very  rapidly.  A  case  is  recalled  of  a  long- 
shoreman, a  man  of  perhaps  thirty-five,  a  giant  in  physique,  who  walked 
into  the  hospital  at  noon  with  a  sprained  ankle;  the  ankle  was  strapped, 
and  he  was  put  to  bed.  That  night  he  was  in  the  wildest  delirium, 
and  the  next  morning  he  was  dead. 

Following  these  prodromal  symptoms  the  delirium  appears  in  its 
complete  manifestations.  It  is  a  t}T)ical  toxic  delirium  with  multiform 
disorders  of  the  sensorium.  The  hallucinations  predominate  in  the 
visual  sphere,  although  tactile  hallucinations  are  very  common.  The 
patient  is  manifestly  very  sick,  he  is  greatly  depressed  physically,  he 
is  tremulous,  the  tremor  being  so  constant  as  to  have  given  the  name 
to  the  condition,  and  his  mood  is  characteristically  one  of  apprehension, 
anxiety,  and  fear.  lie  is  disoriented,  mistakes  the  people  about  him; 
not  infrequently  his  delirium  is  an  occupation  delirium  and  he  believes 
himself  back  at  his  accustomed  work,  but  it  is  characteristically  filled 
with  fearsome  sights,  and  he  is  in  constant  terror  from  the  invasion  of 
the  numerous  animals  that  he  may  see  about  him. 

In  the  somatic  realm  the  pulse  is  rapid,  the  tremor  constant,  the 
skin  bathed  in  perspiration,  the  tongue  foul,  the  appetite  nil,  and  there 
may  perhaps  be  a  marked  grade  of  albuminuria.  The  temperature  is 
characteristically  either  normal  or  below  normal,  but  it  may  be  high, 
in  which  case  we  have  what  is  known  as  the  fchrile  ti/pe  of  the  disease, 
which  is  generally  fatal.  All  sorts  of  complications  may  naturally 
occur.  Acute  cardiac  dilatation  sometimes  causes  death,  while  one 
of  the  most  frequent  of  the  complications  in  severe  cases  is  "  wet  brain." 
59 


930  THE  TOXIC  PSYCHOSES 

In  this  condition  the  patient  sinks  into  a  low  muttering  delirium,  the 
temperature  falls  to  subnormal,  the  face  is  pale  and  bathed  in  cold 
perspiration,  the  pupils  dilated,  there  may  be  some  rigidity  of  the  neck 
with  a  tendency  to  a  bending  back  of  the  head ;  the  patient  sinks  into 
a  comatose  condition  and  dies. 

The  psychosis  runs  an  acute  course  of  about  three  days  and  usually 
terminates  by  a  long  sleep  in  recovery.    Ten  to  15  per  cent.  die. 

Treatment. — ^The  treatment  of  delirium  tremens  should  be  supporting, 
liquid  concentrated  food,  predigested  if  necessary.  The  bowels  and 
the  kidneys  should  be  freely  flushed,  heart  stimulants  are  necessary 
to  prevent  cardiac  failure,  and  h^iDuotics  often  required  to  produce  rest. 
For  the  excitement,  hydrotherapy  in  the  form  of  the  continuous  bath, 
or  if  this  is  not  available,  cold  packs  are  preferable  to  drugs.  The 
constant  thing  to  be  kept  in  mind  is  the  support  of  the  strength  of  the 
patient,  and  the  logical  way  to  accomplish  this  end  is  by  feeding.  If 
the  patient  refuses  food  no  time  should  be  wasted.  He  should  be 
immediately  fed  with  the  tube,  preferably  as  often  as  three  times  a 
day  in  small  amounts,  watching  the  stools  and  governing  the  quantities 
given  in  each  feeding  by  the  amount  the  patient  is  able  to  digest.  If 
the  patient  is  unable  to  retain  the  food  owing  to  acute  irritability  of 
the  stomach  and  constant  vomiting,  feeding  by  the  rectum  should  be 
resorted  to,  while  if  the  prostration  is  extreme  and  there  is  emaciation 
and  the  deprivation  of  fluid  considerable,  h^^odermoclysis  is  a  valuable 
adjunct. 

Korsakow's  Psychosis. — This  psychosis  is  found  typically  in  asso- 
ciation with  alcoholic  polyneuritis,  although  the  same  mental  state 
may  be  found  with  a  polyneuritis  of  different  origin,  as  for  example  the 
metallic  poisons,  some  of  the  infections  such  as  tuberculosis  and 
influenza,  and  some  of  the  endogenous  toxins  as  in  diabetes.  The 
s;sTidrome  occurs  also  in  connection  with  general  organic  changes  of 
the  central  ner\'ous  system  as  in  paresis,  arteriosclerosis,  and  senility. 
In  the  latter  case  it  is  a  part  of  the  clinical  picture  of  presbyophrenia. 

While  this  psychosis  occurs  t^'pically  in  connection  with  pohnieuritis, 
the  evidences  of  a  poljiieuritis  may  be  very  slight.  They  should  be 
carefully  examined  for,  especiallj^  by  pressure  over  the  large  nerve 
trunks,  which  will  often  elicit  painful  pomts,  rather  typically  at  the 
points  of  exit  through  the  bony  foramma.  The  Lasegue  sign  should 
be  sought  for. 

Korsakow's  psychosis  is  sometimes  designated  in  contradistinction 
to  delirium  tremens,  which  is  spoken  of  as  an  acute  alcoholic  delirium, 
as  a  chronic  alcoholic  delirium.  In  fact  the  attack  may  begin  with 
a  typical  delirium  tremens  which  merges  into  the  chronic  delirium  of 
Korsakow's  psychosis  instead  of  clearing  up,  although  this  is  not  the 
usual  method  of  onset.  Not  infrequently,  however,  a  history  of 
previous  attacks  of  delirium  tremens  may  be  elicited. 

The  mental  symptoms  are  the  result  of  a  combination  of  attention 
and  memory  disorders.  The  memory  disorder  is  of  the  nature  of 
a  lack  of  impressibility.    The  result  of  this  combination  is  a  special 


*  KORSAKOW'S  PSYCHOSIS  931 

tj'pe  of  amnesia.  There  is  defect  in  the  recording  of  current  events. 
The  patient  is  usually  disoriented,  to  some  extent  at  least,  and  the 
things  that  have  recently  happened  cannot  be  recalled.  These  defects 
of  memory  are  characteristically  supplied  by  fabrications  of  all  sorts 
of  degrees  of  probability  and  grotesqueness,  which  are  usually  related 
by  the  patient  with  a  composed  bearing  and  with  every  outward 
appearance  of  relating  facts,  or  at  least  occurrences  which  he  himself 
believes.  These  fabrications  do  not  correspond  if  the  patient  is  asked 
at  intervals  about  occurrences  covering  a  certain  period,  and  they  may 
often  be  guided  by  suggestions  from  the  questioner.  Not  infrequently 
the  patient  in  his  fabrications  invents  occurrences  which  account  for 
his  s\Tnptoms,  for  example,  a  patient  who  is  suffering  from  a  certain 
amount  of  pain  in  his  legs  as  a  result  of  his  polyneuritis,  even  though 
he  may  have  been  confined  to  his  bed  for  weeks,  will  say  that  he  was 
out  that  morning  and  climbed  a  long  hill  and  tired  his  legs  out,  and  in 
that  way  he  accounts  for  their  aching. 

The  experiences  which  these  patients  relate  are  of  a  delirious  char- 
acter and  not  infrequently  it  is  rather  difficult  to  distinguish  the 
fabrications  from  the  dreams,  and  it  would  appear  that  the  two  often 
merge  into  one  another. 

Physically  the  patient  usually  presents  the  signs  of  a  pol^^leuritis, 
although  these  may  be  very  mild.  When  the  neuritis  is  severe,  foot- 
drop  and  wrist-drop  are  characteristic  signs,  as  the  nerves  of  the  exten- 
sors of  the  forearm  and  leg  are  most  characteristically  involved  in 
alcoholic  neuritis. 

The  serious  complications  of  the  disease  are  either  dependent  upon 
intercurrent  affections  such  as  pneumonia  or  upon  the  involvement  of 
important  nerves  such  as  the  vagus,  vagus  involvement  of  course  being 
pretty  apt  to  be  fatal.  A  serious  complication  is  a  neuritis  of  the 
phrenic  ner\'e  with  paralysis  of  the  diaphragm  on  one  or  both  sides 
with  resulting  tympanitis  and  serious  embarrassment  of  cardiac  and 
respiratory  activity. 

Pathology. — The  pathology  of  the  disease  shows  that  it  is  by  .no 
means  confined  to  the  peripheral  ners'es  nor  even  to  the  motor  areas 
of  the  central  nervous  system,  but  that  there  is  a  very  general  involve- 
ment of  the  entire  central  nervous  system,  the  cord,  basal  ganglia, 
and  the  cortex  all  showing  lesions,  although  there  ai)pears  to  be  a 
tendency  to  the  focalization  of  the  pathological  process.  As  a  result 
certain  focal  symptoms  as  aphasia,  apraxia,  hemianopia,  etc.,  are  found. 
This  wide  distribution  of  the  pathological  disturbances  and  their  ten- 
dency to  focalization  is  due  to  the  fact  that  the  disease  involves  the 
bloodvessels.  The  smaller  vessels  proliferate  and  present  evidences 
of  endo-,  meso-  and  periarteritis  with  frequent  ru])tures.  When  this 
condition  is  focalized  in  the  midbrain  and  gives  rise  to  various  ocular 
palsies  the  syndrome  of  acute  hemorrhage  pollocncephaltii.s  of  U'cruiclcc 
is  present.  This  is  not  infrequently  observed  in  chronic  alcoholics. 
The  condition  is  typically  ushered  in  by  severe  and  prolonged  vomiting, 
which  is  followed  bv  marked  confusion  with  delirium,  \ertigo,  some- 


932  THE  TOXIC  PSYCHOSES 

times  headache,  and  somnolence.  Optic  neuritis  may  be  present. 
Death  usually  occurs  in  from  one  to  two  weeks. 

With  a  disease  of  this  character  having  a  pathology  so  widely 
distributed  throughout  the  nervous  system  and  dependent  upon 
chronic  toxemia  it  can  be  understood  how  very  many  types  have  been 
described.  These  types  or  clinical  forms  are  nothing  more  than 
descriptions  of  the  disease  in  which  certain  symptoms  are  especially 
prominent,  and  so  there  are  amnesic,  confusional,  delusional,  anxious, 
demented,  delirious,  stuporous,  and  hallucinatory  forms  described  by 
different  authors. 

Treatment. — The  treatment  of  the  condition  is  of  course  the  with- 
drawal of  alcohol  and  the  treatment  of  a  general  toxic  state  along 
supportive  lines.  The  deformities  which  are  due  to  the  paralysis 
should  be  dealt  with  by  recognized  orthopedic  measures.  It  should  be 
remembered  that  while  the  patient  is  in  bed  and  delirious  the  weakened 
extensors  of  the  forearm  and  leg  should  not  be  stretched  by  the  weight 
of  the  limb,  but  should  be  supported,  particularly  the  weight  of  the 
bed  clothes  should  be  removed  from  the  feet. 

Alcoholic  Hallucinosis. — This  condition  is  also  an  expression  of 
chronic  alcoholism  and  may  be  preceded  by  attacks  of  delirium  tremens. 
It  is  characterized  by  hallucinations,  auditory  predominating,  in  this 
respect  strongly  contrasted  to  delirium  tremens,  and  delusions  of  a 
persecutory  character  which  harmonize  and  are  explanatory  of  the 
hallucinations.  It  is  quite  characteristic  that  the  hallucinations  and 
the  delusions  deal  with  sexual  matters,  the  patient  frequently  being 
abused  by  "the  voice"  for  committing  some  sexual  crime  or  is  accused 
of  sexual  perversions.  The  delusions  are  practically  invariably  persecu- 
tory, although  occasionally  expansive  elements  may  enter  the  picture. 
Not  infrequently,  too,  the  patient  is  very  much  frightened,  as  when  he 
thinks  he  is  being  closely  pressed  by  his  persecutors  who  are  determined 
upon  takmg  his  life.  There  are  not  a  few  borderline  mixtures  of  this 
psychosis  and  delirium  tremens. 

The  condition  is  essentially  an  acute  paranoid  state  and  as  such 
its  explanation  is  the  same  as  the  explanation  of  paranoia.  In  other 
words,  there  is  an  unconscious  fixation  at  the  homosexual  level,  and 
this  accounts  for  the  very  great  frequency  of  the  sexual  character  of 
the  hallucinations  and  the  references  to  sexual  perversions.  The 
alcohol  which  is  taken  by  the  individual  because  he  cannot  deal 
efficiently  with  reality  causes- him  to  turn  back  upon  himself  and 
produces  a  reanimation  of  this  early  fixption,  and  then  the  mechanism 
of  projection  comes  in  as  a  distortion  to  make  it  appear  that  the 
suffermg  has  its  origin  from  without. 

A  recent  analysis  of  cases  by  Schneider^  has  brought  out  the  make-up 
of  the  individual  in  a  type  of  psychosis  which  has  too  often  been  thought 
to  be  sufficiently  explained  by  alcohol.  Aside  from  the  fact  that 
hallucinosis  occurs  a,side  altogether  from  alcoholic  indulgence  he  found 

'  Schneider,  Carl  von  A.:  Studies  on  Alcoholic  Hallucinoses,  Psychiatric  Bulletin, 
January,  1916, 


ALCOHOLIC  PSEUDOPARANOIA  933 

that  repeated  debauches  may  terminate  without  hallucinosis,  that  this 
only  supervenes  as  the  result  of  a  special  precipitating  factor.  The 
precipitating  factor  is  of  psychogenic  nature,  mental  shock  followed 
by  worry  and  the  content  of  the  hallucinosis  is  determined  by  the 
nature  of  the  experience. 

The  delusional  system  is  rapidly  systemized  so  that  in  certain 
cases  the  patient  might  give  the  impression  of  a  paranoiac.  The 
course  of  the  disease  is  usually  prolonged  over  a  number  of  weeks, 
and  not  infrequently  over  a  number  of  months.  It  generally  ends  in 
recovery,  but  sometimes  merges  into  chronicity.  The  disease  is  not 
fatal  and  its  pathology  is  therefore  the  pathology  of  chronic  alco- 
holism. 

Alcoholic  Pseudoparesis. — In  a  few  patients  prolonged  alcoholic 
intoxication  produces  a  picture  closely  resembling  general  paresis. 
In  these  cases  we  find  an  expansive  delirium  combined  with  the  signs 
of  alcoholism,  such  as  ataxia,  speech  defects,  and  tremor.  It  must  be 
remembered,  too,  that  pupillary  anomalies  quite  regularly  occur  in 
severe  grades  of  intoxication  and  even  an  Argyll-Robertson  pupil 
has  been  observed.  These  observations  should  be  taken  with  a 
certain  amount  of  reservation  because  it  is  quite  impossible  often 
to  tell,  without  the  aid  of  instruments,  exactly  what  the  pupillary 
reaction  may  be  and  especially  whether  it  is  entirely  lost,  and  it  is 
generally  conceded  that  toxic  conditions  will  produce  a  slowing  of  the 
light  reflex.  On  the  other  hand,  it  has  been  thought  by  some  that 
where  these  marked  pupillary  disturbances  are  found  it  indicates 
the  presence  of  syphilis.  Therefore  these  observations  should  be 
correlated  with  the  serological  findings.  A  case  has  been  recently 
reported  by  Xonne,^  however,  in  which  the  Argyll-Robertson  pupil 
was  demonstrated  to  be  due  to  alcohol:  lues  was  excluded  by  nega- 
tive four  reactions,  optic  neuritis  was  excluded  by  careful  ophthal- 
mological  examination,  and  the  light  reflex  returned  upon  with- 
drawal of  alcohol.  These  cases  clear  up  promptly  upon  the  removal 
of  alcohol. 

Alcoholic  Pseudoparanoia. — In  some  patients  with  chronic  alcoholism 
a  fairly  circumscribed  delusional  system  may  develop  which  character- 
istically takes  the  form  of  delusions  of  marital  infidelity.  In  endeavor- 
ing to  interpret  this  delusion  the  paranoia  mechanisms  must  be  borne  in 
mind.  Quite  commonly,  however,  the  delusion  when  it  occurs  in  a  man 
is  a  defense  reaction  to  impotence,  which  has  been  largely  produced, 
probably,  by  the  alcoholic  indulgence.  Instead  of  realizing  his  own 
impotence,  which  is  an  inacceptable  thought,  he  blames  his  wife  for 
being  untrue  to  him.  These  cases  are  essentially  chronic  and  persist 
at  least  as  long  as  the  alcohol  is  indulged  in,  while  even  when  it  is 
removed  they  may  be  a  long  time  clearing  up  and  may  perhaps  go 
over  into  a  chronic  delusional  state  largely  because  of  an  unconscious 
homosexual  fixation. 

1  Neurol.  Centralbl.,  1915,  Nos.  7-8.    Also  see  section  on  Eye  Reflexes  for  other  cases. 


934  THE  TOXIC  PSYCHOSES 

Alcoholic  Epilepsy. — In  a  chronic  alcoholic  toxemia  it  is  not  strange 
that  epileptiform  convulsions  should  occasionally  develop  and  recur 
from  time  to  time.  The  outward  characteristics  of  the  convulsion 
are  in  every  way  the  characteristics  of  an  epileptic  attack.  If  the 
person  is  not  essentially  epileptogenic  the  convulsions  will  subside 
on  the  removal  of  the  alcohol. 

Dream  States. — In  conditions  of  pathological  drunkenness  it  is  not 
infrequent  for  the  patient  to  have  no  recollection  of  the  period 
during  which  he  was  intoxicated.  If,  during  this  period,  he  has  been 
engaged  in  some  sort  of  occupation,  business  transaction,  travel,  or 
what  not,  or  perhaps  engaged  in  the  performance  of  criminal  acts,  he 
may  still  wake  up  with  no  recollection  at  all  of  this  period,  although 
during  it  he  outwardly  appeared  to  be  in  a  normal  state.  Some  people 
are  especially  liable  to  these  dream  states.  It  would  seem  that  they 
present  a  tendency  to  the  doubling  of  their  personality  and  that  the 
alcohol  helps  to  produce  these  somnambulistic  episodes. 

Dipsomania. — Dipsomania  is  a  term  applied  to  a  periodical  impulse 
to  drink.  Drinking  is  only  the  outward  manifestation  of  the  trouble. 
It  is  a  recurrent  neuropathic  attack  which  demands  the  narcotizing 
result  of  alcoholic  indulgence  and  is  not  infrequently  a  manifestation  of 
the  manic-depressive  psychosis  or  of  a  compulsion  neurosis. 

Opium. — The  habitual  use  of  opium  in  some  form  has  become 
common  among  all  classes  in  society.  The  same  thing  may  be  said 
with  reference  to  the  reasons  for  taking  opium  as  has  been  said  with 
reference  to  alcohol.  The  opium  habitue  is  a  person  primarily  of  neuro- 
pathic taint,  the  mere  opium  taking  or  the  symptoms  it  produces  being 
but  surface  indications  of  the  real  trouble. 

Symptoms. — The  general  symptoms  of  its  use  are  well  known.  In 
small  doses  it  is  mildly  stimulating  and  produces  a  pleasant  euphoria, 
while  if  it  is  taken  in  large  doses  and  continuously  it  leads  to  a  mental 
blunting,  a  general  feeling  of  malaise,  inability  to  make  effort,  with 
marked  physical  disturbances  such  as  tremor,  muscular  weakness, 
constipation,  paresthesias,  etc.  It  is  not  infrequent  for  opium  in  some 
of  its  forms  to  be  combined  with  addiction  to  alcohol  or  cocain.  Con- 
tinuous use  may  simply  produce  a  thoroughly  crippled  individual  who 
is  alternately  in  a  mildly  euphoric  state  or  in  a  condition  of  stupidity, 
malaise,  and  indolence.  When  a  psychosis  develops,  it  is  character- 
istically a  hallucinated  state  usually  with  paranoid  coloring,  or  it  may 
be  distinctly  delirious.  The  prognosis  is  of  course  not  good  unless  the 
underlying  neuropathic  taint  can  be  dealt  with. 

The  use  of  opium  has  much  less  tendency  than  that  of  alcohol  to 
produce  tissue  degeneration. 

Treatment. — The  treatment  is  usually  the  prompt  removal  of  the 
large  doses.  These  can  be  readily  withdrawn  until  the  patient  is 
reduced  to  the  actual  necessary  amount  of  drug  to  get  through  the 
day  comfortably  with,  which  is  about  two  grains.  From  this  point  on 
withdrawal  should  be  gradual,  carefully  looking  after  the  symptoms 
of  abstinence  as  they  appear.    These  symptoms  refer  particularly  to 


BROMIDES  935 

the  heart,  the  nervous  system,  and  the  gastro-intestinal  system.  Some- 
times profound  collapse  may  occur  with  the  withdrawal  of  the  drug. 
Cardiac  conditions  should  be  watched  and  stimulants  administered 
if  there  is  any  sign  of  weakness.  For  the  diarrhea  opium  should  be 
avoided  if  possible.  Acute  withdrawal  and  treatment  by  atropine  for 
the  sympathetic  collapse,  and  pilocarpine  or  eserine,  for  autonomic 
stimulation,  may  be  practised  to  advantage. 

Cocain. — Cocain  is  taken  for  the  same  reasons  that  alcohol,  mor- 
phin  and  other  drugs  are  taken,  but  it  is  much  more  dominating 
in  its  influence  and  more  difficult  to  escape  from  than  either  of  the 
others,  while  it  is  more  disintegrating  than  morphin  or  opium. 

Symptoms. — The  symptoms  of  cocain  intoxication  are  those  of 
marked  stimulation.  The  patient  is  extremely  active  and  very  talkative, 
full  of  all  sorts  of  schemes,  ambitious,  tells  Avhat  he  is  going  to  do, 
unfolds  plans,  and  in  general  is  in  a  man'c  condition  of  mind.  Follow- 
ing this  hyperstimulation  there  is  of  course  profound  malaise  and 
exhaustion.  Associated  with  the  habitual  use  of  cocain  there  is  more 
apt  to  be  marked  and  persistent  sensory  disturbances  in  the  form  of 
paresthesias,  while  in  the  mental  sphere  the  disintegration  of  the  indi- 
vidual is  much  more  marked  than  with  opium,  as  a  rule.  The  moral 
sense  is  blunted,  he  lies  readily,  the  judgment  is  impaired,  and  not 
infrequently  they  develop  distinct  delusions  of  which  the  delusion  of 
marital  infidelity  is  not  infrequent.  Chronic  paranoid  conditions, 
hallucinatory  states,  and  deliria  are  seen  in  cases  of  long-continued 
habituation.  The  drug  may  be  withdrawn  much  more  rapidly  than 
morphin  since  it  does  not  contribute  so  largely  to  produce  an  infantile 
regression  as  does  morphin. 

Miscellaneous  Intoxicants. — A  large  number  of  drugs  are  taken 
habitually,  particularly  the  whole  group  of  pain-relieving  and  sleep- 
producing  drugs.  In  general  the  mental  condition  due  to  these  drugs 
varies  all  the  way  from  delirium  as  the  result  of  an  acute  intoxication 
to  chronic  paranoid  conditions.  The  main  feature  of  the  drug  deliria 
is  their  dream-like  character.  The  patients  have  all  sorts  of  grotesque 
experiences  which  they  weave  into  a  more  or  less  consistent  descrip- 
tion, elaborating  the  details  here  and  there  as  may  be  necessary.  The 
delirious  experiences  pass  like  moving  pictures  before  the  patient's 
mind,  and  they  not  infrequently  are  perfectly  composed  as  they 
recount  them,  no  matter  how  grotesque  or  unusual  or  even  terrifying 
some  of  them  may  be.  The  chief  underlying  motive  for  the  taking  of 
these  drugs  is  to  escape  from  reality  as  much  as  possible  and  to  get 
into  a  world  of  fantasy. 

Bromides.— The  possibility  of  bromide  delirium  from  the  taking 
of  large  doses  of  bromides  for  a  long  period  of  time  should  be  borne 
in  mind.  Patients  are  not  infrequently  admitted  to  hospitals  sup- 
posedly suffering  from  some  psychosis  l)ut  with  a  bromide  delirium, 
the  bromide  having  been  administered  to  quiet  the  nervousness  of 
a  neurosis,  or  perhaps  given  in  the  treatment  of  an  epilepsy.  The 
average  practitioner  does  not  appear  to  realize  the  possibility  of  chronic 
poisoning  that  prescriptions  of  this  sort  present. 


936  THE   TOXIC  PSYCHOSES 

Carbon  Monoxide. — Carbon  monoxide  delirium  is  of  special  impor- 
tance in  connection  with  the  frequent  attempts  at  suicide  with  illumi- 
nating gas  that  one  meets  w^ith  in  our  large  cities.  The  principal  thing 
to  be  thought  of  in  connection  with  carbon  monoxide  poisoning  is  that 
after  the  initial  effects  of  the  poisoning  have  sudsided  and  the  patient 
comes  out  of  the  coma  there  may  be  quite  a  rapid  return  to  normal 
and  the  patient  be  in  an  apparently  normal  condition  for  a  week  or 
ten  days  and  then  a  relapse  occur  with  marked  mental  distm-bance. 
The  patient  should  never  be  discharged  from  the  hospital  until  after 
this  period  of  danger  has  been  passed.  Delirium  is  characteristic  of 
this  form  of  poisoning,  and  an  amnesia  for  the  period  usually  follows, 
often  associated  with  fabrications  and  pseudoreminiscences,  producing 
a  Korsakow  syndrome.  Disturbances  of  speech  are  also  common,  as 
are  disturbances  in  the  emotional  field,  such  as,  for  example,  causeless 
laughter.  Pathologically  there  appear  to  be  thrombotic  occlusions  and 
hemorrhages  which  characteristically  are  found  in  the  basal  ganglia, 
and  in  this  region  may  account  for  the  disturbances  of  mimic. 

Lead. — In  chronic  lead  poisoning  we  may  find  a  condition  of  pseudo- 
general  paresis  or  Korsakow's  syndrome  in  addition  to  the  ordinary 
hallucinatory  and  delirious  episodes. 

Mercury. — This  poison  produces  characteristically  a  condition  of 
great  irritability  associated  with  insomnia  and  anxiety.  This  condi- 
tion may  become  more  pronounced  and  develop  into  a  well  marked 
delirium.  Subacute  mercurial  poisoning  may  bring  on  a  typical 
vagotonic  condition  with  the  physical  symptoms  of  general  or  local 
vagotonia,  the  somatic  symptoms  of  visceral  vagus  alterations  or  the 
psychical  analogies  of  an  anxiety  hysteria,  or  a  manic-depressive 
psychosis. 

There  are,  of  course,  other  poisons,  but  these  are  the  principal  ones. 

General  Considerations. — With  regard  to  all  of  the  habit-forming 
drugs,  particularly  alcohol,  opium,  cocain,  and  the  analgesics  and 
hypnotics,  it  may  be  said,  as  already  indicated  in  discussing  them 
separately,  that  the  true  cause  of  addiction  lies  in  the  make-up  of  the 
individual.  This  cause  would  appear  to  be  a  narcissistic  or  homo- 
sexual fixation.  This  fixation  and  its  results  are  by  no  means  simple 
matters  psychologically  and  differ  very  materially  in  different  individ- 
uals. The  only  hope  for  the  treatment  of  this  group  of  cases,  however, 
is  an  ability  to  modify  this  character  anomaly.  The  treatment,  of 
course,  therefore  can  only  be  tentative  and  symptomatic  during  the 
periods  of  acute  disturbance,  and  it  is  in  the  interval,  when  the  patient 
is  free  from  the  psychosis,  that  the  treatment  should  be  applied. 
This  is,  of  course,  the  psychoanalytic  treatment  and  should  be  addressed 
to  discovering  the  underlying  etiological  factors.  Unless  something 
can  be  accomplished  in  this  direction  very  little  is  to  be  hoped  for. 
The  mere  palliative  treatment  by  drugs  is  largely  a  medical  illusion, 
sometimes,  as  in  specially  vaunted  systems  of  cure,  a  delusion  or 
quackery. 

As  regards  the  metallic  poisons  associated  with  dangerous  trades,  of 


GASTRO-INTESTINAL  DISEASES  937 

course,  the  treatment  here  is  purely  s^inptomatic,  eHminative,  and 
expectant,  while  the  real  effort  that  is  to  be  of  material  help  should  be 
expended  in  prophylaxis. 

Uremia. — In  acute  uremia  the  convulsion  is  one  of  the  most  typical 
signs,  and  cannot  be  distinguished  in  its  outward  manifestations  from 
the  ordinary  epileptic  attack.  Similarly  with  epilepsy,  too,  there  may 
be  quite  well  defined  delirioid  experiences  or  dream  states  in  which 
there  is  disorientation  and  noticeable  disturbance  of  the  sensorium, 
usually  associated  with  more  or  less  constant  acti\ity.  There  may  be 
an  anxious  affect,  or  the  patient  may,  as  is  quite  usual  in  delirium, 
merely  be  interested  in  hallucinatory  experiences.  Acute  uremic 
sjTiiptoms,  in  the  mental  sphere,  of  this  character  may  accompany 
exacerbations  of  the  physical  condition  in  a  patient  suffering  from 
chronic  nephritis. 

In  chronic  uremia  one  finds  not  infrequently  marked  mental  svTnp- 
toms.  The  mental  symptoms  are  usually  of  the  character  of  defect,  a 
general  stupidity  with  a  feeling  of  physical  weakness,  desire  to  sleep, 
with  perhaps  complaints  of  headache,  associated  with  irritability. 
Along  with  the  mental  symptoms  may  go  tremor,  speech  disturbances, 
eye  muscle  palsies,  pupillary  differences,  sluggish  or  failing  light  reac- 
tion, which  point  to  an  organic  disease  of  the  brain.  If  along  with  this 
condition  there  are  Jacksonian  attacks  followed  by  mono-  or  hemi- 
plegia with  perhaps  aphasia  and  distm-bances  of  vision,  the  similarity  to 
general  paresis  on  the  one  hand  and  to  brain  tumor  on  the  other  may 
be  ver\-  great.  Sometimes  along  with  these  symptoms  the  mood  is 
distinctly  euphoric,  which  still  further  suggests  the  possibility  of 
paresis.  A  certain  number  of  the  cases  of  uremic  psychoses  show  close 
relationships  to  dementia  precox  showing  negativism,  incoherence, 
catatonia.    Probably  this  group  is  more  serious  in  its  prognosis. 

Diabetes  Mellitus. — The  mental  disorder  which  is  associated  with 
diabetes  is  usually  of  a  mild  depressive  type,  often  with  ideas  of  ruin 
and  sin.  Anxiety  states  have  also -been  described.  Occasionally  the 
picture  gives  somewhat  of  the  impression  of  general  paresis.  With 
the  chronic  depressed  condition  paranoid  ideas  sometimes  develop. 

In  general  the  severity  of  the  mental  sjTiiptoms  may  alternate  with 
the  severity  of  the  physical  disease,  as  indicated  by  the  sugar  elimi- 
nated. When  the  intoxication  is  very  great  the  patient  may  be 
irritable,  sleepy,  stupid,  complain  of  weakness,  and  headache. 

The  diabetic  state  itself  is  an  etiological  factor  in  the  production  of 
arteriosclerosis,  and  evidences  of  arteriosclerosis  ma\'  be  found  asso- 
ciated with  the  condition.  It  should  be  remembered,  however,  that 
both  the  arteriosclerotic  condition  and  the  senile  state  interfere  with 
the  metabolism  of  the  sugars,  and,  therefore,  may  themselves  be 
associated  with  glycosuria. 

Gastro-intestinal  Diseases. — In  general  it  is  well  known  that  with 
diseases  below  the  diaphragm  there  is  associated  on  the  mental  side  a 
depressive  mood.  The  relation  between  diseases  of  the  gastro-intestinal 
tract  and  associated  glands  to  mental  states  is  extremely  difficult  to 


938 


THE   TOXIC  PSYCHOSES 


interpret.  Many  of  the  gastro-intestinal  disturbances,  rather  than 
being  causes  of  mental  disease,  are  effects.  This  is  particularly  seen 
in  the  realm  of  the  neuroses  and  the  psychoneuroses,  and  is  discussed 
in  the  chapter  dealing  with  these  conditions. 

Certain  cases  of  acute  confusion  develop  associated  with  profuse 
and  offensive  diarrhea,  a  high  grade  of  indicanuria,  vomiting,  low  fever, 
and  perhaps  mild  albuminuria.  Some  of  these  cases  go  on  to  acute 
delirium,  with  tj-phoid  state,  profound  exhaustion,  coma,  and  death. 
Just  exactly  where  these  cases  belong  and  what  they  mean  is  not 
altogether  known. 


Fig.  446. — Skin  lesions  of  pellagra. 

Pellagra. — There  has  been  very  little  study  of  the  mental  symptoms 
of  pellagra  of  late  years.  From  the  few  cases  observed  and  from 
study  with  those  who  have  had  it  under  observation  there  seems  to 
be  a  variety  of  symptom  pictures. 

Many  cases  present  no  nervous  or  mental  symptoms  at  all.  In 
those  who  do  there  seems  to  be  a  tendency  toward  a  variable  localiza- 
tion of  the  disease  process.  There  seem  to  be  patients  in  whom  the 
spinal  cord  suffers  most  and  others  in  whom  the  brain  suffers  most.  In 
this  latter  group  a  condition  of  very  acute  delirium  may  be  developed 
running  a  rapid  course  to  fatal  termination  and  reminding  one  of  the 
acute  forms  of  paresis.  The  more  frequent  condition,  of  which  we 
have  seen  a  number  of  cases,  seems  to  be  more  in  the  nature  of  a 
simple  retardation.  The  patient  moves  slowly,  or  not  at  all,  and 
answers  questions  after  a  long  delaj^  in  a  low  tone  of  voice  and  in 


PELLAGRA  939 

monosyllables.  There  does  not  go  with  this  retardation,  however, 
a  corresponding  emotional  depression  as  in  melancholia.  Pellagra- 
phobia  has  been  observed  in  an  infected  territory. 

^Yith  this  disease,  as  with  many  others,  it  must  not  be  forgotten  that 
it  may  be  associated  with  various  psychoses  without  having  any  specific 
relation  to  them.  This  is  peculiarly  so  in  this  country,  as  the  large 
groups  of  cases  which  have  occurred  have  been  in  hospitals  for  the 
insane. 

Perhaps  Gregor^  has  made  the  most  careful  recent  clinical  study 
of  the  mental  symptoms.  He  considers  his  cases  under  the  followmg 
seven  categories:  (1)  Pellagrous  neurasthenia,  (2)  stuporous  group, 
(3)  mental  aberration,  (4)  acute  delirium,  (5)  katatonia,  (6)  anxiety 
psychosis,  (7)  manic-depressive  group.  It  does  not  seem  clear,  how- 
ever, just  what  is  the  connection  in  all  of  these  cases  between  the 
pellagra  and  the  psychosis. 

1  Beitriige  zur  Kenntnis  der  pellagrosen  Geistesstorungen,  Jahrb.  f.  Psych,  u.  Neurol., 
1907. 


CHAPTER  XXII. 
PSYCHOSES  ASSOCIATED  WITH  ORGANIC  DISEASES. 

Apoplexy.— Immediately  following  the  apoplectic  insult  the  patient 
is  quite  commonly  unconscious.  As  the  symptoms  subside  and  the 
patient  begins  to  "come  to"  there  may  be  marked  disorientation  to 
the  extent  of  a  mild  delirium,  especially  if  fever  be  present.  This 
SATiiptom  of  a  slight  rise  in  temperature  with  onset  of  mild  delirium 
sometimes  occurs  a  few  days  after  the  original  insult  and  is  then  of 
bad  prognostic  omen.  In  severe  attacks  the  unconsciousness  may 
pa^is  on  into  profound  coma  and  death. 

Ordinarily  followuig  an  apoplectic  insult  and  after  recovery  from  the 
acute  symptoms  a  condition  of  more  or  less  impairment  is  left,  and 
when  it  is  borne  in  mind  that  the  patients  who  suffer  from  apoplexy 
are  in  the  main  in  the  senile  or  arteriosclerotic  period  it  will  be  under- 
stood that  the  symptoms  of  the  defect  take  on  characteristically  symp- 
toms of  these  two  conditions,  which  perhaps  become  aggravated 
materially  following  the  insult.  The  defect,  however,  may  appear  to 
be  very  much  greater  than  it  really  is,  because  of  the  inability  of  the 
patient  to  express  himself  owing  to  incident  aphasia  or  apraxia.  The 
emotional  attitude  of  the  patients  is  usually  one  of  irritability,  although 
indifference  also  enters  into  the  picture.  Such  patients  often  lie 
quietly  and  apparently  indifferent  until  an  attempt  is  made  to  com- 
municate with  them.  Under  these  circumstances  if  they  have  serious 
aphasic  or  apraxic  disturbances  and  know,  for  example,  what  they  wish 
to  say,  they  may  become  very  much  excited  and  quickly  fatigued  and 
emotional  as  a  result  of  their  repeated  efforts  and  failures  to  enter 
into  communication.  This  is  also  quite  characteristic  in  the  younger 
patients  with  apractic  disturbances  and  relatively  clear  intelligence. 
It  is,  so  to  speak,  "maddening"  not  to  be  able  to  control  any  longer 
the  power  of  expression.  Some  of  these  patients,  especially  those  who 
suffer  from  sensory  forms  of  aphasia,  being  out  of  communication  with 
their  fellows,  and  not  being  able  to  understand  what  goes  on  about 
them,  may  acquire  a  suspicious  attitude  which  gives  a  paranoid  color- 
ing to  their  mental  condition.  It  can  be  uaderstood  that  the  apoplexy 
which  produces  disorders  of  expression  and  interferes  with  the  patient's 
keen  contact  with  his  fellows  will  not  only  precipitate  the  deteriora- 
tion of  the  senium,  but  will  make  that  deterioration  much  more  rapid. 

The  neurological  signs  of  hemiplegia  are  present,  also  often  the 
signs  of  senility,  and  not  infrequently  of  arteriosclerosis,  while  a  certain 
few  of  these  patients  develop  epileptic  attacks.    (See  Chapter  XII.) 

Traumatism. — The  effects  of  trauma  may  be  divided  into  the 
immediate  or  acute  effects  and  the  more  remote  or  chronic  results, 
(940) 


TRAUMATISM  941 

into  which  the  former  may  or  may  not  merge.  The  ultimate  results 
are  divisible  into  changes  in  constitution  and  defect  conditions,  while 
a  certain  proportion  of  cases  develop  psychoses  following  an  injury 
without  there  being  any  special  connection  between  the  two  or  in 
which  the  injury  can  only  be  conceived  as  a  precipitating  or  contribu- 
toT}'  factor.  Oftentimes  the  injury-  is  the  result  rather  than  the  cause, 
as  is  so  frequently  seen  in  paresis,  the  injury  sers'ing  to  call  attention 
to  the  presence  of  a  disease  up  to  that  time  not  recognized.  Many 
shell  shock  cases  have  to  be  interpreted  in  this  broad  manner. 

The  usual  immediate  result  of  a  head  injury,  either  direct  or  indirect, 
is  a  certain  degree  of  unconsciousness  which  may  vary  all  the  way 
from  a  light  stupor  to  profound  coma.  \Mien  the  unconsciousness  is 
not  profound  the  patients  are  quiet,  but  if  disturbed  they  become  vers'- 
irritable  and  resistive.  Quite  topically,  associated  with  the  stupor, 
is  a  mild  delirium  of  hallucinatory'  character  which  is  especially  pro- 
nounced and  apt  to  be  more  violent  in  patients  who  are  the  subject 
of  alcoholism .  Associated  with  this  may  be  a  slight  rise  of  temperature, 
but  if  the  rise  is  pronounced  meningitis  should  be  thought  of.  Alternat- 
ing conditions  of  apparent  clearness  and  marked  confusion  may  occur. 
Usually  the  stupor  is  of  only  short  duration,  but  may  last  several  days 
and  finally  disappear.  In  severe  cases  the  patient  becomes  comatose 
and  dies  in  that  condition.  In  a  few  cases  the  delirious  reaction  is 
unusually  prolonged  and  may  be  associated  wi^h  Korsakow's  sjiidrome. 

After  recovers'  from  the  immediate  effects  of  the  injur}'  certain 
constitutional  changes  may  slowly  develop,  the  most  typical  of  which 
is  Friedmann's  comylex,  which  is  attributed  to  a  vasomotor  disturb- 
ance in  the  brain.  The  symptoms  of  this  complex  are  headache,  dizzi- 
ness, irritability,  insomnia,  physical  and  mental  fatigability,  a  certain 
change  of  character,  and  intolerance  of  alcohol,  with  which  may  be 
associated  a  nlemory-  defect  of  the  ty^e  of  retrograde  amnesia,  which, 
however,  is  not  a  part  of  the  complex,  although  there  may  be  a  certain 
memory'  defect  which  is  quite  common  to  all  highly  ners'ous  worried 
people.  The  headache  is  apt  to  be  complained  of  as  a  feeling  of  pain 
or  a  peculiar  feeling  of  constriction  of  the  head  and  is  associated  with 
feelings  of  fulness,  especially  on  stooping  over.  The  dizziness  is  also 
characteristically  intensified  by  stooping  or  by  sudden  movements. 
The  irritability  of  temper  may  be  so  marked  as  to  lead  to  explosive 
outbreaks,  which  simulate  psychic  epilepsy.  Kaplan  has  named  this 
condition  the  explosive  diathesis.  They  are  much  aggravated  by 
alcohol.  Sleep  is  liable  to  be  disturbed,  physical  and  mental  endiu-ance 
is  very  much  lessened,  and  the  patient  may  become  depressed,  moody, 
and  in  various  other  ways  show  a  subtle  change  in  character  which  is 
appreciated  by  all  of  his  close  associates.  The  intolerance  to  alcohol 
is  very  characteristic  of  this  post-traumatic  constitution.  Sometimes 
associated  with  the  explosive  diathesis,  but  also  occurring  alone,  are 
temporary  dream  states.  Various  other  hysteriform  and  epileptiform 
manifestations  may  also  occur. 

Finally,  certain  defects  are  left  as  a  result  of  the  traumatism,  depend- 


942       PSYCHOSES  ASSOCIATED   WITH  ORGANIC  DISEASES 

ing,  of  course,  upon  the  nature,  the  extent,  and  the  location  of  the 
injury.  As  regards  the  more  characteristic  results  of  head  injury  of  a 
distinctly  psychotic  nature,  it  should  be  borne  in  mind  that  the  change 
in  character,  transformation  of  the  personality,  as  it  might  be  called, 
is  often  a  very  subtle  process  and  one  extending  over  a  very  consider- 
able period  of  time.  It  might  be  practically  impossible  to  evaluate 
the  situation  at  all  if  one  were  dependent  upon  a  cross-section  of  the 
patient's  mental  life.  When  a  longitudinal  section,  however,  is  avail- 
able one  finds  in  typical  cases  an  individual  who  up  to  a  certain  point 
in  life  has  gotten  along  well,  showing  efficient  reactions  and  developing 
by  steady  progress  in  some  chosen  line  of  work.  Such  an  individual 
receives  a  head  injury  and  from  the  time  of  this  injury  on  there  will 
be  noted  in  the  history  a  gradual  falling  off  in  efficiency.  It  may  be 
quite  impossible  to  put  one's  finger  upon  any  specific  thing  in  the 
situation  and  set  it  down  as  a  pathological  type  of  reaction,  but  the 
individual  reaction  has  changed  in  character,  and  from  efficiency  there 
is  evolved  inefficiency.  Naturally,  it  is  a  long  time  before  the  true 
explanation  of  such  a  change  is  reached.  In  fact,  it  is  a  long  time  before 
it  is  realized  that  any  change  at  all  has  taken  place,  and  it  is  because 
of  this  fact  that  the  older  psychiatrists  used  to  speak  of  traumatic 
insanity  as  sometimes  eventuating  many  years  after  the  injury.  Not 
infrequently  this  falling  off  in  efficiency  has  going  with  it  a  gradual 
deterioration  in  morale,  and  with  the  heightened  susceptibility  of  the 
patient  to  alcohol,  which  trauma  produces,  it  is  natural  that  alcohol 
should  enter  into  the  picture  very  largely  and  often  be  regarded  as  the 
cause  of  the  whole  trouble. 

Meyer's^  classification  of  the  effects  of  traumatism  in  the  nervous 
system  is  as  follows: 

1.  The  direct  focal  and  the  more  diffuse  destruction  of  the  nerve 
tissue  or  of  parts  of  it;  and  the  reaction  of  the  tissues. 

(a)  The  immediate  effects — edema. 

(b)  The  scar  formation. 

2.  The  distinctly  diffuse  commotions  in  which  the  general  reaction 
and  the  psychic  elements  preponderate,  including  the  remote  reactive 
results  of  exaggerations  of  vasomotor  and  emotional  responsiveness. 

He  classifies  the  psychoses  developing  as  follows: 

1,  The  direct  jmst-traumatic  deliria  with  the  Jollowing  subdivisions: 
(a)  Preeminently  febrile  reactions. 

(6)  The  delirium  nervosum  of  Dupuytren,  not  differing  from  deliria 
after  operations,  injuries,  etc. 

(c)  The  delirium  of  slow  solution  of  coma  with  or  without  alcoholic 
basis. 

(d)  Forms  of  protracted  deliria  usually  with  numerous  fabulations, 
etc.  (with  or  without  alcoholic  or  senile  basis). 

2.  The  yost-traumatic  constitution. 

(a)  Types  with  mere  facilitation  of  reaction  to  alcohol,  grippe,  etc. 

>  The  Anatomical  Facts  and  Clinical  Varieties  of  Traumatic  Insanity,  Am.  Jour. 
Insanity,  January,  1904. 


ACUTE  CHOREA  943 

(6)  Types  with  vasomotor  neurosis. 

(c)  T^-pes  with  explosive  diathesis. 

(d)  TN-pes  with  hysteroid  or  epileptoid  episodes  with  or  without 
conMjlsions  (such  as  most  reflex  psychoses). 

(e)  T^-pes  of  paranoiac  development. 

3.  The  traumatic  defect  conditions. 

(a)  Primary  defects  allied  to  aphasia. 
(6)  Secondary  deterioration  in  connection  with  epilepsy, 
(c)  Terminal  deterioration  due  to  progressive  alterations  of  the 
primarily  injured  parts  with  or  without  arteriosclerosis. 

4.  Psychoses  in  which  trauma  is  merely  a  contributing  factor. 
(o)  General  paralysis  with  or  without  traumatic  stigmata. 

(6)  ]Manic-depressive  and  other  transitory  psychoses,  catatonic 
deterioration  and  paranoiac  conditions,  with  or  without  traumatic 
stigmata. 

5.  Traumatic  psychoses  from  injury  not  directly  affecting  the  head. 
This  group  of  traumatic  psychoses  includes  also  a  certain  number  of 

the  postoperative  psychoses,  psychoses  following  operations  upon  the 
eye  and  residence  in  a  dark  room,  the  so-called  ophthalmic  psychosis, 
the  psychoses  of  insolation  and  many  shell  shock  cases  seen  under  war 
conditions.  Of  course  this  statement  applies  in  general  only  to  such 
acute  psychoses  as  do  not  belong  in  other  groups,  as  the  shock  of 
surgical  operations,  etc.,  may  well  be  a  precipitating  factor  in  the  onset 
of  such  psychoses.  ^Yhen,  however,  they  are  essentially  psychoses 
the  result  of  shock  they  have  a  symptomatology  and  history  quite 
the  same  as  the  traumatic  psychoses.  They  can  be  understood  if  the 
h^-pothesis  of  Friedmann  is  borne  in  mind  that  the  complex  named 
after  him  is  due  to  vasomotor  disturbance.  If  this  be  true  it  can  be 
understood  how  severe  emotional  traumas,  as  well  as  physical  traumas 
may  produce  a  vasomotor  imbalance,  although,  of  course,  it  must 
never  be  forgotten  that  whatever  may  in  the  last  analysis  be  the  actual 
cause,  the  cause  operates  upon  a  certain  khid  of  individual  and  the 
sjinptom  picture  is  necessarily  modified  accordingly.  In  some  shell 
shock  cases  concussion  and  actual  destniction  of  tissue  takes  place. 

The  above  symptomatology  refers  to  those  traumatisms  that  are 
accidental.  Some  traumatisms,  however,  clearl>-  belong  in  the 
symptomatology  of  the  neuroses  and  psychoses.  The  injury  in  such 
cases  is  the  expression  of  an  unconscious  wish,  often  a  symbolic 
punishment  inflicted  for  sinful  acts,  the  outgrowth  of  which  has  been 
termed  a  castration  complex.  Some  patients  actually  castrate  them- 
selves, others  do  so  symbolically  by  putting  out  their  eyes,  others 
bring  about  apparent  accidents,  such  as  a  broken  leg,  with  the  same 
unconscious  wish  as  the  motive. 

Acute  Chorea  (Sydenham's).— Quite  usually  a  patient  with  acute 
chorea  is  irritable,  somewhat  emoti()nall>-  unstable,  fretful  and 
impatient,  a  state  of  mind  one  would  expect  from  the  nature  of  the 
illness.  While  this  condition  is  quite  usual  it  ma\'  become  somewhat 
more  aggravated,  the  restlessness  and  emotional  instability  be  more 


944       PSYCHOSES  ASSOCIATED  WITH  ORGANIC  DISEASES 

pronounced,  with  evidences  of  transitory  disturbances  in  the  sensor- 
ium,  and  perhaps  slight  apprehensiveness  and  suspiciousness.  The 
halhicinatory  disturbances  may  become  very  much  aggravated, 
together  with  clouding  of  consciousness  and  marked  delirium  in 
connection  with  the  febrile  movement.  Under  these  circumstances, 
of  course,  we  are  dealing  essentially  with  a  fever  delirium.  Along 
with  these  milder  manifestations  of  mental  disorder  one  occasionally 
finds  sjTnptoms  of  a  hysteriform  character. 

Chorea  insaniens  is  generally  considered  as  a  distinct  form  of  acute 
chorea.  It  is  associated  with  high  temperature  and  marked  mental 
disturbances,  usually  beginning  early  in  the  disease.  The  mental 
disorder  is  essentially  of  a  delirious  character  with  hallucinations 
and  clouding  of  consciousness.  The  hallucinations  are  quite  apt  to 
be  terrifying  and  the  patient  consequently  apprehensive  and  fearful, 
although  the  opposite  condition  of  elation  has  been  described.  The 
disease  is  quite  frequently  associated  with  pregnancy  and  is  not 
infrequently  fatal. 

Korsakow's  psychosis  is  sometimes  seen  in  patients  suffering  from 
acute  chorea  as  a  result  of  polyneuritis  resulting  from  overtreatment 
with  arsenic.     (See  section  on  Chorea.) 

Chronic  Chorea  (Huntington's). — It  has  always  been  recognized 
that  this  disease  was  associated  with  mental  symptoms,  and  it  has 
generally  been  considered  that  there  was  a  tendency  to  progressive 
deterioration,  ending  in  well  marked  dementia.  While  in  a  general 
way  this  may  be  true,  it  is  well  to  be  cautious  in  estimating  the  mental 
condition  of  chronic  choreics.  They  are  extremely  inaccessible  in 
many  instances,  especially  those  patients  whose  speech  apparatus  is 
affected  by  the  disorder,  and  it  requires  great  patience  to  make  a 
satisfactory  examination  of  their  mentality.  On  the  other  hand  the 
patients  themselves  find  extensive  explanations  so  difficult  to  com- 
municate that  they  are  content  with  the  shortest  possible  responses, 
so  that  they  frequently  mislead  the  examiner  into  the  belief  in  an 
intellectual  poverty  which  does  not  really  exist. 

With  these  warnings  it  may  be  conceded  that  in  general  the  chronic 
choreic  is  of  somewhat  enfeebled  mentality  with  a  tendency  to  emo- 
tional depression  and  to  a  mood  of  suspiciousness.  The  emotional 
depression  can  be  readily  understood,  as  the  patient  realizes  quite 
well  his  plight,  namely,  that  he  is  affected  with  a  chronic  disease 
which  practically  isolates  him  from  his  fellows.  Not  infrequently 
there  is  a  marked  emotional  instability  and  irritability  and  such 
choreic  patients  may  have  great  difficulty  in  getting  along  in  wards 
where  they  come  in  contact  with  other  patients.  Finally,  with  the 
increasing  deterioration  there  is  a  tendency  to  indifference,  to  emo- 
tional deterioration.  The  suspicious  mood,  which  so  many  of  these 
patients  have,  we  believe  has  at  least  in  part  its  explanation  in  the 
patient's  isolation  as  a  result  of  the  nature  of  the  disease.  We  find 
suspicious  moods  in  all  persons  who  are  cut  off  from  active  contact 
with  their  fellows,  more  especially  in  people  who  are  closely  confined, 


MULTIPLE  SCLEROSIS  945 

either  voluntarily  or  otherwise,  those  who  suffer  from  pronounced 
varieties  of  aphasia,  which  make  it  impossible  for  them  to  communicate 
their  thoughts  or  to  receive  communications  from  others  and  pre- 
dominantly among  the  deaf.  To  a  certain  extent,  too,  the  dementia 
is  probably  due  to  this  cutting  off  of  active  contact  with  life  by  impair- 
ing the  means  of  communication.  The  choreic  finds  it  extremely 
difficult  to  talk  to  others.  He  may  find  it  equally  difficult  to  read, 
perhaps  because  of  defect  of  vision  which  is  liable  to  be  present,  as 
it  must  be  remembered  that  this  is  a  disease  of  later  life,  and  in  various 
ways  he  loses  touch  with  what  is  going  on  about  him.  Finally,  when 
he  has  long  passed  any  ability  to  pick  up  the  thread  of  events  he 
becomes  indifferent,  and  when  it  is  realized  that  he  is  frequently  in 
the  arteriosclerotic  period,  often  approaching  the  senium,  it  can  be 
seen  that  this  removal  from  acute  contact  with  reality  tends  to  produce 
inactivity,  as  it  were,  and  the  appearances  of  dementia,  if  not  actual 
dementia,  and  so  the  chronic  choreic  shows  emotional  defects,  atten- 
tion disorder,  lack  of  impressibility,  defect  .of  recall  and  in  other  ways 
demonstrates  that  he  has  lost  interest,  as  it  were,  in  life. 

A  certain  few  cases  show  distinctly  more  pronounced  psychotic 
sjTnptoms,  occasionally  showing  well  marked  persecutory  ideas.  (See 
Chapter  X.) 

Paralysis  Agitans. — It  is  probable  that  the  majority  of  cases  of 
paralysis  agitans  show  a  certain  amount  of  mental  disturbance, 
although  in  a  great  number  of  cases  this  disturbance  is  so  slight, 
compared  with  the  ph}'sical,  and  so  easily  seen  to  be  dependent  upon 
it  that  it  is  quite  overlooked.  This  simple  disturbance  is  in  the 
emotional  sphere  and  for  the  most  part  is  one  of  slight  depression 
but  rarely  one  of  euphoria.  Occasionally  delirioid  episodes  supervene, 
with  marked  confusion,  but  in  general  consciousness  is  clear  and  the 
patient  remains  well  oriented,  except,  of  course,  in  the  terminal  con- 
dition when  arteriosclerotic  changes  and  the  deterioration  of  the 
senium  have  set  in.  Occasionally  there  is  a  well  marked  psychosis, 
in  which  instance  it  is  most  apt  to  be  of  a  depressive  hypochondriacal 
character,  often  with  paranoid  coloring.  The  depression  may  be  suffi- 
ciently great  to  result  in  efforts  at  suicide.  In  these  cases  disorders 
of  the  sensorium  may  also  be  present. 

As  intimated  above  it  must  be  remembered  that  Ave  are  dealing 
with  a  disease  of  later  life  and  that  not  infrequently  arteriosclerotic 
and  senile  changes  show^  themselves  by  changes  in  the  mental  sphere. 

Multiple  Sclerosis. — ^The  outward  manifestations  of  the  mental 
disorder  associated  with  this  disease  are  mainly  in  the  emotional 
sphere.  It  has  been  stated  both  that  the  majority  of  patients  were 
depressed  and  that  the  majority  were  exalted.  Both  these  state- 
ments are  based  upon  the  obser\ation  of  very  few  patients.  Although 
exaltation  and  depression  may  be  manifest  and  there  may  also  be 
spasmodic  laughing  and  crying,  due  probably  to  lesions  of  the  basal 
ganglia,  which  are  unaccompanied  by  emotion,  there  is  a  certain 
amount  of  deterioration  which  is  progressive  and  which  depends,  of 
60 


946       PSYCHOSES  ASSOCIATED   WITH  ORGANIC  DISEASES 

course,  upon  the  destructive  changes.  When  the  distribution  of  the 
sclerotic  patches  is  quite  similar  to  the  distribution  of  the  lesions  in 
general  paresis  the  outward  symptoms  of  the  two  conditions  may  be 
very  much  alike  and  lead  to  confusion  in  diagnosis.  The  diagnostic 
problems,  however,  can  be  cleared  up  by  the  laboratory  findings. 

Polyneuritis. — See  Korsakow's  Psychosis. 

Heart  Disease. — Depressive  states  are  most  in  evidence  here. 
Mental  symptoms,  however,  are  most  apt  to  be  associated  with 
failure  of  compensation.  With  precordial  distress  goes  typically  a 
mental  state  of  anxiousness.  Transitory  confusions  with  dreamy 
hallucinations  occur  with  compensation  disturbances  and  edema. 

Various  other  diseases  have  from  time  to  time  mental  symptoms 
associated  with  them.  The  great  majority  of  such  diseases,  if  not  all 
of  them,  have  elements  of  infection,  or  toxemia  and  exhaustion  com- 
bined, with  all  or  part  of  them.  The  mental  symptom-complex  of 
confusion  arises  most  tj'pically.  In  some  cases,  especially  the  less 
acute,  paranoid  conditions  occur  and  hallucinosis  is  of  occasional 
occurrence. 

Head  has  shown  that  certain  visceral  diseases,  especially  of  cardio- 
vascular and  pulmonary  origin,  often  have  associated  mental  symp- 
toms, although  they  may  not  appear  except  on  the  most  careful  exami- 
nation. The  symptoms  found  are:  (1)  Hallucinations  of  vision, 
hearing  and  smell;  (2)  moods,  either  of  depression  or  exaltation  and 
(3)  suspicions  usually  occurring  when  a  depression  has  persisted  for 
some  time. 

These  conditions  take  their  origin  in  part  as  a  result  of  reflected 
visceral  pains.  Each  spinal  segment  has  both  a  visceral  and  a  cutan- 
eous representation.  Disease  occurring  in  the  visceral  area  is  referred 
to  the  cutaneous  surface  supplied  by  the  same  segment.  The  cuta- 
neous distribution  of  the  fifth  nerve  corresponds  to  the  visceral  dis- 
tribution of  the  vagus,  so  pain  occurring  in  the  vagus  territory  will 
be  referred  to  the  scalp  and  thus  occur  points  of  tenderness  in  this 
region  with  which  the  hallucinations  are  associated.  The  mood  of 
exaltation  is  essentially  transitory  and  arises  as  a  contrast  phenomenon 
of  the  depression  and  as  a  result  of  the  disappearance  or  lessening  of 
the  reflected  somatic  pain. 

Still  more  light  may  be  thrown  upon  the  psychoses  associated  with 
these  various  chronic  diseases  when  the  unconscious  factors  are  better 
known.  A  disease  like  paralysis  agitans  or  chronic  chorea  for  example 
seems  to  be  due  to  a  wearing  out  of  certain  mechanisms,  a,  so  to  speak, 
localized  senility  which  may  be  conceived  as  due  to  having  too  constant 
and  too  severe  demands  made  upon  these  mechanisms  over  a  long 
period  of  time.  It  may  be  that  here  we  see  a  chronic  tension  of  a 
given  mechanism,  a  chronic  state  of  preparedness,  as  it  were,  for  a 
form  of  expression  which  the  repressions  do  not  permit  to  come  to 
pass  and  so  the  machinery  is  worn  out  in  futile  efforts.  A  deeper 
study  of  the  unconscious  factors  is  needed  in  all  these  conditions. 


CHAPTER   XXIII. 

PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC 
PSYCHOSES. 

The  grouping  of  the  presenile,  senile  and  arteriosclerotic  psychoses 
together  in  one  chapter  is  a  matter  not  only  of  convenience,  for 
obviously  one  Avould  expect  all  sorts  of  admixtures,  particularly  as 
between  the  senile  and  arteriosclerotic  groups,  but  there  are  many 
pictures  here  which  are  not  distinctive,  especially  in  the  presenile 
period,  which  Kraepelin  begins  his  discussion  of  by  saying  it  is  the 
darkest  region  of  all  psychiatry  today. 

The  Presenile  Psychoses. — In  the  presenile  period  there  are  a 
number  of  psychotic  pictures  the  exact  significance  and  nosological 
placement  of  which  is  not  at  all  understood.  There  is  no  doubt, 
however,  that  in  this  period  depressions  are  much  more  in  evidence 
than  excitements,  the  depressions  seeming  to  mount  up  in  frequency 
during  the  period  of  involution.  Hiibner  found,  for  example,  21  cases 
of  single  attacks  of  melancholia  after  the  fiftieth  year,  but  onl}'  2  cases 
of  single  attacks  of  excitement. 

Involution  Melancholia. — Sympfoms. — The  Kraepelin  school  for  a 
considerable  time  considered  that  certain  depressions  of  later  life, 
characterized  by  an  anxious  apprehensive  agitation,  with  profound 
emotional  depression,  composed  a  nosological  unit  to  which  was  given 
the  name  involution  melancholia,  or  more  briefly  melancholia,  the 
term  melancholia  being  limited  in  its  application  to  this  particular 
tj'pe  of  depression. 

The  symptoms  of  this  psychosis  are  generally  preceded  by  a  con- 
siderable period  during  which  there  are  vague  head  symptoms,  such 
as  pressure,  pain,  vertigo,  together  with  anorexia,  irritability,  insomnia, 
mental  insufficiency,  and  a  mild  neurastheniform  state  with  perhaps 
some  emaciation.  This  condition  becomes  progressively  worse  and 
the  patients  develop  an  agitated  depression,  with  anxiety,  apprehen- 
sion, fear  of  impending  danger  with  quite  usually  delusions  of  sin. 
The  depression  is^  characteristically  very  profound  and  the  motor 
agitation  may  be  quite  considerable.  The  patient  may  go  about 
wringing  his  hands,  moaning  and  groaning,  perhaps  repeating  over 
and  over  again  such  phrases  as  "Oh,  my  God!  Oh,  my  God!"  "It  is 
a  fearful  thing.  Good  Lord  help  me!"  One  patient  constantly  repeats 
"Doctor,  will  I  be  done  away  with  tonight?"  and  "Then  will  I  be 
here  tonight  just  the  same  as  last  night,  and  will  I  be  here  tomorrow 
just  the  same  as  today?"  Another  patient  believes  herself  very  sinful, 
refuses  to  eat  because  the  food  should  be  used  for  others,  walks  aJDOut 
in  an  agitated  manner  picking  her  fingers  and  attempted  suicide 
because  she  was  afraid  she  was  to  be  put  to  death. 

(947) 


948     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

Even  in  these  cases  of  quite  extreme  agitation  and  profound  depres- 
sion consciousness  remains  unclouded,  orientation  is  little  if  at  all 
impaired,  and  the  form  of  thought  is  well  maintained. 

Another  t;s^e  of  case  presents  symptoms  of  retardation.  Many 
of  these  cases  may  be  so  retarded  as  to  be  almost  stuporous  and  some 
of  them  present  an  amount  of  resistance  which  reminds  one  of  the 
characteristic  negativism  of  dementia  precox.  This  latter  group  was 
originally  described  by  Kraepelin  as  "late  catatonia."  Its  nosological 
status  is  at  present  in  doubt. 

Certain  cases  of  anxious  depression  may  be  highly  agitated  with 
Insomnia,  refusal  of  food,  rapid  emaciation,  clouding  of  consciousness, 
hallucinations,  self-inflicted  injuries,  attempts  at  suicide,  a  rapid 
course  ending  in  death. 

In  the  marked  cases  of  apprehensive  and  agitated  depression  there 
is  frequently  a  considerable  degree  of  precordial  distress  with  perhaps 
tachycardia  and  often  a  sense  of  oppression  over  the  chest  with  a 
feeling  of  difficulty  of  breathing.  These  symptoms  are  apt  to  appear 
in  attacks,  at  which  times  the  depression  is  more  pronounced. 


Fig.  447. — Facies  of  involution  melancholia. 


The  danger  from  suicide  is  very  great  in  this  psychosis,  particularly 
because  the  patient  is  not  so  frequently  rendered  inactive  by  marked 
retardation. 

The  delusional  content  of  consciousness  varies  widely,  but  hypo- 
chondriacal delusions,  nihilistic  delusions  and  the  feeling  of  unreality 
are  common.  Occasionally  the  delusions  take  on  bizarre,  absurd  and 
fantastic  forms,  and  there  may  be  delusions  of  grandeur  late  in  the 
course  of  the  disease.  There  may  be  a  strange  mixture  of  depressive 
and  grandiose  ideas,  as  in  the  patient  of  Weygandt's,  who  believed 
she  was  going  to  be  boiled  in  a  silver  kettle. 

The  statistics  of  this  disease  show  that  about  40  per  cent,  got  well, 
the  remaining  60  per  cent,  terminating  in  various  ways;  some  by 
suicide,  some  by  death  from  intercurrent  disease,  which  they  were 
ill  adapted  to  withstand;  some  by  death  from  general  marasmus  or 
the  development  of  tuberculosis;  some  lapsed  into  chronicity;  and 
finally,  a  few  improved  sufficiently  to  leave  the  hospital  and  get  along 


THE  PRESENILE  PSYCHOSES  949 

at  home,  though  still  somewhat  depressed.  A  certain  number  of  this 
class  may  get  worse  under  home  surroundings  and  have  to  be  returned 
to  the  hospital. 

This  group  of  involution  melancholia,  so-called,  was  studied  with 
great  care  by  Dreyfus.^  He  studied  the  life  histories  of  81  cases,  34 
of  which  were  personally  investigated.  Eight  were  not  personally 
investigated  and  39  were  deceased.  As  a  result  of  his  study  he  con- 
cluded that  with  the  exception  of  2  cases  undiagnosed,  2  cases  in  which 
a  mistake  in  diagnosis  had  been  made,  and  possibly  2  more  doubtful 
cases,  all  w'ere  cases  of  manic-depressive  psychosis.  This  conclusion 
was  reached  by  finding  the  fundamental  symptoms  of  this  disease 
present.  Of  these  cases  66  per  cent,  were  recovered  or  were  recovering 
at  the  time  of  death,  8  per  cent,  developed  arteriosclerosis,  25  per 
cent,  died  unrecovered  of  intercurrent  disease  or  suicide. 

The  conclusion  reached  by  Dreyfus  that  the  involution  melancholia 
of  Kraepelin  is  really  a  form  of  manic-depressive  psychosis  has  now 
been  accepted  by  Kraepelin  himself.  A  study  of  this  group  of  cases 
shows  quite  characteristically  the  presence  in  the  history  of  periods 
of  affect  fluctuation;  and  quite  usually  periods  of  slight  depression 
which  were  not  severe  enough  to  attract  attention  seriously  to  the 
mental -condition,  or  perhaps  were  not  at  all  understood  at  the  time, 
being  accounted  for  in  various  other  ways. 

Even  admitting  that  this  group  of  involution  melancholias  really 
belongs  to  the  manic-depressive  psychosis,  still  the  problem  is  not 
wholly  solved  and  it  must  be  admitted  that  in  all  probability  the 
involution  period  has  certain  modifying  effects  upon  the  psychosis. 

In  the  first  place  the  depressions  as  noted  heretofore  are  very  much 
more  frequent  than  the  excitements  and  they  are  very  much  longer  in 
duration  than  during  earlier  life.  This  of  course  can  be  easily  under- 
stood by  the  failing  resistance  of  the  involution  period.  A  psychosis 
that  has  manifested  itself  only  by  mild  and  hardly  appreciable  evi- 
dences throughout  the  youth  of  the  individual  may  well  get  a  firm  hold 
when  the  powers  of  efficient  reaction  are  failing. 

With  the  recent  work  that  has  been  done  on  the  ductless  glands 
and  with  the  somewhat  characteristic  mental  pictures  that  go  along 
with  disturbances  of  the  internal  secretions  one  must  bear  in  mind 
that  perhaps  many  of  the  changes  of  the  involution  period  are,  in 
part  at  least,  determined  by  changes  in  these  glands,  particularly  as 
the  result  of  atrophy  of  the  uterus,  the  ovaries,  the  prostate,  the 
testicles  and  the  adrenals,  with  the  possible  result  of  an  imbalance 
being  brought  about  in  the  relationship  between  them.  This,  of 
course,  if  it  is  so,  would  only  be  one  of  the  modifying  factors  of  the 
involution  period  which  one  might  expect  to  see  reflected  in  the 
diseases  at  this  time  of  life. 

The  following  case  illustrates  this  type  of  psychosis:  The  ])atient, 
a  woman,  was  admitted  to  the  hospital  at  the  age  of  fifty.     She  was 

•  Die  Melanoholie,  ein  Zustandsbild  des  manisch-depressiven  Irreseins,  Jena,  Gustav 
Fischer,  1907. 


950     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

very  much  depressed,  said  that  she  was  very  unworthy  and  should 
be  hung,  that  she  had  committed  the  unpardonable  sin  and  would 
burn  forever  in  the  flames  of  Hell.  A  little  later,  in  addition  to  these 
ideas  that  she  had  committed  the  unpardonable  sin  and  that  her 
soul  was  lost,  she  said  that  she  was  dead,  what  existed  of  her  now  was 
her  spirit,  that  her  body  had  passed  away,  and  because  she  was  dead 
it  was  useless  to  attempt  suicide.  This  condition  had  its  origin  some 
two  years  before,  following  an  operation,  when  she  had  cleared  up, 
and  as  a  result,  so  stated,  from  overwork,  had  broken  down  again  and 
developed  these  ideas  and  subsequently  was  admitted  to  the  hospital 
after  having  made  a  suicidal  attempt.  In  addition  to  the  delusions 
she  had  visual  hallucinations,  saw  spirits  and  heard  them  talking  to  her. 

This  patient  gave  the  general  impression  on  her  admission  to  the 
hospital  of  a  patient  suffering  from  involution  melancholia.  A  further 
inquiry,  however,  developed  the  history  of  numerous  previous  attacks 
of  depression,  which  suggested  a  manic-depressive  reaction  type. 
Just  before  her  discharge  from  the  hospital,  however,  when  she  had 
become  much  more  accessible,  we  learned  that  in  her  early  life,  before 
she  was  twenty,  she  had  had  a  love  aft'air.  This  love  affair  had  been 
a  very  profound  emotional  experience,  particularly  because  she  had 
found  it  necessary  to  give  up  the  young  man  because  of  his  drinking 
habits.  That  this  was  not  willingly  done,  however,  was  shown  by  her 
subsequent  life,  which  was  taken  up  by  constant  activities,  largely  of 
a  social  nature  and  apparently  for  the  purpose  of  side-tracking  her 
disappointment.  She  was  an  attractive  young  woman  and  had  many 
admirers,  but  did  not  permit  herself  to  become  attached  to  any  one 
of  them.  She  led  a  very  active  life  and  was  able  to  sublimate  the 
energy  of  her  suppressed  emotions  until  the  period  of  the  menopause, 
when  she  broke  down  with  self-accusatory  delusions  and  suicidal 
tendencies.  She  believed  she  had  committed  the  unpardonable  sin; 
this  sin  was  having  committed  adultery  in  her  mind  with  the  young 
man  with  whom  she  had  been  in  love  in  her  youth.  The  psychosis 
lasted  somewhat  over  five  years,  at  the  end  of  which  she  finally  suc- 
ceeded in  reaching  a  compromise  by  developing  tendencies  diametri- 
cally opposed  to  her  delusional  system.  She  became  talkative, 
humorous  and  particularly  facetious,  and  just  before  her  discharge 
she  made  the  remark  that  she  was  born  dead,  but  made  it  with  a  smile, 
and  with  the  added  comment  that  she  had  gotten  along  pretty  well 
all  her  life  for  a  dead  person.  This  condition  of  relative  equilibrium 
enabled  her  to  go  back  to  her  family  and  in  the  absence  of  disturbing 
factors  she  will  probably  get  along  all  right,  at  least  for  a  considerable 
time.  Nothing  has  been  heard  of  her  for  some  years.  The  compen- 
sation,^the  balance  struck,  was  a  fairly  efficient  one. 

Such  a  case  as  this  illustrates  a  common  type  of  depression  which 
occurs  in  the  involution  period.  It  is  a  type  which  if  dealt  with 
solely  from  the  descriptive  standpoint  would  have  been  considered 
an  involution  melancholia,  while  a  little  more  careful  history  indicates 
that  it  is  a  manic-depressive  reaction  type,  while  a  still  more  profound 


THE  PRESENILE  PSYCHOSES  951 

probing  into  the  facts  of  the  life  make  the  whole  thing  quite  under- 
standable in  psychological  terms.  It  is  manifestly  a  psychogenic 
depression,  but  whether  it  should  be  called  manic-depressive  or  not 
is  a  question.  Xow  that  psychoanalytic  methods  are  showing  the 
presence  of  endopsychic  conflicts  in  the  manic-depressive  psychosis, 
such  a  case  as  this  shows  the  close  connection  between  a  pure  psy- 
chogenic psychosis  and  the  manic-depressive  reaction  t\-pe,  and  the 
further  relation  of  these  conditions  to  the  depressions  occurring  during 
the  involution  period. 

Pathology. — ^An  increased  neuroglia  formation  in  the  deeper  layers 
of  the  cortex  has  been  described,  and  in  this  disease  we  find  most 
often  a  termination  in  the  condition  described  by  Meyer  as  central 
neuritis.  This  is  essentially  a  parenchymatous  degeneration  with 
swelling  of  the  cell  body,  a  disappearance  of  the  chromophile  sub- 
stance and  eccentricity  of  the  nucleus.  In  rapidly  fatal  cases  there 
are  evidences  of  extensive  destructive  changes  in  the  cortex.  There 
are  also  found  a  certain  amount  of  disintegration  products. 

Treatment. — One  of  the  very  important  considerations  in  this  group 
of  depressions,  as  shown  by  the  sjinptomatology,  is  the  prevention 
of  suicide.  It  is  therefore  very  much  more  frequently  necessary 
to  intern  these  patients  so  that  they  can  be  properly  watched. 
Insomnia,  agitation,  failure  of  nutrition  with  suicidal  tendencies  are 
the  conditions  that  have  to  be  met.  For  the  agitation  and  the  insomnia 
hydrotherapy  is  the  best  means  of  treatment.  As  few  drugs  as  possible 
should  be  used.  For  the  suicidal  tendencies  constant  watchfulness, 
which  should  have  as  its  ideal  an  unobstrusiveness  which  does  not 
suggest  the  suicidal  idea  constantly  to  the  patient's  mind.  Failing 
nutrition  must  be  met  by  tube-feeding  if  necessary. 

An  analysis  of  the  patient's  mental  symptoms  with  the  view  of 
understanding  them  is  of  course  highlj^  desirable,  but  this  class  of 
patients  do  not  lend  themselves  easily  to  analytic  technic,  at  least 
until  they  have  begun  to  quiet  down,  oftentimes  not  until  they  are 
convalescent. 

Other  Psychoses  of  this  Period. — Various  other  types  of  ^psychoses 
have  been  described  as  belonging  in  this  period.  T^-pes  of  depressive 
Wahnsinn,  some  of  them  associated  with  anxiety  and  proceeding  to 
deterioration,  while  Kraepelin  has  described  a  very  small  class  of 
so-called  presenile  delusional  psychoses,  aft'ecting  especially  women, 
in  which  delusions  of  infidelity  are  usually  prominent,  together  with 
hallucinations.  The  delusions  are  variable  and  do  not  seem  to  at 
all  logically  control  the  conduct.  Farrar  has  described  three  t^^)es 
of  psychoses  belonging  to  this  period:  melancholia  vera,  anxietas 
prcBsenilis  and  depressio  apathetica. 

In  melancholia  vera  there  is  in  the  main  an  autopsychosis.  The 
delusions  are  auto-accusatory,  with  ideas  of  sin  but  with  clear  con- 
sciousness; there  is  no  defect  of  orientation.  The  patient  believes  his 
soul  is  lost,  that  he  is  to  suffer  eternal  torment  hereafter,  and  about 
these  beliefs  there  is  no  doubt,  but  on  the  contrary  a  marked  "sub- 


952      PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC    PSYCHOSES 

jective  certainty."  There  may  be  some  slight  tendency  to  somato- 
psychic dehisions,  insight  is  defective  and  sHght  anxiety  may  be 
present. 

In  anxietas  proBsenilis  there  is,  on  the  contrary,  in  the  main  an 
allopsychosis.  There  is  a  very  marked  "subjective  uncertai7ity"  which 
gives  an  unreal  tinge  to  the  outer  world  and  out  of  which  grows  the 
fear  of  things  unkno\\Ti,  culminating  in  the  marked  anxiety  which  is 
characteristic  of  this  form  of  the  psychosis.  Remorse  or  dread  of  the 
future  are  not  elements  in  the  depression;  on  the  contrary,  it  is  the 
great  unknoTMi  and  overwhelming  present  that  seems  about  to  destroy 
them.  These  cases  occur  later  in  life  than  the  former,  show  more 
evidences  of  senile  decay,  such  as  arteriosclerosis  and  present  such 
symptoms  as  verbigeration,  rhythmical  movements,  suggestibility; 
the  prognosis  is  less  favorable. 

In  depressio  ajxithetica  there  is  simply  a  let-down,  a  stopping  on  the 
part  of  one  who  has  been  leading  an  active  life.  Interest  abates,  the 
struggle  is  drawn  away  from  and  we  have  a  picture  of  mild  depression 
with  clear  consciousness  and  no  disturbance  of  orientation.  There  is 
some  "subjective  uncertainty"  delusions  and  sensory  fabrications 
play  little  part.  The  symptoms  are  negative  rather  than  positive; 
the  prognosis  is  relatively  good. 

Finally  one  must  remember  that  a  true  anxiety  neurosis  may  develop, 
and  it  not  infrequently  does,  at  this  period  of  life. 

In  conclusion  then  it  will  be  seen  that  we  have  during  this  period 
of  life  a  group  of  psychoses  of  widely  different  forms,  although  tending 
toward  a  certain  amount  of  uniformity  in  their  outward  expression 
in  that  depressions  are  so  much  more  frequent  at  this  period.  In  the 
first  place  there  are  frank  attacks  of  manic-depressive  psychosis  with 
here  as  elsewhere  marked  difi'erences,  depending  upon  the  severity 
of  the  constitutional  taint  upon  the  one  hand,  or  its  absence  and 
the  presence  of  a  reactive  t;yT)e  upon  the  other.  Apprehension  and 
anxiety  appear  frequently  at  this  period  of  life,  and  a  true  anxiety 
psychosis  is  not  uncommon.  Paranoid  conditions  occasionally  occur 
and  disorders  of  the  sensorium  are  not  infrequent.  In  a  certain  group 
of  cases  negativistic  tendencies  are  sufficiently  marked  to  make  the 
outward  semblance  to  catatonia  quite  pronounced. 

Certain  of  the  psychoses  of  this  period  tend  toward  more  or  less 
physical  changes.  In  other  words,  the  physical  side  of  the  disease 
has  to  be  kept  constantly  in  mind  and  must  be  considered  to  be  the 
most  important  prognostic  feature.  The  changes  incident  to  vascular 
degeneration,  while  of  course  not  usually  prominent  in  the  fifth  and 
sixth  decades,  may  well  be  in  evidence,  while  the  changes  in  the  duct- 
less glands  are  to  be  borne  in  mind.  Other  changes  undoubtedly  occur, 
but  their  nature  and  their  bearing  upon  the  psychosis  are  not  at  all 
understood.  The  psychosis  must,  nevertheless,  be  expressed  in  psy- 
chological terms  and  the  explanation  of  the  mental  symptoms  must 
be  sought  by  psychoanalytic  study.  We  have  here,  then,  diseases 
which  have  a  marked  phj'sical  side  apart  from  the  mental  manifesta- 


THE  SENILE  PSYCHOSES  953 

tions,  the  physical  side  being  more  prominent  than  in  dementia  precox 
and  less  prominent  than  in  general  paresis. 

The  Senile  Psychoses.— The  normal  course  of  life  leads  to  a  certain 
amount  of  gradual  mental  and  physical  deterioration  during  the  later 
years.  Whether  this  occurs  or  not  probably  depends  upon  mam- 
factors,  for  we  see  some  men  at  sixty  as  old  as  they  should  be  at 
seventy-five  and  other  people  at  eighty  presenting  a  wonderful  degree 
of  elasticity  and  enthusiasm  without  any  apparent  falling  off  in  mental 
powers  or  interests.  This  variation  was  wont  to  be  expressed  by 
saying  that  "a  man  is  as  old  as  his  arteries,"  but  it  is  certain  that 
there  are  many  other  factors  besides  the  condition  of  the  bloodvessels 
that  lead  to  senile  deterioration.  In  fact  senile  deterioration  may 
take  place  and  lead  to  very  profound  dementia  without  material 
disease  of  the  bloodvessels  at  all. 

Intermediate  Conditions. — A  certain  nimiber  of  the  involution  psy- 
choses continue  over  into  the  senile  period  when  the  patients  undergo 
the  mental  and  physical  changes  of  senile  involution.  This  is  in  part, 
at  least,  the  reason  for  the  termination  in  dementia  of  a  certain  group 
of  involution  cases,  aside  from  the  added  obvious  fact  that  vascular 
degeneration  is  also  an  important  etiological  moment.  The  cases 
that  show  this  outcome  in  dementia  are  more  especially  the  paranoid 
types.  This  group  of  cases  shows,  therefore,  that  there  is  a  gradual 
transition  from  the  psychoses  of  the  distinctly  involution  period  to 
those  of  the  senile  period,  a  perfectly  understandable  condition  if  we 
conceive  of  the  psychotic  manifestation  as  being  expressions  of  mental 
conflicts  that  are  at  the  basis  of  the  individual  character  and  which 
'express  themselves  in  the  later  years  of  life  when  efficient  reaction  is 
becoming  progressively  less  possible,  and  which  later  on  become  fixed, 
chronic  and  disintegrating  at  a  time  when  the  physical  changes  of  the 
senium  cooperate  to  this  end. 

Normal  Senile  Involution. — The  more  usual  symptoms  of  senile 
involution  occurring  after  sixty  are  in  the  main  a  loss  of  memory  for 
recent  events,  due  to  lack  of  impressibility  to  the  extent  even  that 
events  of  only  an  hour  before  are  completely  forgotten,  lack  of  ability 
to  recognize  faces,  marked  egotism,  so  that  others'  wants  and  comforts 
are  not  considered,  which  may  be  associated  with  some  irritability 
on  interference.  There  is  developed  more  and  more  as  the  years  go 
on  a  true  misoneism,  so  that  the  patient  will  positively  not  tolerate 
any  change  in  the  usual  order  of  things,  everything  must  be  done  the 
same  from  day  to  day,  the  same  seat  is  preempted,  a  particular  kind 
of  food  demanded  and  the  like  with  other  comforts.  With  this  mis- 
oneism and  the  lack  of  memory  for  recent  events  goes  a  marked 
tendency  to  reminiscence.  The  events  of  youth  and  the  years  long 
past,  unlike  those  of  recent  occurrence,  are  viA'idly  recalled  and  the 
patient  thus  really  lives  in  a  world  of  former  days,  constantly  recalling 
and  reiterating  things  that  occurred  long  ago.  This  condition  becomes 
progressively  worse,  the  patient  leading  a  \egetative  existence  almost 
wholly,  no  mental  initiative,  failure  of  judgment  and  a  progressive 


054     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

loss  of  comprehension  of  the  environment,  so  that  there  is  no  adequate 
grasp  of  the  present  at  all. 

With  this  mental  failure  goes  a  corresponding  change  on  the  physical 
side.  The  signs  of  age  are  evident  in  the  wasted  muscles,  the  wrinkled, 
inelastic  skin,  gray  hair,  the  raucous  voice,  arcus  senilis,  senile  cuta- 
neous affections  and  signs  of  arteriosclerosis  in  the  superficial  arteries. 
In  this  condition  it  should  be  remembered  that  the  condition  of  the 
palpable  arteries  may  not  indicate  at  all  the  condition  of  the  cerebral 
vessels.  The  superficial  vessels  may  show  marked  arteriosclerotic 
changes,  while  the  cerebral  vessels  are  in  relatively  good  condition, 
or,  on  the  contrary,  the  cerebral  vessels  may  be  seriously  affected  in 
a  person  whose  radials  are  comparatively  soft  and  whose  temporals 
are  not  noticeably  tortuous. 

Upon  this  background  of  dementia  there  may  appear  the  usual 
pictures — excitements,  depressions,  paranoid  states,  stuporous  states 
and  confusions.  All  of  these  conditions,  however,  must  be  recon- 
structed in  psychological  terms  to  receive  any  explanation  at  all,  and 
it  is  not  difficult  oftentimes  to  make  out  some  logical  reason  for  the 
particular  type  of  delusional  formation. 

In  the  senile  dement  and  in  the  delusions  of  this  period  there  is 
noticed  an  apparent  indifference  in  the  emotional  sphere,  an  emotional 
poverty.  Persecutory  delusions,  delusions  of  infidelity,  hypochon- 
driacal delusions  take  on  grotesque  forms  and  their  expression  is 
not  accompanied  by  an  adequate  affect.  A  patient  tells  of  severe 
injuries,  of  having  been  shot,  while  perhaps  smiling;  another  patient 
tells  a  long  and  pitiful  story  about  her  affairs  and  about  a  claim  she 
has  against  the  government,  but  tells  it  in  a  stereotyped,  matter-of- 
fact  way  without  the  expression  of  any  feeling.  This  condition  is 
usually  described  as  one  of  emotional  blunting,  emotional  deteriora- 
tion, Bleuler,^  however,  lays  ^reat  stress  upon  what  he  believes  to 
be  the  fact,  namely,  that  there  is  no  lack  of  capacity  for  feeling,  but 
that  the  afPectivity  is  only  disturbed  secondarily,  that  patients  with 
organic  brain  disease  fail  to  get  a  sufficiently  clear  idea  and  therefore 
do  not  react  adequately  in  the  emotional  sphere.  Wherever  it  is 
possible  to  get  a  sufficient  comprehension  of  the  situation  the  emotion 
of  the  proper  quality  is  manifested  and  with  commensurate  intensity. 

Simple  Senile  Deterioration.- — This  condition  of  gradually  progressing 
dementia  without  marked  psychotic  distiu-bances  is  designated  as 
simple  senile  deterioration. 

The  senile  dement  is  apt  to  be  restless  and  suffer  from  insomnia, 
reversing  the  time  of  day  and  sleeping  perhaps  in  the  daytime  and 
lying  awake  at  night,  perhaps  wandering  about  the  house  at  night 
in  a  more  or  less  disoriented  condition.  This  tendency  to  disorien- 
tation, without  the  development  of  delusions  occiu's  in  the  course  of 
the  progressive  deterioration,  and  it  is  where  disturbances  of  the 
sensorium  are  marked  and  confusion  becomes  very  much  more  in 

'  Affecti\4ty,  Suggestibility,  Paranoia.  Translated  by  Chas.  Ricksher,  New  York  State 
Hospital  Bulletin,  February  15,  1912. 


THE  SENILE  PSYCHOSES  955 

evidence  that  we  have  the  condition  of  senile  delirium.  This  con- 
fusion, however,  may  be  only  transitory  and  is  quite  characteristic- 
ally in  evidence  in  the  intervals  between  waking  and  sleeping. 

Senile  Delirium. — This  is  merely  a  form  of  senile  deterioration  in 
which  confusion  dominates  the  picture  and  in  which  usually  disorders 
of  the  sensorium  are  also  markedly  in  evidence.  Patients  are  highly 
disoriented,  they  do  not  know  where  they  are,  or  what  time  of  day 
it  is,  do  not  know  whether  they  have  just  had  their  dinner,  or  whether 
it  is  time  to  go  to  bed  or  get  up,  are  frequently  quite  active  and 
irritable  and  are  apt  to  die  from  exhaustion.  The  delirium  may  take 
the  form  of  an  occupation  delirium. 

Presbyophrenia." — A  certain  proportion  of  these  patients  resemble 
very  closely  the  Korsakow's  psychosis,  being  disoriented  as  to  time 
and  place  and  supplying  memory  defects  by  fabrications.  One  old 
man,  for  example,  who  was  so  feeble  he  could  hardly  stand,  relates 
that  he  had  been  working  for  a  man,  making  some  sort  of  a  wire 
affair  for  the  past  seven  months. 

Course.^The  course  of  senile  dementia  in  its  various  forms  is  a 
progressive  one.  The  patients  tend  to  become  profoundly  demented, 
wholly  disoriented  and  die  naturally  of  marasmus.  Where  the  change 
is  somewhat  more  acute,  delirious  or  confusional  episodes  may  be  in 
evidence  from  time  to  time,  and  of  coiu-se  if  the  excitement  is  at  all 
prolonged  exhaustion  is  the  result.  The  patients  are  naturally  sus- 
ceptible to  intercurrent  affections,  particularly  pneumonia,  nephritis 
and  cystitis  and  many  of  them  die  in  delirium  the  result  of  a  terminal 
infection. 

Diagnosis. — Paranoid  conditions  may  resemble  paranoid  states  of 
earlier  life,  but  occiuring  in  the  senium,  usually  show  evidences  of 
organic  brain  disease  and  marked  mental  deterioration. 

Some  of  the  patients  also  show  similarities  to  dementia  precox, 
especially  those  that  develop  negativistic  reactions.  Just  what  rela- 
tion these  cases  have  to  the  precox  of  earlier  life  is  not  fully  known. 
The  marked  mental  dilapidation  and  the  period  of  life  at  which  they 
develop  usually  give  the  clue  to  the  diagnosis. 

A  few  cases  sho\v  a  mixed  symptomatology,  presenting  the  picture 
of  senile  deterio.ation  on  the  one  hand,  together  with  the  various 
symptoms  of  arteriosclerotic  disease  with  focal  lesions  on  the  other 
hand.  Here  are  found  all  sorts  of  mixtm-es  of  pupillary  disturbances, 
disturbances  of  speech  and  various  forms  of  paralysis. 

Certain  borderland  conditions  show  marked  emotional  states  of 
either  depression  or  excitement  which  dominate  the  picture  for  the 
time  being.  These  cases  show  beneath  the  emotional  state  the 
presence  of  a  defe:t  which  shows  the  basal  disorder. 

The  similarity  between  presbyophrenia  and  Korsakow's  disease  is 
very  considerable,  \ouet'  has  made  a  careful  study  of  the  two  con- 
ditions and  has  set  down  the  following  differential  criteria: 

1  Presbyophr6nie  de  Wernicke  et  les  Psychopoly  nC-vrites,  L'Enccphale,  February  10, 
19U. 


956     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC    PSYCHOSES 


Korsakow's  psychosis  affects  persons  particularly  of  adult  age; 
presbyoplirenia,  on  the  contrary,  belongs  to  the  period  of  old  age, 
the  age  of  predilection  oscillating  about  seventy  years.  Presbyo- 
phrenia, it  is  known,  is  quite  uniquely  an  affection  of  women,  w^hile 
Korsakow's  psychosis  is  far  from  being  rare  among  men.  The  humor 
of  the  presbyophrenic  is  always  gay,  euphoric  and  satisfied,  quite 
different  from  the  psychopolyneuritic,  who  is  ordinarily  depressed  or 
apathetic  and  whose  face  preserves  an  invariable  immobility.  The 
traits  of  the  presbyophrenic  are  extreme  mobility,  they  laugh,  make 
grimaces  in  which  the  mimic  muscles  participate.  The  facies  of  the 
psychopolyneuritic,  however,  are  always  dull  and  without  expression. 
Loquacity  is  a  symptom  scarcely  ever  lacking  in  presbyophrenia; 
the  patients  talk  without  stopping  about  ever\i:hing,  with  equal 
volubility.  This  symptom  is  lacking  in  Korsakow's  psychosis.  The 
presbyophrenic  is  polite,  amiable  and  cordial,  characteristics  which 
one  seeks  in  vain  among  the  psychopolynem-itics.  Disorders  of  con- 
sciousness  are  much   more   marked   in  the    presbyophrenic.     These 


Fig.  448. — Presbyophrenic  facies. 

patients  have  no  understanding  of  their  state  of  illness  and  content 
themselves  with  laughing  when  one  asks  a  question  relative  to  their 
physical  or  mental  health.  On  the  contrary  the  psychopolyneuritic 
gives  a  fairly  good  account  of  himself  and  his  position,  and  is  the 
first  to  lament  his  situation.  The  judgment  of  these  patients,  even  in 
the  chronic  forms,  is  less  noticeably  affected,  and  their  degree  of 
intellectual  enfeeblcment  less  marked.  The  amnesia,  finally,  is  more 
profound  in  the  presbyophrenic  and  besides  in  this  aft'ection  the  patient 
does  not  possess  at  all  a  knowledge  of  this  amnesia — the  inverse  of 
what  one  obsers'es  in  the  chronic  forms  of  Korsakow's  disease,  where 
the  subjects  speak  spontaneously  to  their  mterlocutors  of  the  profound 
troubles  of  their  memory.  Presbyophrenia  is  rare  in  its  typical  forms, 
but  common  in  the  form  esfrustes.  Where  the  s\Tnptoms  only  approach 
or  where  certain  of  them  are  lacking,  the  presbyophrenia  has,  perhaps, 
the  distinctive  characteristics  of  arteriosclerosis,  which  are  seen  much 
more  among  these  patients  than  among  simple  senile  dements. 
Patients  die  nearly  always  of  cerebral  hemorrhage  and  their  nervous 
centers  present  at  autopsy  pronounced  atheromatous  lesions. 


THE  SENILE  PSYCHOSES  957 

Pathology. — Grossly  the  brain  shows  signs  of  atrophy  and  is  decreased 
in  weight,  the  bones  of  the  skull  are  thinned,  sometimes  in  well  defined 
regions,  particularly  the  temporal  regions.  There  is  a  compensatory 
external  hydrocephalus  as  a  result  of  the  atrophy  of  the  brain.  The 
convolutions  are  shnmken  and  the  fissures  correspondingly  widened. 
The  atrophy  is  not  uniformly  distributed  and  may  be  very  much  more 
marked  in  some  areas  than  others.  Arteriosclerosis  is  a  frequent 
finding,  but  is  not  a  necessary  part  of  the  picture.  In  fact  the  two 
processes  are  quite  distmct  in  every  way,  although  frecjuently  asso- 
ciated. When  arteriosclerosis  is  present  there  may,  of  course,  be 
found  its  results  in  such  lesions  as  softenings. 

The  arch itech tonic  of  the  cortex  is  greatly  disturbed.  The  nerve 
cells  show  advanced  degeneration  with  large  quantities  of  degenerative 
products  of  a  lipoid  nature  within  them,  amounting  to  a  severe  grade 
of  fatty  degeneration.  A  very  characteristic  picture  are  the  basket 
formations  about  the  nerve  cells.  The  neurofibrils  appear  to  be 
thickened  and  produce  whirls  and  loops  about  the  ner\T  cell.  It  is 
thought  by  some  that  these  basket  formations  are  due  to  incrustations 
upon  the  neurofibrils  of  perhaps  neuroglia  origm.  The  condition  is 
particularly  well  seen  in  presbyophrenia. 

The  i^iary  plaques^are  perhaps  the  most  distinctly  pathological 
findings  in  senile  dementia.  In  fact  they  are  distinctive  as  much  as 
any  pathological  finding  can  be  distinctive  of  any  one  condition. 
Their  presence  in  considerable  numbers  practically  makes  the  diag- 
nosis of  senile  dementia,  while  their  absence  or  extreme  rarity  would 
negative  such  a  diagnosis.  They  are  small  spots  of  necrosis  which 
are  not  dependent  upon  softenings  and  are  in  no  way  associated  with 
the  bloodvessels. 

There  is  a  great  deal  of  neuroglia  proliferation  throughout  the 
brain  and  marked  evidences  of  disintegration  and  the  presence  of 
scavenger  cells  and  the  like. 

Aside  from  these  changes  the  usual  changes  of  this  period  of  life  are 
found,  particularly  in  the  heart,  the  kidneys  and  other  internal  organs. 

Treatment. — The  mild  cases,  especially  those  that  maintain  their 
orientation  fairly  well,  can  be  cared  for  at  home.  Those  with  marked 
confusion,  especially  with  a  tendency  to  wandering,  need  an  atten- 
dant to  be  with  them.  There  is  danger  of  their  beconung  lost  and 
coming  to  grief,  or  if  they  wander  about  the  house  at  night  they  are 
apt  to  meet  with  some  accident,  more  often  to  fall  doAMi  stairs  and 
sustain  fractures.  Patients  who  are  very  resistive,  present  surgical 
troubles,  are  filthy  hi  habits,  or  show  a  tendency  to  commit  sexual 
offences,  should  be  cared  for  in  an  institution. 

As  regards  the  more  special  treatment,  little  is  to  be  said.  Hygienic 
surroundings,  a  simple  diet,  looking  after  the  emunctories,  and  if 
insomnia  is  present  the  occasional  exhibition  of  a  hyi)notic  constitutes 
about  all  there  is  to  be  done.  In  this  class  of  cases,  more  perliaps  than 
in  any  other,  is  the  use  of  alcohol  as  a  hypnotic  indicated.  \  little 
whisky  and  hot  water,  or  a  glass  of  beer  or  ale  acts  very  nicely.     It 


958     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

should  be  given,  however,  strictly  under  medical  authority  and  super- 
vision, as  these  patients  are  apt  to  be  susceptible  to  its  influences. 
In  the  earlier  stages  of  the  disease  potassium  iodide  is  the  drug  par 
excellence  for  its  general  alterative  properties  and  its  effect  on  the 
arterial  tension. 

As  soon  as  evidences  of  mental  deterioration  appear  relief  from 
busmess  worries,  cares,  responsibilities  and  mental  stresses  of  all 
sorts  is  indicated,  with  the  general  hope  of  limiting  disintegration  as 
far  as  possible.  Whether  this  is  of  value  or  not  is  pretty  difficult  to 
state,  for  it  must  be  remembered  that  there  appear  to  be  certain 
hereditary  tendencies  involved  even  in  this  condition  and  that  arterio- 
sclerotic disease  and  senile  dementia  both  appear  to  be  more  prevalent 
in  certain  families. 

Alzheimer's  Disease. — ^This  disease  was  first  described  by  Alzheimer 
in  1906  and  since  that  time  a  number  of  cases  have  been  reported. 
The  disease  occurs  usually  in  the  fifth  decade,  although  cases  have 
been  reported  in  the  early  part  of  the  fourth  decade  and  as  late  as  the 
beginning  of  the  seventh.  The  symptomatology  is  one  of  a  gradually, 
often  of  a  rather  rapidly  progressing  dementia,  intermpted  perhaps 
with  episodes  of  a  certain  amount  of  excitement  and  anxiety.  A 
rather  rapidly  progressing  dementia  in  a  man  of  about  forty,  or  a 
little  over,  is  in  itself  a  rather  unusual  picture,  and  m  the  absence 
of  signs  of  brain  tumor  or  syphilis,  Alzheimer's  disease  should  be 
thought  of.  The  dementia  is  marked  by  a  considerable  degree  of 
disorientation.  The  symptoms  otherwise  are  very  largely  neurological 
and  particularly  Realized  about  the  function  of  speech.  Various 
aphasic  and'apraxic  s^Tiiptoms  are  prommently  in  evidence.  Para- 
phasia and  asymbolia  are  quite  frequent.  There  may  also  be  spasticity 
and  convulsions,  although  there  are  never  well  marked  paralyses. 

The  pathology  of  this  condition  throws  almost  the  only  light  upon 
its  nature.    There  is  marked  and  extensivedegeneration  of 


cells  with  disturbances  of  cortical  architechtonic  and  the  presence  of 
large  quantities  of  disintegration  products,  while  there  are  found 
numbers  of  basket  formations  and  topical  miliary  plaques.  The  con- 
dition of  the  blo_od vessels  is  quite  normal.  The  pathological  picture 
thus  resembles  closely  that  found  in  senile  dementia.  In  general,  the 
disease  is  considered  as  a  presenile  dementia,  although  some  believe 
it  to  be  a  distinctive  disease. 

The  presence  of  this  disease,  if  it  be  considered  a  presenile  dementia, 
offers  another  one  of  those  warnings  to  us  not  to  be  too  dogmatic. 
It  would  appear  that  the  senium  is  by  no  means  a  clearly  defined 
period  of  life,  and  that  the  pathological  changes  which  are  usually 
thought  of  as  dependent  upon  old  age,  may  occur  within  wide  limits 
and  perhaps  represent  failure  of  special  tissue  resistances. 

Arteriosclerotic  Psychoses. — These  psychoses  are  dependent,  on 
their  physical  side,  upon  arteriosclerotic  changes  in  the  cerebral  blood- 
vessels, and  this  condition  is  due  in  turn  to  the  general_caiises3^h jgh 
produce  arteriosclerosis.     In  the  mam  the  two  factors  are  chronic 


ARTERIOSCLEROTIC  PSYCHOSES  959 

toxemia  and  high  blood-pressure.  It  must  be  borne  in  mind,  how- 
ever,  thaT^aiTadvanced  degree  of  arteriosclerosis  may  exist,  particu- 
larly in  the  peripheral  vessels,  and  the  cerebral  vessels  retain  their 
elasticity,  while  on  the  contrary  the  cerebral  vessels  may  be  severely 
sclerosed  while  the  peripheral  .J2alpablevessels_s^  change. 

Arteriosclerosis  is  essentially  a~regional  disease.  ~ 

The  psychoses  of  arteriosclerosis  form  another  one  of  the  connect- 
ing Imks  which  jom  the  periods  of  involution  and  the  senium.  ■Many 
of  the  involution  psychoses  merge  into  arteriosclerotic  deterioration, 
and  arteriosclerosis  is  frequently  combined  with  the  changes  incident 
to  the  senium. 

Aside  from  the  usual  causes  of  arteriosclerosis  there  are  undoubt- 
edly psychological  causes  at  work.  The<^ypertens!o5>  which  is  con- 
ceived as  so  important  an  element  in  the  causation  of  arteriosclerosis 
has  to  be  thought  of  as  a  heightened  visceral  tonus  and  as  such  depen- 
dent, to  some  extent  at  least,  upon  psychological  factors.  There 
would  seem  also  to  be  certain  heredilavy  factors  at  play.  Certain 
families  show  a  high  incidence  of  death  dependent  upon  arterio- 
sclerotic disease. 

There  are  four  fairly  well  defined  varieties  of  this  disease  based 
upon  both  clinical  and  pathological  findings  as  follows: 

1.  Arteriosclerotic  Brain  Atrophy. — This  occurs  in  two  forms:  a  mild 
form  with  severe  arterial  sclerosis  but  an  absence  of  focal  brain  lesions. 
The  s^Tnptoms  are  easy  fatigue,  slight  failure  of  memory-,  dizziness 
and  headache.  The  severe  tj-pe  may  resemble  the  mild  at  first  but  is 
progressive,  leads  to  profound  dementia  and  presents  in  its  course 
apoplectiform  and  epileptiform  attacks  and  focal  symptoms. 

2.  Subcortical  Encephalitis  (Binswanger). — In  this  condition  the 
white  matter  is  largely  involved  as  a  result  of  disease  of  the  long 
medullary^  arteries.  Apoplectiform  and  epileptiform  attacks  occur 
and  also  tfansitor\-  attacks  of  confusion,  aphasia  and  paresis,  dis- 
turbances suggesting  focal  lesions.  Focal  lesions  are  not  found  exten- 
sively but  areas  of  softening  often  occur  in  the  basal  ganglia. 

3.  Perivascular  Gliosis. — In  this  condition  there  is  a  disappearance 
of  nervous  elements  about  the  diseased  vessels  and  re])lacement  by 
neuroglia. 

4.  Senile  Cortical  Devastation. — Here  extensive  destruction  of 
cortical  areas  in  the  ^'ascular  territories  of  the  diseased  vessels  is 
found.  In  this  condition  the  arteriosclerosis  is  localized  largely  in 
the  small  cortical  vessels  which  come  off  from  the  pia.  The  basal 
vessels  remain  relatively  normal. 

Pick  has  very  thoroughly  described  certain  large  atro])hies  involv- 
ing whole  lobes  or  portions  of  lobes.  The  occijjital  lobes  may  be 
involved,  ])roducmg  blindness,  or  the  temporal,  jiroducing  deafness, 
for  example.  The  atrophy,  however,  does  not  always  follow  a  vas- 
cular area,  and  so,  while  it  is  generally  supposed  to  be  due  to  arterio- 
sclerotic disturbances  in  the  irrigation  of  these  territories,  the  cause 
is  not  always  altogether  clear. 


960     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

Sy^nptoms .—hi  the  main  the  symptomatology  of  cerebral  arterio- 
sclerosis is  one  of  gradually  progressive  mental  deterioration,  to 
which  are  added  the  evidences  of  focal  lesions  which  are  the  results 
of  thrombotic  softenings!     (^See  L hapter  on  Hemorrhage.) 

The  prodromal  disturbances  of  the  arteriosclerotic  psychoses  are 
very  apt  to  extend  over  a  considerable  period  of  time  and  manifest 
themselves  in  the  mam  as  nervousness  and  irritability,  with  headache, 
dizziness,  insomnia,  associated  of  course  with  the  special  signs  of 
the  vascular  disease,  more  particularly,  as  a  rule,  liigh  blood-pressure. 
With  this  series  of  symptoms  there  may  be,  of  course,  associated  U 
certain  amount  of  deafness,  with  sclerosis  of  the  drum  membrane, 
cardiac  attacks  with  the  Stokes- Adams  syndrome  and  evidences  of 
interstitial  nephritis.  Quite  frequently,  too,  these  patients  show  the 
signs  of  arteriosclerosis  of  the  vessels  of  the  spmal  cord,  with  perhaps 
some  sclerosis  of  the  palpable  vessels.  On  the  mental  side  the  patient 
may  have  a  feeling  of  growing  inefficiency,  at  least  his  work  shows  a 
falling  off  in  efficiency,  which  characteristically  manifests  itself  at  first 
in  a  failure  to  do  the  creative  things.  Pick  has  called  particular 
attention  on  the  emotional  side  to  a  lack  in  the  finer  modulations  of 
the  emotions.  Bleuler's  suggestion  must  be  remembered,  that  patients 
with  organic  brain  disease  fail  to  show  a  natural  emotional  reaction, 
not  because  of  any  defect  of  emotion  but  because  of  a  lack  of  grasp 
of  the  situation,  and  when  the  situation  is  fully  appreciated  an  adequate 
emotional  response  issues. 

These  prodromal  symptoms  gradually  merge  into  symptoms  of 
greater  severity  and  may  be  punctuated  from  time  to  time  by  attacks 
of  excitement  or  of  depression  and  delusions  may  develop  which 
are  characteristically  of  the  paranoid  type.  The  following  case  illus- 
trates this  paranoid  trend  very  well:  A  man  who  had  been  a  successful 
business  man  in  his  younger  days  began  to  fall  off  in  efficiency  as  he 
entered  the  arteriosclerotic  period  of  life  and  finally  entered  one  of 
the  Soldiers'  Homes  as  he  was  unable  to  adequately  support  himself. 
Here,  because  of  his  training  as  an  accountant,  he  was  employed  in 
the  office,  \^^lile  employed  here  the  characteristic  series  of  paranoid 
ideas  developed.  In  the  first  place  he  developed  exalted  ideas  of  his 
own  ability  which  were  distinctly  defensive  in  character,  compen- 
sating him  for  the  real  fact,  his  failing  ability.  Alongside  of  these 
exaggerated  ideas  he  had  delusions  of  a  persecutor^'  character.  He 
was  interfered  with  by  those  about  him  who  were  envious  of  him 
because  he  had  secured  such  a  good  position  in  the  Governor's  office. 
This  is  another  defense  reaction  in  the  opposite  direction  and  serves 
to  explain  to  him  how  a  really  efficient  man  after  all  can  turn  out  such 
poor  work.  It  is  not  his  fault,  but  the  fault  of  those  about  him,  and 
so  he  is  again  saved  from  the  realization  of  his  failings.  Removed 
from  this  situation  and  taken  to  a  hospital  where  he  could  no  longer 
indulge  in  alcohol,  which  had  been  a  factor  in  bringing  about  this 
condition,  he  recovered  from  these  distinct  psychotic  manifestations, 
but  without,  however,  a  full  insight  into  what  his  condition  had  been. 


ARTERIOSCLEROTIC  PSYCHOSES  961 

This  lack  of  insight  probably  had  its  basis  in  the  organic  changes. 
The  man  was  no  longer  capable  of  adjustment,  except  within  narrow 
limits  and  while  removal  from  the  painful  conditions  relieved  the 
situation,  he  was  unable  to  fully  understand  it. 

This  case  shows  very  well  how  even  in  a  psychosis  dependent  upon 
organic  brain  disease  the  mental  s\Tnptoms  as  such  must  receive  a 
psychological  interpretation . 

From  time  to  time  these  patients  show  periods  of  confusion  and 
bewilderment  with  disorientation.  These  periods  may  be  of  consider- 
able duration  and  some  of  them  at  least  are  dependent  upon  thrombotic 
attacks  which,  when  they  do  not  occur  in  the  motor  area,  are  easily 
overlooked.  Even  though  they  do  occur  in  the  motor  area  the  dis- 
turbance of  consciousness  may  not  be  very  great,  or  at  least  there 
is  nothing  comparable  to  the  unconsciousness  and  coma  of  cerebral 
hemorrhage;  there  is, perhaps,  only  a  slight  confusion,  and  the  paraly- 
sis, if  it  exists,  is  only  very  slight  and  is  often  explained  by  the  family 
as  the  result  of  some  inconsequential  cause.  Then,  again,  the  patient 
may  be  so  blunted  mentally  as  not  to  complain  of  a  slight  impairment 
of  function. 

These  patients  are  especially  susceptible  to  alcohol  and  become 
easily  very  badly  confused  from  small  quantities. 

One_  of  the  characteristic  features  with  regard  to  this  group  of 
psychoses  is  that  the  so-called  "nucleus  of  the  personality"  is  well 
presened.  The  patient  preserves  all  of  the  outward  appearances  of 
his  old  self  until  the  mental  dilapidation  has  reached  an  extreme 
degree.  There  is  not,  as  a  rule,  that  marked  "change"  in  the  indi- 
vidual that  is  seen  in  some  of  the  psychoses. 

The  areas  of  softening  produce  focal  lesions  and  these  focal  lesions 
are  most  prominent  in  the  motor  areas)  producing  various  upper 
motor  neuron  paralyses,  and  in  the  speech  areas  producing  various 
forms  of  aphasia  and  apraxia.  The  marked  focal  lesions,  particularly 
those  which  lead  to  well  defined  speech  disturbances,  hasten  very 
greatly  the  dementia.  An  aphasia  which  puts  the  patient  out  of 
actual  touch  with  his  fellows  hastens  the  tendency  to  mental  deterio- 
ration in  this  class  of  patients  who  are  no  longer  fluid,  but  are  well 
along  on  the  downhill  path  of  life.  They  need  every  stimulus  of 
mental  activity  to  even  hold  their  own,  and  as  soon  as  an  important 
function  like  speech  is  destroyed  they  are  quite  apt  to  lapse  pr()nii)tly 
into  a  serious  deterioration. 

It  is  in  this  class  of  patients  that,  as  a  result  t)f  the  focal  lesions, 
epileptiform  attacks  develop  late  in  life — the  so-called  "  late  epilep- 
sies." Their  significance  in  the  main  is  hi  pointing  to  the  focal 
disorder  and  as  an  indication  that  the  patient  needs  careful  oversight. 

Pathology.— The  pathology  shows  the  arteriosclerotic  i)rocess  in  the 
cerebral  vessels  in  vari(nis  stages  of  progress.  There  may  be  miliar\- 
aneurisms  of  the  smaller  vessels  and  other  vessels  may  be  c()nii)letely 
occluded  with  resulting  areas  of  softening.  These  areas  of  softening 
usually  show  entire  disintegration  of  the  nersous  elements  with 
61 


962     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC   PSYCHOSES 

large  numbers  of  scavenger  elements  about.  The  nen'e  cells  show 
various  grades  of  degenerative  change  dependent  upon  deficient 
nutrition  as  a  result  of  decrease  in  size  of  the  lumen  of  the  vessels. 
There  is  neuroglia  overgrowth  about  the  vessels  and  in  the  degenerated 
territories. 

Diagnosis. — ^The  diseases  most  apt  to  be  mistaken  for  the  arterio- 
sclerotic psychoses  are  paresis  and  the  psychoses  of  cerebral  syphilis, 
which  should  be  considered  together  now  that  the  etiology  of  paresis 
has  been  so  thoroughly  established.  The  reason  for  the  possibilities 
of  mistake  is  dependent  upon  the  fact  that  both  processes  are  more  or 
less  diffuse,  producing  a  progressive  mental  deterioration,  and   that 


■ 

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Fig.  449. — Extensive  thrombotic  softening,  the  result  of  arteriosclerosis.  Patient  aged 
eighty-three  years.  There  were  also  softenings  on  the  right  side  in  the  lower  portion 
of  the  cuneus  and  upper  portions  of  the  lingual  and  fusiform  lobules.  There  is  general 
atrophy  of  the  convolutions. 

both  processes  tend  to  be  more  severe  at  certain  points  and  thus  pro- 
duce focal  symptoms.  Sj^hilitic  and  metasyphilitic  disturbances 
occur,  as  a  rule,  at  a  much  earlier  age  than  the  arteriosclerotic,  gen- 
erally not  later  than  the  fourth  decade,  while  the  arteriosclerotic 
period  is  not  entered  until  the  fifth  decade.  When  an  arteriosclerotic 
has  had  syphilis  also  the  differentiation  becomes  more  difficult.  The 
Wassermann  of  the  cerebrospinal  fluid,  however,  would  be  negative, 
while  with  large  areas  of  softening  on  the  surface  of  the  cerebrum  there 
would  be  considerable  evidence  of  disintegration  products  in  the 
cerebrospinal  fluid.  On  the  mental  side  the  preservation  of  the 
"nucleus  of  the  personality"  is  much  more  in  evidence  than  in  paresis. 


m 


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L-' 


./  ...yiaT;:?' 


.-""V   ) 

,     J- 


Q 


r"  >     ^. 


Fig.  450. — Cellular  alterations  in  areas  of  softening.  Arteriosclerosis,  acute  soften- 
ing, lacunar  softening,  senile  deterioration,  etc.:  K,  Kornchenzellen;  E,  endothelial 
cells;  ADV,  adventitia  ceils;  F,  new  fibroblasts. 


Fig.  451. — Widely  distributed  arteriosclerotic  softenings;  patient  aged  sovonty-two 
years.  There  were  also  numerous  woftenings  in  the  basal  ganglia.  Brain  greatly 
Bhrunken.  It  can  bo  seen  from  the  distributioo  of  these  lesions  how  the  clinical  picture 
might  simulate  paresis. 


964     PRESENILE,  SENILE  AND  ARTERIOSCLEROTIC    PSYCHOSES 

Treatment.^ — In  a  general  way  the  treatment  should  be  prophylactic 
as  far  as  possible.  With  the  first  symptoms  of  prolonged  and  intract- 
able high  blood-pressure  the  individual  should  be  removed  from  the 
influences  of  physical  and  mental  stress,  and  the  usual  means  should 
be  employed  to  keep  the  blood-pressure  down  and  to  reduce  or  dc 
away  with  any  toxemic  condition  present.  A  carefully  regulated 
diet  from  which  alcohol  and  tobacco  are  excluded,  hydrotherapy 
intelligently  applied,  possibly  a  visit  to  some  watering  place,  and  in 
some  instances  a  visit  to  a  somewhat  higher  altitude,  with  moderate 
outdoor  exercise  and  the  exhibition  from  time  to  time  of  drugs  to 
reduce  the  pressure  and,  especially  where  there  are  kidney  complica- 
tions, the  drinking  of  considerable  quantities  of  mildly  alkaline  water 
are  in  general  the  things  to  be  depended  upon.  Insomnia  has  to  be 
dealt  with,  and  in  cases  where  the  patient  is  under  absolute  control 
a  small  amount  of  alcohol  at  night  will  sometimes  produce  the  desired 
result.  Unless  the  patient  is  under  absolute  control,  however,  it 
best  to  give  hot  milk,  or  such  simple  hj-pnotics  as  veronal. 


<  CHAPTER   XXIV. 

IDIOCY,  BIBECILITY,  FEEBLE-:MIXDEDXESS,  AND 
CHARACTEROLOGICAL  DEFECT  GROUPS. 

^^ITHIN  the  past  few  years  the  defective  classes  have  assumed 
enormous  sociological  importance,  so  that  this  chapter  has  easily 
become  perhaps  the  most  important  in  the  whole  group  of  disorders 
at  the  psychological  level,  considered  from  the  social  angle.  Of  course 
only  a  brief  outline  of  the  various  t^-pes  of  defect  can  be  given  in  a 
^ext-book  such  as  this. 

In  drawing  a  distinction  between  dementia  and  idiocy  Esquirol 
oaid:  "The  demented  man  is  deprived  of  the  good  that  he  formerly 
enjoyed;  he  is  a  rich  man  become  poor;  the  idiot  has  always  lived  in 
misfortune  and  poverty."  In  other  words  the  idiot,  the  imbecile,  and 
the  feeble-minded  lack  something ;  the  psychotics  are  suffering  from  a 
disorder  of  that  which  they  possess. 

This  definition,  so  admirably  worded  by  Esquirol,  adequately 
expressed  the  distinction  between  the  insane  and  the  idiot  and 
imbecile  according  to  the  knowledge  of  his  day,  and  has  been  used 
continuously  since  to  express  such  distinction.  It  can  no  longer  be 
said  to  be  a  valuable  formula  except  it  be  used  with  many  reservations 
and  explanations. 

In  the  first  place,  from  the  standpoint  of  this  book,  there  is  no  such 
thing  as  insanity,  at  least  in  a  medical  sense,  as  Esquirol  used  that 
term  and  aS  other  psychiatrists  in  the  past  and  the  present  have  used  it. 
Insanity  is  purely  a  legal  and  sociological  concept,  and  as  such  does 
not  imply  anything  more  than  the  judgment  of  a  man's  fellows  on  the 
desirability  of  having  him  live  in  the  community.  Defectiveness,  imder 
which  term  the  various  grades  of  idiocy,  imbecility,  and  feeble-minded- 
ness  are  included,  is  quite  as  generic  a  term,  and  while  it  refers  to  a 
condition  the  fundamental  characteristic  of  which  is  lack  of  develop- 
ment, it  includes  a  great  variety  of  states,  and  in  its  application  is  a 
relative  term  only. 

It  is  quite  as  illogical  to  group  all  defectives  together  and  endea\or 
to  draw  a  conclusion  from  their  study  as  a  whole,  as  it  is  to  group  all 
of  the  so-called  insane  together  and  endeavor  to  draw  a  conclusion 
from  the  study  of  all  of  them.  There  is  obviously  very  little  similarity 
between  the  cretin  and  the  defective  as  a  result  of  cerebral  hemorrhage 
during  a  prolonged  and  difficult  labor,  and  therefore  any  i)rincij)les 
or  conclusions  which  are  reached  as  regards  the  whole  defective  class 
without  an  adequate  appreciation  of  the  nniltitude  of  ditferent  con- 
ditions that  are  found  under  this  generic  caption  must  be  avoided. 

(965) 


966         IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

It  must  be  remembered,  too,  that  with  any  defective  patient  it  is 
possible  to  have  associated  a  psychosis,  so  that  the  picture  may  be  still 
more  complicated.  Therefore  in  other  parts  of  this  book  various  forms 
of  what  are  grouped  in  this  chapter  are  treated  of  from  an  etiological 
standpoint. 

In  considering  the  etiology  of  various  defect  states  the  important 
general  principle  that  should  be  borne  in  mind  is  the  location  of  the 
defect;  (1)  as  to  whether  it  is  in  the  germ  plasm  or  acquired,  and 
(2)  as  to  the  time  of  development  of  the  individual  when  it  became 
operative.  It  is  only  those  defects  which  are  the  result  of  a  lack 
of  some  element  in  the  germ  plasm  that  are  truly  of  a  hereditary 
nature. 

The  process  of  development  may  be  interrupted  at  any  time  from 
the  beginning  of  the  growth  of  the  child  in  the  uterus  to  the  attain- 
ment of  its  complete  adult  development,  and  as  this  interruption  may 
take  place  during  intra-uterine  life  it  is  in  such  instances  that  it  is 
important  to  make  the  distinction  between  a  truly  inherited  and  an 
acquired  defect.  In  both  instances  the  defect  is  congenital,  that  is, 
exists  from  birth. 

Disease  or  injury  may  affect  the  child  during  intra-uterine  life  or 
during  the  process  of  birth  or  after  birth  and  during  the  processes 
of  extra-uterine  development.  Previous  to  birth,  illness  or  injury  to 
the  mother  are  the  common  etiological  factors ;  during  birth  asphyxia- 
tion and  injury  by  the  forceps  are  common  causes,  while  after  birth 
the  infectious  diseases  and  direct  injuries  enter  largely  into  the  etiology. 
Finally,  there  are  relative  conditions  of  defect  which  are  due  to  lack 
of  the  opportunity  to  develop,  such  defect,  for  example,  as  results  from 
the  deprivation  of  the  important  sense  organs,  as  the  eyes  and  ears. 
If  the  patient  be  born  blind  and  deaf,  under  ordinary  circumstances 
he  will  be  very  defective  mentally,  because  he  has  not  the  opportunities 
for  learning  which  the  ordinary  child  has.  On  a  still  different  plane 
relative  defect  due  to  sordid  and  unsanitary  conditions  and  lack  of 
educational  facilities  is  found.  Children  are  ignorant  who  have  no 
opportunity  to  go  to  school  and  learn,  and  may  even  lack  the  ordinary 
brightness  that  comes  about  spontaneously  if  they  have  lived  under 
insanitary  conditions  that  impaired  their  general  health  and  energies, 
especially  if  these  conditions  be  complicated  by  the  presence  in  the 
child  of  some  such  debilitating  factor  as  infected  tonsils,  adenoids, 
high  degree  of  myopia,  otitis  media  with  deafness,  and  such  other  like 
things  which  impair  the  general  health  and  vigor  of  the  child  and 
interfere  with  the  patency  of  the  avenues  through  which  he  gains  his 
information  of  the  outer  world.  This  general  enfeeblement  of  the  child 
may  also  be  the  result  of  debilitating  habits,  such  as  masturbation 
and  the  use  of  narcotics  and  alcohoUc  drinks,  the  latter  of  which 
especially  are  an  extreme  expression  of  a  vicious  environment. 

Classification. — ^The  general  group  of  defectives  is  a  very  wide  one 
and  includes  not  only  the  idiots  and  the  imbeciles  and  the  frankly 
feeble-minded,  but  the  still  higher  grades  of  backward  children,  and 


IDIOCY  967 

then  a  whole  borderland  group  of  ne'er-do-wells  m  whom  the  defect  is 
much  less  clearly  defined  than  in  the  lower  grades. 

David  Starr  Jordan  has  said  that  "A  good  citizen  is  one  who  can 
take  care  of  himself  and  has  something  left  over  for  the  common 
welfare."  This  saying  represents  in  a  general  way  the  basis  on  which 
the  classification  of  this  group  may  be  based.  The  defectives  as  a 
class  may  be  said  not  only  not  to  have  anything  left  over  for  the 
common  welfare,  but  only  in  the  highest  grades,  and  then  under  the 
most  favorable  circumstances,  can  they  care  for  themselves.  A  general 
basis  of  classification  has  been  their  educability  and  their  capacity  to 
care  for  themselves,  according  to  some  such  scheme  as  follows,  for 
example : 

Feeble-mindedness. — A  condition  of  mental  defectiveness  capable 
of  much  improvement  by  educational  methods.  The  afflicted  individ- 
ual may  ultimately  take  a  place  in  the  world  and  be  self-supporting 
under  favorable  circumstances. 

Imbecility. — ^A  condition  of  mental  deficiency  which  can,  however, 
be  materially  improved  by  training,  but  not  sufficiently  for  the  subject 
to  take  a  place  in  the  world. 

Moral  Imbecility.^ — A  condition  of  mental  defectiveness  which  is 
sho^^'n  predominantly  by  the  absence  of  the  highest  functions,  particu- 
larly the  moral;  capable  of  training  to  a  considerable  degree,  but 
always  a  menace  to  society. 

Idio-imbecility. — A  condition  midway  between  idiocy  and  imbecility. 

Idiocy. — A  condition  of  profound  mental  defectiveness.  The  lower 
grades  are  unteachable,  while  the  higher  may  be  trained  slightly  in 
self-help,  i.  e.,  to  attend  to  the  calls  of  nature. 

More  recently  an  attempt  has  been  made  to  define  with  greater 
accuracy  the  different  grades  of  defect,  and  this  effort  has  taken  the 
form  of  an  attempt  to  correlate  the  psychological  development  of  the 
defective  with  the  psychological  development  of  the  average  child, 
so  that  the  defective  as  a  result  of  this  correlation  is  said  to  correspond 
to  the  development  of  the  average  child  at  such  and  such  an  age.  In 
other  words,  age  has  come  to  have  a  psychological  rather  than  a 
chronological  significance,  and  an  individual  who  may  be  forty  years 
old,  but  who  is  only  developed  mentally  to  the  extent  that  an  average 
child  is  developed  at  the  age  of  seven,  is  spoken  of  as  having  the 
psychological  age  of  seven  years. 

Until  the  use  of  this  scheme  of  classification  all  people  were  classified 
among  other  ways,  in  accordance  with  their  chronological  age.  For 
example,  in  this  country  a  person  attains  his  majority  and  can  cast 
his  vote  at  the  age  of  twenty-one.  This  refers  of  course  entirely  to  the 
chronological  age.  It  can  easily  be  seen  that  if  there  are  a  material 
percentage  of  persons  in  the  comnnniity  who  are  defectives,  although 
they  may  have  acquired  the  chronological  age  of  twenty-one  they  have 
not  the  mental  development  and  the  judgment  that  it  is  exi)ected 
goes  with  such  an  age,  and  therefore  from  the  psychological  standpoint 
are  not  twenty-one  and  ought  not  on  that  basis  be  permitted  to  vote. 


968 


IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 


The  psychological  classification  of  the  age  as  outlined  by  the  Binet- 
Simon  test  is  a  far  more  accurate  way  to  standardize  the  mental 
development  of  the  individual  than  the  chronological  age  method, 
and  although  there  are  objections  which  may  be  urged  against  it  and 
although  it  is  concededly  not  a  perfect  method  and  is  at  present 
undergoing  gradual  modifications,  still  it  is  so  much  better  than  the 
chronological  method  that  it  should  be  used  in  its  place,  and  is  by 
far  the  best  standard  which  we  have  for  designating  the  development 
of  the  defective.  The  classification,  according  to  this  method  which 
has  been  adopted  by  the  American  Association  for  the  Study  of  the 
Feeble-minded,  is  as  follows: 


Mental  age. 
Under  one  year 

1  year 

2  years 

3  years 

4  years 

5  years 

6  years 

7  years 

8  years 

9  years 

10  years 

11  years 

12  years 


Capabilities. 
Helpless 

Feeds  self.     Eats  everything 
Eats  discriminatingly 
No  work.     Plays  little 
Tries  to  help 
Only  the  simplest  tasks 
Tasks  of  short  duration.     Washes  dishes 
Little  errands  in  house.     Dusts 
Errands.     Light  work.     Makes  beds 
Hea\'ier  work.     Scrubs,   mends,  lays  bricks, 

cares  for  room  wdth  simple  furniture 
Good  institution  helpers.     Routine  work 
Fairly  compUcated  work  with  only  occasional 

oversight 
Uses   machinery.      Cares    for   animals.      No 

supervision.     Cannot  plan 


Class. 
Low 
Middle 
High 
Low 


Middle 

High 
Low 


Middle 


High 


Idiot. 


Imbecile. 


-Moron. 


The  classification,  according  to  the  psychological  age,  while  it  is 
practically  useful  for  defining  the  stage  of  development  which  the 
individual  has  reached,  is  also  of  some  value  in  difi^erentiating  hered- 
itary and  acquired  conditions.  Somewhere  from  65  to  80  per  cent, 
of  defectives  have  feeble-minded  parents,  and  therefore  in  most  of 
them  the  condition  is  hereditary.  In  these  hereditary  cases  it  would 
seem  as  if  the  child  developed  quite  normally  up  to  a  certain  point  and 
then  stopped  rather  suddenly,  and  that  this  stopping  was  a  pretty 
general  one,  so  that  the  development  ceases  at  a  certain  level  wathout 
many  irregularities.  A  patient,  for  example,  will  test  to  seven  years 
of  age,  will  do  practically  all  of  the  tests  for  seven  years,  and  fail 
completely  with  all  of  the  tests  above  that.  In  other  words,  the  age 
is  very  accurately  seven.  The  defect  is  a  clean-cut  one.  When  this 
condition  of  affairs  is  found  there  are  reasons  to  suppose  that  the 
trouble  lies  in  the  germ  plasm. 

In  acquired  conditions  the  stoppage  is  not  so  abrupt  as  a  rule.  For 
example,  with  the  deterioration  that  goes  along  with  epilepsy  in  child- 
hood, the  tests  show  much  greater  irregularity,  a  much  more  uneven 
development,  and  this  might  be  expected  of  any  condition  in  which 
deterioration  was  going  on.  In  numerous  deteriorating  conditions 
such  results  are  found  and  therefore  it  can  be  seen  that  one  is  dealing 
not  only  with  a  stoppage  of  development,  but  with  a  disease  process 
which,  while  it  is  in  operation,  is  producing  symptoms  of  its  own. 


CLINICAL    VARIETIES  969 

The  most  useful  classification  of  the  feeble-minded  is  a  clinical  one. 
It  would  be  quite  as  absurd  to  enter  into  a  discussion  of  the  general 
psychology  or  the  general  pathology  of  defectives  as  it  would  be  of  the 
so-called  insane,  and  therefore  in  this  chapter  the  different  well  defined 
forms  will  be  briefly  and  separately  described. 

Clinical  Varieties. — Amaurotic  Family  Types  (Tay-Sachs  Disease). — 
This  is  a  disease  which  generally  aflects  more  than  one  child  in  the 
family  and  appears  to  be  confined  in  its  incidence  to  the  Jewish  race. 
Its  etiology  is  unknown.  Its  pathology  is  in  general  a  degeneration 
of  certain  elements  of  the  brain,  more  particularly  the  cortical  elements 
and  pyramidal  tracts,  including  particularly  the  optic  nerves.  It  has 
been  described  in  three  stages: 

First  Stage. — The  infant  is  usually  all  right  at  birth  and  the  disease 
does  not  make  itself  manifest  for  some  few  months  thereafter,  usually 
at  about  the  fourth  month.  At  this  tune  the  first  symptoms  observed 
are  some  weakness  in  the  neck  muscles  and  indications  of  dimness  of 
vision.  If  the  fundus  is  examined  during  the  fourth  or  fifth  month 
there  will  be  found  a  whitish-gray  s^Tnmetrical  patch,  oval  in  shape, 
with  a  horizontal  axis  accupying  the  macula  lutea.  In  the  center  of 
this  patch  is  seen  the  fovea  centralis  which  appears  as  a  dark  cherry- 
red  spot.  Optic  atrophy  follows,  and  later  total  amaurosis.  Second 
stage:  In  this'stage  the  weakness  of  the  neck  muscles  is  more  marked 
and  the  head  falls  backward  if  unsupported,  and  while  lying  on  the 
back  the  infant  is  unable  to  turn  over  to  either  side.  The  handgrasp 
is  noted  to  be  feeble,  objects  are  dropped  and  the  infant  is  generally 
apathetic.  The  vision  is  materially  reduced  in  this  stage,  but  the 
senses  of  taste  and  hearing  are  preserved,  the  sense  of  hearing  appearing 
to  be  unusually  acute.  Third  stage:  In  this  stage  the  affected  muscles 
are  atrophied,  and  later  the  atrophy  extends  to  all  of  the  muscles  of 
the  body,  emaciation  becomes  marked,  the  reflexes  exaggerated,  and 
late  in  the  course  of  the  disease  the  extremities  become  rigid,  and  there 
is  retraction  of  the  head. 

Spasmodic  contractions  and  convulsions  have  been  noted.  There 
is  at  no  time  any  rise  in  temperature,  and  the  thoracic  and  abdominal 
viscera  remain  normal.    Death  usually  occurs  in  less  than  two  years. 

Sclerotic  Types. — Certain  types  of  mental  defect  are  seen  associated 
with  a  condition  of  the  brain  which  in  general  may  be  said  to  be  due 
to  an  overproduction  of  neuroglia  tissue  and  corresponding  atrophy 
and  disorientation  of  nerve  elements.  The  exact  nature  of  this  process 
is  not  definitely  knoA\Ti.  It  is  not  improbable  that  there  are  a  number 
of  different  conditions  comprised  in  this  general  picture. 

The  sclerosis  may  be  diffused  pretty  generally  or  it  may  be  localized 
in  patches  and  the  affected  portions  may  be  atrophic  or  they  nuiy  be 
hypertrophic.  When  the  process  is  hypertrophic  and  invohes  a  large 
portion  of  the  brain  one  finds  what  has  been  called  a  hy])ertroi)hied 
brain.  The  brain  is  much  larger  and  heavier  than  the  normal,  and 
is  much  firmer  in  consistence  after  being  hardened.  It  does  not  look 
like  a  normal  brain,  but  the  surface  has  a  cauliflower  ai)pearance. 


970  IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

The  condition  is  usually  associated  with  grave  degrees  of  mental  defect 
and  with  epilepsy.  The  localized  varieties  are  more  apt  to  be  asso- 
ciated with  convulsions  than  the  diffuse,  while  there  may  also  be  noted 
marked  tremors. 

This  disease  has  been  recently  correlated  with  other  changes  than  the 
local  cerebral  changes.  Kufs^  in  a  valuable  extensive  article,  including 
autopsy  material,  has  accumulated  the  evidence  for  a  distinct  disease 
entity  which  comprises  various  manifestations.  In  the  first  place, 
besides  these  changes  in  the  brain,  which  have  briefly  been  referred 
to,  there  is  a  distinct  cutaneous  affection  in  the  form  of  adenoma 
sebaceum,  which  affects  more  particularly  the  face  and  the  back. 


Fig.  452. — Adenoma  sebaceum.  The  skin  affection  associated  with  tuberous  sclerosis 
of  the  brain,  a  profound  degree  of  mental  defect  and  various  tumors  of  the  ^dscera, 
especially  the  kidneys.     (Courtesy  of  Dr.  Martin  W.  Barr.) 

Along  with  this  condition  is  frequently  found  associated  mixed  tumors 
of  the  kidney — tumors  made  up  of  various  elements  of  which  the 
smooth  unstriped  muscle  tissue  is  the  most  prominent. 

These  three  symptoms,  then,  adenoma  sebaceum,  mixed  tumor 
of  the  kidney,  tuberous  sclerosis  of  the  brain,  are  the  triad  which 
constitute  the  most  important  evidences  of  this  disease  entity.  In 
addition  to  this  pathology  it  is  to  be  noted  first,  about  the  sclerosis 
that  it  involves  sometimes  the  cerebellum,  that  tumors  of  the  ventricles 
are  quite  characteristic,  and  that  relatively  commonly  there  is  asso- 
ciated with  this  triad  of  symptoms  rhabdomyoma  of  the  heart.    Myoma 

1  Beitrage  zur  Diagnostik  und  pathologischen  Anatomie  der  tuberosen  Hirnsklerose 
und  der  mit  ihr  kombinierten  Nierenmischtumoren  und  Hautaffektionen  und  iiber 
den  Befund  einer  akzessorischen  Nebenniere  in  einen  Ovarium  bei  derselben.  Ztschr. 
f.  die  gesamte  Neurologie  und  Psychiatric,  Band  xviii,  Heft  3. 


CLINICAL  VARIETIES  '  971 

of  other  organs,  such  as  the  stomach  and  the  uterus  have  also  been 
observed. 

While  on  the  mental  side,  along  with  this  condition  there  usually 
is  associated  a  marked  degree  of  mental  defect  with  epilepsy,  still 
Kirpicziks  has  reported  a  case  occurring  in  a  man  twenty-eight  years 
old  without  mental  impairment. 

Cretinism. — ^This  disease  is  endemic  in  certain  parts  of  Europe,  but 
so  far  as  we  come  in  contact  with  it  in  this  country  is  sporadic. 

The  disease  is  due  to  a  defect  in  the  secretion  of  the  thyroid  gland. 
All  degrees  of  defect  may  be  present,  from  athyroidism  through  the 
various  degrees  of  hypothyroidism.  The  several  degrees  of  cretinism 
which  are  described  and  which  correspond  with  different  degrees  of 
defect  are  three,  namely,  the  lowest  grade,  in  which  the  defect  is 
greatest,  the  cretins,  the  middle  grades,  the  semicretins,  and  the 
highest  grades,  the  cretinoids. 

The  disease  usually  begins  quite  early  in  the  life  of  the  child,  some- 
times during  the  first  year,  although  it  may  be  delayed  for  several  years. 
The  general  symptoms  are  those,  first,  of  retarded  development;  the 
child  appears  less  bright  than  he  should  be,  walking  is  learned  slowly, 
speech  is  delayed  in  development,  the  anterior  fontanelle  is  late  in 
closing;  and  ^second,  the  characteristic  cretinous  appearance.  The 
body  is  dwarfed,  the  head  relatively  large,  and  the  legs  short  and 
bowed,  hands  and  feet  stmnpy,  and  the  ossification  of  the  bones  is 
delayed.  The  appearance  of  the  face  is  t^'pical,  the  nose  is  broad  and 
flattened,  the  lips  are  thick,  the  tongue  thick  and  often  protruding 
from  the  partly  opened  mouth,  the  eyes  widely  separated,  the  eyelids 
often  heavy  and  swollen,  and  the  hair  coarse  and  scanty.  The  skin  is 
cachectic  in  appearance  and  dry  and  thick,  resembling  the  skin  of  the 
myxedematous  patient,  the  neck  is  short,  the  abdomen  protuberant, 
sometimes '  with  umbilical  hernia.  The  signs  of  puberty  are  late  in 
making  their  appearance,  there  is  often  a  failure  in  the  complete 
development  of  the  genital  organs,  and  many  of  these  patients  are 
sterile.  The  pulse  and  respiration  are  slow,  the  temperature  may  be 
subnormal,  and  the  movements  of  the  patient  are  usually  very  delib- 
erate. Mentally  the  cases  show  various  grades  of  defect  from  the 
lowest  grades  of  idiocy  through  the  ^'arious  degrees  of  imbecility.  In 
general  cretins  are  quite  good-natured,  pliable  individuals  who  are 
easy  to  get  along  with  and  care  for. 

Diagnosis. — In  the  matter  of  diagnosis  the  principal  conditions 
which  have  to  be  dift'erentiated  are  rickets,  achondroplasia,  and 
mongolism. 

Rickets  can  usually  be  dift'erentiated  by  the  characteristic  beading 
of  the  ribs,  the  symmetrical  enlargement  of  the  epiphyses,  and  the 
absence  of  the  typical  signs  of  cretinism. 

Achondroplasia  should  not  be  mistaken  for  cretinism.  There  is  an 
imperfect  development  of  the  long  bones,  but  none  of  the  signs  of  cretin- 
ism, particularly  none  of  the  impairment  of  mental  development. 


972  IDIOCY,   IMBECILITY  AND  FEEBLE-MINDEDNESS 


Fig.  453  Fig.  454 

Figs.  453  and  454.— Cretinism  before  and  after  treatment.     (Josefson.) 


h 

Fig.  455  Fig.  456 

Figs.  455  and  456. — Showing  bony  changes  before  and  after  treatment.     (Josefson.) 


MONGOLISM 


973 


^Mongolism  is  at  times  extremely  difficult  to  differentiate.  It  is 
very  important  that  this  condition  be  not  mistaken  for  cretinism,  par- 
ticularly with  reference  to  the  matter  of  treatment,  as  treatment  is 
capable  of  modifying  the  cretin,  but  not  the  mongol.  The  following 
table  of  differential  signs  taken  from  Shuttleworth  and  Potts, ^  will 
serve  to  point  out  the  detailed  characteristics  between  these  two 
conditions: 


MOXGOLISM. 

1.  Characteristics  noticeable  from 
birth. 

2.  Skull  brachycephalic:  contour 
rounded  or  short  oval;  longitudinal  and 
transverse    diameters    nearly    correspond. 

3.  Forehead  usually  smooth. 

4.  Palpebral  fissures  "almond-shaped," 
and  more  or  less  oblique  upward  and  out- 
ward. Frequent  epicanthus.  Strabis- 
mus common.  Ciliary  blepharitis  frequent. 

5.  Cheeks  chubby,  often  florid.  Com- 
plexion mottled. 

6.  Lips  often  transversely  fissured. 
Lower  lip  may  be  pursed  up  over  upper. 

7.  Tongue  large  and  coarsely  papil- 
lated  if  not  fissured.  Tongue  frequently 
protruded  and  drawn  back. 

8.  Skin  smooth  in  infancy,  but  furfur- 
aceous  later;  not  redundant  or  "baggy." 

9.  Hair  "wirj%"  often  "mouse  color," 
but  sometimes  blonde.  Downy  growth 
common  on  forehead  and  cheeks. 

10.  ThjToid  gland  palpable  to  greater 
or  less  extent. 

11.  No  fatty  tumors  (pseudoUpomata) 
in  posterior  triangle  of  neck. 

12.  Long  bones  somewhat  shorter  than 
usual,  but  slender. 

13.  Hands  broad;  thumb  and  little 
finger  short,  the  latter  often  cur\ed  toward 
ring  finger.     Fingers  taper  at  ends. 

14.  Feet  large  and  flat.  Fissure  be- 
tween great  and  next  toe  often  seen. 

15.  Abdomen  often  distended;  occa- 
sional umbilical  hernia;  often  inguinal 
hernia. 

16.  Expression  more  or  less  vivacious 
and  mobile,  observant  and  imitative. 


Cretinism. 

1.  Characteristics  often  not  notice- 
able until  sixth  or  seventh  month. 

2.  Skull  dolichocephalic:  flat  at  top 
(fontanelles  close  late),  expanded  later- 
ally;  broad   behind,   often  asymmetrical 

3.  Forehead  usually  wrinkled. 

4.  Palpebral  fissures  horizontal,  but 
appear  small,  owing  to  pseudo-edema  of 
eyelids.  .Strabismus  and  ciliary  bleph- 
aritis less  common. 

5.  Often  circumscribed  malar  flush; 
complexion  ashy  or  waxy. 

6.  Lower  lip  often  everted.  Mouth 
open.     Drivelling  common. 

7.  Tongue  large,  but  not  coarsely 
papillated  or  fissured.  Tip  of  tongue 
thickened,    and    constantly    protruding. 

8.  Skin  dry  and  scaly;  forms  folds 
here  and  there,  being  redundant  and 
"baggy." 

9.  Hair  harsh,  coarse,  and  scanty. 
Usually  of  darkish  tint  (Bourneville  saj'S 
brown) ;   scalp   often   eczematous. 

10.  Thyroid  gland  impalpable  to  most 
thorough  examination. 

11.  Fatty  tumors  (pseudolipomata) 
frequently  found  in  po.sterior  triangle 
of  neck,  etc. 

12.  Long  bones  shortened  and  thick- 
ened, in  some  cases  bowed. 

13.  Hands  broad,  thick,  and  stumpy, 
with  wrinkled  skin.  Fingers  square  at 
tips. 

14.  Feet  squat;  skin  redundant  about 
ankles  and  dorsum  of  foot. 

15.  Abdomen  very  bulky  and  promi- 
nent with  folds  of  skin;  umbilical  hernia 
common. 

16.  Expression  dull  and  immobile; 
unobservant  and  apathetic. 

Deficient  stature,  flattened  bridge  of  nose,  \vith  expanded  alte,  late  and  irregular 
dentition,  deferred  closure  of  fontanelles  and  retarded  puberty  are  similar  in  each  variety. 

Recently  cataract  has  been  described  as  frequentlx-  (^e^'eI()ping  in 
these  cases — an  indication  of  a  possible  endocrine  dysfunction. 

Treatment. — This  has  been  taken  up  in  Chapter  I\'  on  Diseases  of 
the  Internal  Secretions,  or  Endocrinopathies. 

Mongolism. — The  Mongolian  or  Kalmuc  type  of  defectiveness  is 
so-called  because  of  the  resemblance  of  the  patient  to  the  Mongolian 
race,  particularly  the  slant  of  the  eyes  and  the  general  facial  expression. 

1  Mentally  Deficient  Children,  London,  1910. 


974         IDIOCY,   IMBECILITY  AND  FEEBLE-MINDEDNESS 

The  present  belief  with  reference  to  this  condition  is  that  it  is  con- 
genital and  not  hereditary  in  the  true  sense,  that  is,  not  dependent 
upon  a  condition  of  the  germ  plasm.  The  condition  is  believed  to  be 
the  result  of  some  abnormal  state  of  the  mother  which  may  be  produced 
by  injury  or  shock,  but  which  in  general  is  believed  to  be  the  result  of  a 
worn-out  reproductive  capacity,  the  mother  being  unable  to  bring  the 
child  to  complete  development  in  the  uterus.  Corresponding  with 
this  assumption  the  Mongol  is  generally  the  last  child  born  in  the 
family,  and  not  infrequently  comes  from  good  stock. 


Fig.  457. — Mongolian  type,  aged  eighteen  years,  apathetic  idiot,  semimute,  speaks 
only  a  few  words,  enunciation  imperfect.  Learned  to  feed  himself  and  is  cleanly.  Dwarf- 
ish, brachycephalic,  forehead  flat  and  wrinkled  transversely.  Eyes  oblique,  photophobia, 
chronic  conjunctivitis.  Tongue  very  large,  filling  mouth  completely;  deeply  fissured, 
papillae  enlarged.  Hands  broad.  Fingers  short  and  thick.  (Courtesy  of  Dr.  Martin 
W.  Barr.) 


There  are  three  prominent  physical  signs  of  this  condition  which 
are  sufficient  when  present  to  make  a  diagnosis.  They  are  the  form 
of  the  skull  and  the  peculiarities  of  the  palpebral  fissures  and  of  the 
tongue.  The  skull  is  brachycephalic,  diminished  in  its  anteroposterior 
diameter,  flattened  on  the  face  and  occiput,  but  without  recession  of 
the  frontal  and  supraoccipital  regions,  as  in  t.he  microcephalic.  The 
tongue  is  large,  the  circumvallate  papillae  are  hypertrophied,  and 
there  are  marked  irregular  transverse  fissures.    This  condition  of  the 


MICROCEPHALIC  TYPES  975 

tongue  is  characteristic  of  this  t}-pe,  and  is  not  found  in  any  other 
variety.  Thompson  has  suggested  that  the  fissuration  is  dependent 
upon  two  factors:  an  extreme  vulnerability  of  the  mucous  membrane, 
and  the  habit  of  sucking  the  tongue,  commonly  present  in  these 
children.  The  hands  and  feet  are  broad,  clumsy  and  spatulate,  while 
an  incurving  of  the  little  finger  has  also  been  described  as  of  frequent 
occurrence  in  these  children.  In  addition  to  these  quite  characteristic 
s\-mptoms  the  children  are  clumsy,  joints  loose,  the  skin  rough,  the 
abdomen  protuberant  and  there  is  a  tendency  to  chronic  inflammatory 
conditions  of  the  mucous  surfaces.  The  circulation  is  generally  poor, 
congenital  cardiac  anomalies  may  be  present,  such  as  imperfect  closure 
of  the  foramen  ovale,  ^^tal  resistance  is  very  low,  and  these  patients 
are  quite  apt  to  die  relatively  early  in  life  from  tuberculosis. 

Mentally  these  children  are  usually  at  a  very  low  grade  of  develop- 
ment, generally  gravitating  about  four  years  of  age.  They  may  be 
less  or  they  may  possibly  reach  the  seven-year  limit,  but  rarely  go 
beyond  it.  As  a  rule  they  are  good-natured  and  easy  to  care  for  by 
those  who  are  understanding  and  sympathetic. 

A  special  form  of  cortical  aplasia  has  been  described  as  belonging 
to  this  condition. 

ThjToid  does  no  good  in  these  conditions.  It  is  therefore  necessary 
to  carefully  separate  them  diagnostically  from  cretinism,  a  condition 
which  resembles  it  very  closely  on  casual  observation.  For  differential 
diagnosis  see  Cretinism. 

Hydrocephalic  Types. — ^\  arious  degrees  of  mental  defect  may  be 
associated  with  hydrocephalus.  Hydrocephalus  may  of  course  occur 
previous  to  birth,  but  is  rarely  congenital,  as  a  child  with  any  marked 
degree  of  hydrocephalus  could  not  be  born  alive.  The  condition 
may  be  relatively  acute,  in  which  case  it  leads  rapidly  to  death,  but  it 
may  come  to  an  arrest  or  be  extremely  slow  in  progress.  In  this  latter 
group  of  cases  we  find  patients  sometimes  who  li\'e  to  a  fairly  advanced 
age,  although  as  a  rule  this  disease  terminates  life  before  the  patient 
has  passed  middle  life  and  generally  much  earlier.  The  symptoms  in 
the  slowly  progressive  cases  are  the  symptoms  of  gradual  obliteration 
of  the  mental  faculties,  and  are  undoubtedly  dependent  upon  pressure 
effects.  These  symptoms  are  in  general  loss  of  intelligence,  gradual 
loss  of  vision,  hearing,  the  function  of  language,  and  gradually  the 
sinking  into  a  semistuporous  condition,  and  death.  Tlie  causes  of 
hydrocephalus  are  probably  numerous,  syphilis,  tuberculosis,  brain 
tumor,  and  meningitis  are  among  chem. 

Microcephalic  Types. — IMental  defect  associated  with  extreme 
smallness  of  the  head.  No  definite  rule  can  be  laid  down  as  to  what 
size  of  head  should  be  considered  microcephalic.  Ireland  gives  the 
general  rule  that  heads  below  seventeen  inches  in  circumference 
(431  millimeters)  may  be  so  considered.  This  rule  is  not  absolute, 
and  when  it  is  considered  that  these  small-headed  varieties  may  prob- 
ably be  the  result  of  various  etiological  factors  it  will  be  appreciated 
that  the  term  microcephalic  had  better  be  used  purely  as  a  descriptive 


976         IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 


Fig.  458. — Hydrocephalus,  anteroposterior  diameter  9f  inches;  transverse  diameter 
71  inches.  This  man  for  many  j-ears  made  a  h\'ing  as  a  Ijell-ringer.  Prev-ious  to  death 
he  became  profoundly  demented  and  his  head  so  hea\'y  that  he  could  not  lift  it  from  the 
pillow.    Autopsy  showed  the  brain  to  be  literally  little  else  than  a  bag  of  fluid. 


Fig.  459. — On  left,  high-grade  imbecile,  aged  thirteen  years;  mentally,  six  years. 
right,  microcephalic  imbecile,  aged  nine  years;  mentally,  three  years. 


On 


MICROCEPHALIC   TYPES 


977 


term  rather  than  as  a  term  to  apply  to  a  definite  class  of  defectives 
even  though  that  class  be  considered  solely  from  a  morphological 
standpoint. 


Fig.  460. — Erma.     Frout  view. 


Fig.  461. — Erma.     Side  view. 


Fig.  462. — Marj'-     Front  view.  Fig.  463. — Mar>'.     Side  view. 

Microcephalic  twins,  aged  eight  years.     (Courtesy  of  Dr.  A.  C.  Rogers.  Faribault, 

Minnesota.) 
62 


978  IDIOCY,  IMBECILITY  AND  FEEBLE-MIKDEDNESS 

It  is  probable  that  two  sets  of  causes  may  produce  the  extremely 
small  skulls  which  are  found  in  the  microcephalic  types.  The  old 
theory  that  the  condition  was  due  to  premature  synostosis  has  long 
since  been  discarded,  as  has  also  the  operation  of  craniectomy  based 
upon  that  theory. 

The  characteristic  condition  of  the  microcephalic  brain  is  its  extreme 
smallness,  more  particularly  pronounced  in  the  temporosphenoidal, 
parietal  and  occipital  regions.  The  posterior  lobes  of  the  cerebrum 
do  not  cover  the  cerebellum.  The  convolutions  of  the  cerebrum  are 
more  simple  in  pattern  than  in  the  normal  brain,  and  in  addition  there 
may  be  localized  ageneses  with  resulting  microgyria.  There  may  also 
be  associated  morbid  processes  such  as  encephalitis.  The  h;v^oplasia 
usually  also  involves  the  spinal  cord. 


^3if. 


f^^ 


Fig.  464. — Paralji;ic  type.     Athetoid  movements  of  hands  and  arms. 


The  general  appearance  of  these  patients  is  quite  characteristic. 
The  conformation  of  the  skull  is  "sugar  loaf,"  or  as  it  is  technically 
termed  "oxycephalic."  This  is  characterized  by  a  rapidly  receding 
forehead  with  a  fiat  occiput.  Along  with  the  receding  forehead  there  is 
usually  also  a  receding  chin  which  gives  a  pointed  aspect  to  the  face, 
which,  associated  with  a  small  stature  gives  a  quite  characteristic 
general  appearance  to  these  patients  and  has  led  to  their  being  char- 
acterized as  "bird-like"  in  appearance. 

The  mental  development  of  these  patients  varies  between  wide 
limits,  although  the  tendency  is  for  them  to  belong  to  the  lower  grades 
of  defect.  They  are  usually  well  disposed,  good-natured,  affectionate, 
and  not  difficult  to  care  for.  They  generally  do  not  live  to  advanced 
years,  but  die  at  rather  an  early  age. 

Paralytic  Types. — There  are  a  large  variety  of  cases  in  this  group. 
The  paraly.ses  may  involve  any  portion  of  the  body  or  be  of  almost 
any  extent.  ^Monoplegias  and  diplegias  are  common,  but  localized 
palsies  and  hemiplegias  are  also  not  infrequent.  They  depend  either 
upon  lack  of  development  of  certain  portions  of  the  brain  or  more 
frequently  upon  injuries  and  sometimes  new  growths.  Hemorrhage  is 
the  most  frequent  injury  that  produces  the  various  palsies.    This  may 


EPILEPTIC  TYPES 


979 


occur  as  the  result  of  prolonged  labor  or  injury  by  forceps  deli\ery, 
or  may  be  the  result  of  injury  during  the  early  months  of  life.  The 
amount  and  the  character  of  the  mental  defect  varies  within  wide  limits 
and  no  description  which  would  apply  to  this  whole  class  would  be 
possible,  principally  because  the  class  is  not  homogeneous. 

Porencephaliis.—\\ kh'm  this  group  of  paralytic  t^x-s  one  of  the 
conditions  which  is  found  and  which  has  been  frequently  described  is 
porencephalus.     This   condition   is  consequent  upon   g-oss  cerebral 
lesions  such  as  a  lack  of  cerebral  substance 
resulting  in  a  cyst  connected  with  the  ven- 
tricle— true  jjorencephalus,  or  due  to  cysts 
not  connecting  with   the   ventricles   and 
resulting  from  softening,  hemorrhage,  or 
inflammation — jahe  porencephalus. 

Traumatic  Types.  —Arrest  of  mental 
development  may  occur  as  the  result  of 
an  injury  to  the  brain  during  the  develop- 
mental period.  The  most  frequent  t>T)e  . 
of  injury  producing  this  result  is  pro- 
longed labor  with  mstrumental  delivery. 
Where  the  injury  produces  a  lesion  in  the 
motor  pathway  with  a  resulting  paralysis 
the  patient  is  generally  included  within 
the  paralytic  group. 

Epileptic  Types.  —  Epilepsy  occurring 
early  in  life  is  one  of  the  causes  of  lack  of 
mental  development.  Just  how  this  result 
is  brought  about  by  the  epilepsy  is  not 
altogether  clear.  In  part  it  is  due  to  the 
fact  that  the  child  has  to  be  differently 
treated  from  normal  children,  is  often 
deprived  of  the  same  educational  advan- 
tages because  of  its  disease,  but  apart 
from  this  there  seems  to  be  a  direct  rela- 
tion between  the  lack  of  development  and 
mental  deteiioration,  and  the  epilepsy 
itself.  This  is  probably  dependent  upon 
the  fact  that  the  epilepsy  is  a  disease 
which    strikes   very   deep,    close   to   the 

foundation  of  the  neurological  mechanisms.  Its  manifestations  are 
evidently,  in  part  at  least,  dependent  upon  disturbances  at  the 
physicochemical  level.  Disturbances  which  are  as  fundamental  as 
this  necessarily  are  difficult  to  deal  witJi  and  also  necessarily  impair 
the  superposed  levels.     (.See  Chapter  on  Ejulcpsy.) 

Epileptic  attacks  are  found  in  many  of  the  other  forms  of  defect, 
more  particularly  in  those  defects  associated  with  gross  cerebral 
lesions  such  as  are  found  in  the  paralytic  and  traumatic  types.  It  has 
generplly  been  assumed  that  the  localized  lesion  was  the  cause  of  the 


Fig.  465. — Hemiplegic,  aged 
eleven  years.  (Courtesy  ot  Dr. 
A.  C.  Rogers,  Faribault,  Min- 
nesota.) 


980  IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

epileptic  manifestations.  While  this  may  be  so  in  certain  cases,  still 
in  a  general  way  it  must  be  borne  in  mind  that  classical  epilepsy  has, 
presumably,  a  hereditary  background  dependent  upon  a  defect  in 
the  germ  plasm  and  that  perhaps  only  those  children  develop  con- 
vulsions as  a  result  of  injury  or  localized  cerebral  lesions  in  whom 
hereditary  conditions  are  favorable.  At  least  the  hereditary  factor' 
should  not  be  neglected  in  the  study  of  the  patient  simply  because  a 
localized  lesion  has  been  found.  Perhaps  the  localized  lesion  could 
not  have. produced  such  a  result  without  the  hereditary  factor — the 
spasmophilic  tendency. 

The  epileptic  type  of  defective,  besides  the  symptoms  of  his  defect, 
characteristically  manifests  the  symptoms  of  the  epileptic  character 
and  is  therefore  quite  a  difficult  problem  with  which  to  deal. 

Inflammatory  Types. — This  group  includes  those  conditions  resulting 
from  inflammation  of  the  meninges  and  of  the  brain,  found  most 
frequently  as  a  result  of  acute  infectious  fevers  such  as  pneumonia, 
typhoid,  and  the  exanthemata.  Local  areas  of  meningitis  or  meningo- 
encephalitis are  not  infrequent  in  connection  with  the  specific  fevers, 
and  when  extensive  or  severe  and  occurring  early  in  life  produce  an 
arrest  of  development,  to  a  certain  degree,  of  the  mental  faculties 
together,  not  infrequently,  with  epilepsy.  Striimpell's  polioencepha- 
litis superior  belongs  in  this  group. 

Sensorial  Types. — The  defect  in  this  group  is  the  result  of  deprivation. 
Here  there  is  no  defect  of  the  germ  plasm  or  no  defect  in  the  structure 
of  the  brain,  but  owing  to  injury  or  disease  which  has  destroyed 
the  patient's  vision  or  hearing  or  both  the  child  is  cut  off  from  com- 
munication with  the  outside  world  to  such  an  extent  that  mental 
development  is  impaired  thereby.  Theoretically  these  patients  are 
of  course  educable,  but  it  is  only  very  rarely  that  one  is  found  with  the 
capacity,  the  patience,  and  the  ingenuity  to  develop  such  a  child  by 
educational  methods.  The  cases  of  Helen  Keller  and  Laura  Bridgman 
stand  out  as  illustrative  of  what  can  be  done. 

Milder  grades  of  defect  due  to  deprivation  of  the  ordinary  educa- 
tional advantages  occur  on  the  same  principle. 

Syphilitic  Types. — Syphilitic  types  of  defect  have  always  been 
known  in  that  group  of  cases  presentmg  distinct  evidences  of  hereditary 
sj^hilis,  particularly  the  Hutchinson  teeth,  linear  scars  about  the 
mouth  and  nose,  and  keratitis.  Until,  however,  the  discovery  of  the 
Treponema  pallidum  and  the  elaboration  of  the  Wassermann  reaction 
there  was  no  comprehension  of  the  frequency  with  which  syphilis 
entered  into  the  etiology  of  the  various  types  of  mental  defect.  One 
of  the  best  knowii  authors,  who  just  antedated  this  period,  says  that 
the  number  of  cases  of  defectives  due  to  syphilis  is  quite  insignificant, 
probably  not  more  than  1  or  2  per  cent.,  and  comments  upon  this 
in  the  face  of  the  frequency  of  syphilitic  disease  in  the  fully  developed 
brain  and  the  frequency  of  so-called  inherited  syphilis. 

There  is  one  distinct  type  of  disease  which  only  recently  has  come 
to  be  recognized  with  any  degree  of  accuracy  which  used  to  be  classed 


SYPHILITIC   TYPES 


981 


among  the  defective  states  and  which  is  due  to  syphilis,  namely, 
juvenile  general  paresis.  It  is  probable  that  still  in  many  places  this 
disease  is  not  recognized,  but  is  put  down  probably  as  some  form  of 
progressive  defect.  The  number  of  juvenile  paretics  is  not  very 
large. 

The  application  of  the  Wassermann  test  to  the  defectives  as  a  class 
shows  that  not  far  from  20  per  cent,  of  the  patients  taken  indiscrim- 
inately show  a  positive  Wassermann  reaction.  Of  course  it  is  quite 
another  question  as  to  just  what  the  relationship  is  in  these  patients 
between  the  syphilis  and  the  mental  defect.     In  one  case  it  may  be 


Fig.  466. — Sensorial  type,  imbecile  by 
deprivation,  middle  grade.  Male,  aged 
about  thirty  years,  deaf-mute.  Taught 
himsell  to  talk  and  to  read  and  WTite  in 
a  fashion.  Often  crael  to  children  but 
devoted  to  animals.  Appropriates  things 
not  his  own.  Very  keen  and  observant. 
(Courtesy  of  Dr.  Martin  W.  Barr.) 


Fig.  467. — Sensorial  type,  imbecile 
by  deprivation,  middle  grade.  Boy, 
aged  ten  years,  deaf-mute,  no  ears. 
(Courtesy  of  Dr.  Martin  W.  Barr.) 


that  the  syphilis  has  produced  vascular  disease  and  the  defect  is  due  to 
a  vascular  lesion.  In  another  case  it  is  the  typical  effect  of  a  general 
paresis,  or  perhaps  a  meningoencephalitis,  and  still  further  ])robably 
syphilis  has  a  decidedly  deleterious  effect  upon  development  in  ways 
that  we  do  not  at  present  understand,  so  tliat  it  may  well  be  that 
certain  of  the  t\pes  of  defect  which  do  not  present  characteristic 
syphilitic  lesions  may  be  due  to  subtle  nutritional  changes,  which,  in 
the  last  analysis,  have  their  origin  in  syphilis.  The  great  ])art  that  this 
disease  plays  in  this  class  of  eases  is  yearly  becoming  more  and  more 
evident. 


982  IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

Inflammatory  conditions  of  all  sorts  and  their  results  are  dependent 
upon  this  cause — meningitis,  hemiplegia,  porencephalus,  hydro- 
cephalus, which  may  be  associated  with  epileptic  convulsions. 

Idiot-savants. — These  are  rare  cases,  who,  although  often  deeply 
defective,  still  have  some  special  ability  wonderfully  developed.  It 
may  be  music,  calculation,  or  memory  for  some  special  class  of  facts,  etc. 

The  calculators  can  name  the  answer  to  mathematical  problems 
almost  instantly;  the  musical  prodigies  often  play  well  and  may  even 
improvise;  one  patient  under  the  observation  of  one  of  the  authors 
would  instantly  name  the  day  of  the  week  for  any  date  for  years 
back.  Many  of  these  patients  have  a  capacity  for  mimicry  and 
buffoonery,  and  from  this  class  undoubtedly  were  recruited  in  the 
old  days  many  of  the  court  fools.. 

The  psychology  of  these  individuals  is  not  understood  and  they 
themselves  are  quite  unable  to  give  any  explanation  of  their  special 
abilities.  Their  abilities,  however,  are  really  not  so  great  as  they 
appear.  They  appear  exaggerated  because  they  stand  out  upon  a 
background  of  pronounced  defect,  also  because  they  are  unusual 
in  the  sense  of  not  being  the  common  possession  of  mankind.  The 
calculators,  for  example,  do  very  wonderful  calculations  in  the  way  of 
adding  up  long  series  of  figures  very  rapidly.  There  are,  however, 
well-known  devices  for  increasing  the  rapidity  of  the  ordinary  old- 
fashioned  method  of  adding,  and  many  of  our  experts  at  figures  today 
■  could  compete  with  some  degree  of  success  with  these  calculators. 
The  patient,  for  example,  who  could  name  the  day  of  the  week  for 
years  back  upon  merely  being  given  the  date,  and  do  it  instantly, 
spent  most  of  his  time  in  studying  calenders.  It  would  not  be  surpris- 
ing if  almost  anyone  could  accomplish  such  a  feat  if  he  spent  any 
such  commensurate  degree  of  effort  upon  it. 

Other  descriptive  terms  are  used  to  describe  certain  t^^pes  of  defec- 
tives, for  instance  in  addition  to  the  so-called  Mongol  or  Kalmuc  types 
there  are  described  American  Indian  and  Negroid  types  because  of  the 
obvious  resemblances.  Then  again,  for  purposes  of  practical  classifica- 
tion the  lower  grades  of  defectives  are  spoken  of  as  either  apathetic 
or  excitable,  and  certain  of  these  latter  who  keep  up  certain  definite 
and  characteristic  movements  almost  continuously  are  described  as 
rhythmic  idiots.  Other  motor  disturbances  of  course  also  occur.  Aside 
from  paralysis  and  epilepsy,  probably  one  of  the  most  common  is 
athetosis. 

Mild  Grades  of  Defect. — A  systematic  examination  of  large  numbers 
of  children  has  disclosed  the  fact  that  a  gi-eat  number  of  the  disorders 
of  conduct  and  types  of  inefficiency  which  are  manifested  among  them 
are  dependent  upon  some  degree  of  feeble-mindedness.  Systematic 
examinations  of  school  children,  for  example,  have  resulted  in  showing 
a  not  inconsiderable  percentage  of  the  general  school  population  so  far 
behind  in  their  mental  development  as  only  to  be  described  by  a  term 
indicating  an  inherent  defect.  These  defects  range  all  the  way  from 
well  marked  imbecility  among  the  younger  children  up  through  the 


MILD  GRADES  OF  DEFECT 


983 


grades  of  the  so-called  backward  children;  for  the  most  part,  the 
different  grades  of  the  moron,  of  the  feeble-minded.  The  measuring 
rod  which  has  been  used  for  determining  these  conditions  has  been 
the  Binet-Simon  scale  of  intelligence  tests,  and  the  treatment  which 
has  been  applied  has  been  the  segregation  of  these  defective  children 
from  the  general  school  population  into  classes  and  sometimes  whole 
schools  devoted  particularly  to  them,  thereby  gaining  both  the  advan- 
tage of  the  application  of  special  educational  efforts  to  these  children 
and  the  relief  of  the  normal  child  from  the  dragback  to  which  he  was 
subjected  by  having  the  defective  in  the  same  class  with  him. 

Many  of  the  mental  defects,  it  must  be  borne  in  mind,  are  only 
relative  affairs  and  are  dependent  upon  general  conditions  of  ill  health, 
and  poor  nutrition,  cardiac  disease,  chronic  poisoning  (alcohol,  lead) 


Fig.  468. — .\merican  Indian 
type.  (Courtesy  of  Dr.  Martin 
W.  Barr.) 


Fi(i.  469.— \,m  (  1,111.  .y 

Dr.  Martin  W  .  J3aiT.) 


and  infections  (malaria,  tuberculosis).  An  important  group  are  due 
to  adenoid  vegetations  in  the  posterior  ])har>Tix.  Under  such  con- 
ditions of  ill  health  development  is  impaired  and  does  not  i)roceed  at 
a  normal  rate.  With  anemia,  impaired  digestion,  and  infected  tonsils 
which  produce  a  constant  toxemia,  the  child  cannot  be  expected  to 
proceed  in  his  development  with  normal  rapidity. 

In  addition  to  such  conditions  as  this  it  is  found  that  the  defect  is 
often  due  to  high  grades  of  myopia  which  make  it  impossible  for  the 
child  to  learn,  because  he  cannot  see  to  read  or  even  see  the  blackboard. 
In  the  .same  way  deafness  and  other  cjuite  gross  lesions  have  been 
found  to  account  for  many  of  these  conditions. 

In  addition  to  these  types  there  are  the  usually  milder  grades  of 
defect — infantilism — dependent  upon  the  dysfunction  of  the  various 
endocrine  glands.    Here  are  found  the  thymic  tj'pes,  status  thymico- 


984 


IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 


lymphaticus,  hyper-  and  hypothyroidism,  dysgenitalism  (hj^per- 
genitalism),  dyspituitarism  (including  dystrophia  adiposogenitalis), 
dysadrenahsm,  and  pluriglandular  imbalances. 

Chondrodystrophy  and  micromelia  may  be  associated  with  mental 
defect,  although  many  of  these  deformed  individuals  are  bright  and 
witty,  for  example  the  court  fools  of  olden  times. 

There  are  many  other  conditions  which  may  have  some  grade 
of  mental  defect  associated  such  as  cerebral  tumors,  spinal  paralysis, 
Westphal-Striimpell  pseudosclerosis,  Friedreich's  ataxia,  facial  hemi- 
dystrophy,  etc. 


Fig.  470. — In  center  a  moron,  aged  twenty-four  years;  mentally,  ten  years.  At  right, 
moron,  aged  eleven  j^ears;  mentally,  eight  years.  At  left,  imbecile,  aged  nine  years; 
mentally,  six  years. 

It  should  be  remembered,  too,  that  the  child  may  have  a  neurosis 
or  a  psychoneurosis,  and  these  conditions  may  produce  conduct  dis- 
orders which  show  a  moral  obliquity  which,  if  they  are  not  carefully 
investigated,  may  too  easily  be  classified  as  due  to  defective  conditions. 
Such  psychic  states  are  susceptible  of  psychoanalysis,  and  often  of 
cure. 

Psychopathic  Constitution.^ — There  are  many  anomalies  of  char- 
acter which,  because  normal  or  usual  to  the  individual,  cannot  be  said 
to  properly  constitute  a  psychosis,  but  because  they  lead  to  a  rather 
ineflRcient  type  of  adjustment  of  the  individual  to  his  environment, 
and  because  persons  exhibiting  these  peculiarities  often  become 
actively  disordered,  may  be  considered  as  borderland  conditions. 

Something  of   the   hysteric    and   epilejjtic   characters  has  already 


PSYCHOPATHIC  CONSTITUTION  985 

been  discussed.  The  general  t\-pe  of  inefficiency  of  the  neurasthenic 
and  the  psychasthenic  character  has  been  described,  and  attention 
has  been  called  to  the  unresistive  and  the  post-traumatic  types  with 
their  intolerance  of  alcohol  and  fever.  There  also  may  be  said  to  exist 
a  manic-depressive  type  and  lately  attention  has  been  directed  to  the 
"shut-in  '  type  as  the  t^-pe  par  excellence  found  in  those  cases  which 
develop  dementia  precox. 

Besides  these  there  are  the  "cranks,"  who  with  some  pet  scheme 
closely  approach  the  paranoiac  type  and  that  host  of  ill-balanced, 
eccentric  individuals  who  may  be  superficially  brilliant  but  lack  con- 
tinuity of  purpose  and  capacity  for  the  continuous  expenditure  of 
effort  in  any  one  direction.  Their  life,  to  use  the  well  chosen  words 
of  Regis,  is  one  "long  contradiction  between  the  apparent  wealth  of 
means  and  poverty  of  results." 

There  are  also  the  pathological  liar,  or  pseudologia  phantastica,  and 
certain  types  of  swindlers. 

Constitutional  anomalies  of  mood  are  seen,  those  who  are  always 
depressed — psychopathic  depression — and  the  opposite  state — psycho- 
pathic exaltation.  Others  never  seem  to  be  quite  able  to  successfully 
cope  with  conditions;  they  are  the  failures  of  life,  the  cases  of  constitu- 
tional inferiority. 

Conditions  oT  psychogenic  depression  occur  quite  often  in  psycho- 
pathic individuals.  ^lany  weak  characters  who  are  led  into  crime 
de\'elop  a  symptomatic  depression  when  caught  and  sentenced.  More 
pronounced  defects  of  character  are  seen  in  the  criminal  classes,  many 
of  whom  lack  the  ordinary  moral  inhibitions  and  are  properly  classed 
as  moral  imbeciles. 

There  are  many  psychogenic  states  that  occur  in  psychopathic 
individuals — deviates.  The  so-called  prison  psychoses  are  types  and 
come  about  as  the  patient's  reaction  to  the  difficulties  in  which  he 
finds  himself.  They  may  be  hysterical,  catatonic,  paranoid,  or  manic- 
depressive  in  type,  according  to  the  t^-pe  of  individual.  They  clear 
up  when  the  stress  is  removed — pardon,  expiration  or  commutation 
of  sentence,  etc. 

These  are  the  t^-pes  to  which  Siemerling  has  given  the  name  "  situa- 
tion  psychoses'  meaning  that  the  psychosis  is  a  result  of  the  situation 
in  which  the  patient  finds  himself.  The  imprisonment  or  jjerhaps 
the  death  sentence  are  intolerable  facts  which  can  neither  be  escaped 
nor  permitted  to  enter  consciousness  and  the  various  types  of  reaction 
— hysterical,  catatonic,  etc.,  are  the  methods  em])loyed  to  try  and 
square  \^-ith  reality  according  to  the  make-u])  of  the  individual.  Many 
"shell  shock"  neuroses  develop  in  this  grouj). 

This  whole  group  of  reactions  is  found  in  psychopathic  individuals, 
the  tj-pes,  of  course,  from  which  the  criminal  classes  are  recruited  and 
which  present  to  society  some  of  the  most  difficult  of  its  problems. 
The  solution  of  these  problems  will  only  be  in  sight  when  the  make-up 
of  the  individual  is  appreciated  as  a  factor. 

The  question  of  constitutional  inferiority  involves  many  live  issues. 


986  IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

This  term,  like  other  cHnical  designations,  does  not  apply  to  a  well 
defined  class,  but  in  the  main  it  may  be  said  to  cover  two  groups,  those 
that  are  inferior,  more  especially  from  the  intellectual  angle,  and  those 
that  are  inferior,  more  particularly  from  the  emotional  angle.  The 
latter  group  are  naturally  more  important,  at  least  from  a  sociological 
standpoint,  because  they  include  those  ill-balanced  individuals  who  so 
frequently  run  counter  to  the  established  order  of  things  and  therefore 
come  within  the  purview  of  the  criminal  law  for  more  or  less  serious 
offences.  A  discussion  of  this  group  is  quite  impossible  in  this  place, 
both  because  of  its  great  extent  and  its  present  lack  of  classification. 
It  includes  a  considerable  number  of  the  juvenile  delinquents,  of  the 
recidivist  type  of  criminal,  of  the  paupers  and  prostitutes,  of  the 
ne'er-do-wells,  the  black  sheep  of  the  family,  and  at  the  higher  levels 
of  erratic,  half-genius,  half-crazy  persons  with  brilliant  spots  here  and 
there,  but  without  continuity,  whose  efficiency  is  materially  impaired 
and  who  live  often  a  more  or  less  wandering  existence.  Many  of  the 
inferior  are  less  driven  by  their  emotions  and  a  great  deal  of  the 
drudgery  of  the  world's  work  is  probably  done  by  these  dull  and 
relatively  stupid  persons. 

Theoretically  these  cases  do  not  improve.  The  intellectually  inferior 
can  only  be  improved  up  to  the  point  of  their  capacity  to  learn.  The 
emotionally  uncontrolled,  however,  tend  spontaneously  to  a  certain 
degree  of  clinical  improvement  v/hen  they  have  passed  the  fourth 
decade  and  the  drive  of  the  emotions  begins  to  quiet. 

Simulation. — Simulation  is  not  an  adequate  diagnosis.  It  is  neces- 
sary to  know  what  lies  back  of  the  simulated  symptoms  and  then  it 
will  be  recognized  as  a  reaction  which  may  or  may  not  be  the  best  the 
individual  is  capable  of  under  the  circumstances.  If  it  is  the  best  then 
manifestly  we  are  dealing  with  a  defective  personality,  in  most  cases 
at  least.  If  it  is  not  the  best  then  perhaps  the  better  tendencies  can 
be  reinforced  by  encouragement  on  the  one  hand  or,  on  the  other,  by 
the  fear  of  punishment  or  by  being  made  to  actually  suffer  pain.  If 
the  punishment  method  is  used  it  should  be  intelligently  calculated  to 
effect  the  ends  sought  along  the  same  lines  that  a  conditioned  reflex  is 
produced.  If  it  is  used  as  a  species  of  self-indulgence  on  the  part  of 
those  inflicting  it  it  cannot  be  expected  to  produce  good  results. 

In  diagnosing  simulation  care  must  be  used  to  avoid  concluding 
that  because  a  symptom  is  useful  it  is  therefore  simulated.  All  symp- 
toms are  useful  and  this  criterion  would  indict  phagocytosis.  The 
diagnosis  of  malingering  should  never  be  made  except  it  is  clear  that 
the  symptom  was  consciously  and  intentionally  created  with  the  clear 
conscious  intent  to  deceive  and  even  when  such  a  diagnosis  is  made  it 
may  not  be  evident  that  the  individual  could  have  done  better.  Even 
if  it  is  evident  that  the  individual  could  have  done  better  the  object 
should  not  be  punishment  but  to  make  the  material  (individual) 
available  for  the  purposes  in  hand  if  possible — useful  as  a  soldier,  as 
a  citizen — and  to  this  end  punishment  should  only  be  considered  as  a 
means. 


ANOMALIES  OF   THE  SEXUAL  IXSTLWCT  987 

ANOMALIES  OF  THE  SEXUAL  INSTINCT. 

Quantitative  Anomalies.— These  are  frigidity  or  lack  of  desire  for 
sexual  congress — sexual  anesthesia — or  eroticism — sexual  hyperesthesia. 

Qualitative  Anomalies. — ^  These  are  inversions  and  perversions. 
Inversion  consists  of  a  lack  of  harmony  between  the  physical  and  the 
psychical  sex  and  leads  to  homose.vuaJity  or  desire  for  persons  of  the 
same  sex.  Various  physical  anomalies  are  often  found  in  these  persons. 
For  example,  the  general  conformation  of  the  body,  pilosity,  etc.,  may 
indicate  one  sex,  while  the  genitalia  are  of  the  other. 

Sex  inheritance  is  alternative.  That  is,  both  male  and  female  char- 
acters are  present  in  the  germ  and  only  one  normally  develops.  Some- 
times there  seems  to  be  an  uncertainty  as  to  which  will  develop  and 
the  result  is  a  certain  mixture  which  may  take  place  either  in  the 
bodily  or  psychic  sphere  alone  or  in  both. 

The  perversions  are  many  and  include  the  various  anomalous  means 
of  gratifying  the  sexual  appetite. 

With  respect  both  to  inversion  and  perversion  it  must  be  remem- 
bered that  in  the  young  child  the  sexual  instinct  has  not  developed  and 
later  as  it  develops  and  comes  into  prominence  it  differentiates  and 
tends  to  specialize  by  centering  its  aims  in  a  special  direction,  i.  e., 
toward  the  opposite  sex  and  normal  coitus.  The  child,  before  this 
takes  place  is,  to  use  a  term  of  Freud's  polymorphous-perverse.  He 
may  be  developed  in  any  direction  by  appropriate  influences  or  he 
may  stay  in  the  undeveloped,  infantile  stage. 

The  most  important  of  the  perversions  are: 

Masturbation. — Masturbation  is  very  frequent  among  psychopaths 
and  very  often  a  result  rather  than  a  cause  of  mental  anomalies,  though 
undoubtedly  an  important  factor  in  some  cases  of  acute  psychosis.  A 
transient  period  of  onanism  in  infancy  is  probably  normal  and  serves 
to  focalize  the  sexual  sensations  on  the  genital  zone  and  thus  establish 
the  hierarchy  of  the  erogenous  zones  with  the  primacy  of  the  genital 
zone. 

Active  Algolagnia  (Sadism).— The  gratification  of  the  sexual  feeling 
by  the  infliction  or  sight  of  pain — real  or  simulated.  In  the  latter  case 
the  sadism  is  symbolic .  As  the  male  is  normally  the  more  active  and 
aggressive  in  the  sexual  relation,  as  might  be  expected,  this  anomaly 
is  more  frequently  found  hi  men. 

Passive  Algolagnia  (Masochisvi). — ^The  gratification  of  the  sexual 
feeling  by  suffering  pain — real  or  simulated.  In  the  latter  case  it  is 
symbolic.  The  female,  being  the  more  passive  of  the  two  sexes  in  the 
sexual  relation,  so  an  exaggeration  of  this  passivity  is  more  frequently 
found  among  women. 

Homosexuality. — Sexual  desire  for  the  same  sex  which  seeks  either  to 
dominate  or  be  dominated  by  the  sexual  object  and  thus  gives  rise  to 
object  homo-erotics  and  subject  homo-erotics. 

Narcissism. — A  form  of  auto-erotic  sexuality  in  which  a  person  is  in 
love  with  himself — his  own  body  or  someone  like  himself. 


988  IDIOCY,  IMBECILITY  AND  FEEBLE-MINDEDNESS 

Fetichism. — Sexual  excitement  and  gratification  by  the  sight,  con- 
tact or  possession  of  some  object  or  part  of  the  body.  The  object 
is  usually  some  wearing  apparel,  such  as  shoes,  handkerchief,  petticoat, 
or  a  part  of  the  body  other  than  the  sexual  organs. 

Bestiality. — Sexual  relation  with  animals. 

Exhibitionism. — Sexual  gratification  by  exposing  the  genital  organs. 

Necrophilia. — The  desire  to  have  sexual  congress  with  a  dead  body. 

INIost  of  these  conditions  stand  for  what  was  normal  at  a  certain 
stage  in  development  but  should  have  been  left  behind  in  progress; 
or  else  they  are  the  result  of  aberrant  development  from  these  lower 
points  when  there  has  been  a  stagnation  of  the  developmental  process 
and  so  are  included  in  this  chapter.  The  higher  psychic  ramifications 
are  fully  discussed  in  the  chapters  on  the  neuroses,  psychoneuroses, 
epilepsy,  and  certain  psychoses,  notably  dementia  precox  and  manic- 
depressive  psychosis,  in  all  of  which  disturbances  of  psychosexual 
development  are  present. 

This  list  of  the  anomalies  of  the  sexual  instinct  as  defined  refers 
to  the  actual  expression  of  this  instinct  in  outward  activity.  All  of 
these  various  manifestations,  however,  may  appear  in  the  fantasies 
of  the  patient,  expressed  symbolically  and  without  any  appreciable 
tendency  to  carry  them  into  action.  In  fact,  the  analysis  of  practically 
any  one  would  show  a  majority  at  least  of  these  tendencies  in  s^onbolic 
expression  in  the  unconscious.  It  can  easily  be  understood  why  this 
should,  be  so,  because  such  tendencies  as  homosexuality,  narcissism 
exhibitionism,  etc.,  represent  stages  in  normal  development,  the 
records  of  which  are  preserved  in  the  unconscious.  In  their  crude 
manifestations  they  belong  to  the  symptoms  of  mental  defect,  in  their 
higher  symbolic  expressions  in  the  neuroses  and  psychoses  they  indicate 
the  tendencies  of  the  individual  which  have  not  been  adequately 
socialized. 

All  of  these  instinctive  tendencies  inay  be  adequately  sublimated 
and  socialized.  The  sadist  may  become  a  surgeon,  a  butcher,  a  military 
commander;  masochistic  types  accomplish  their  best  under  the  control, 
direction  and  domination  of  others;  homosexuality  is  sublimated  in 
very  valuable  forms  of  social  activity — such  as  welfare  work  and  ordi- 
narily gets  expression  in  social  groups  (clubs)  of  the  same  sex;  nar- 
cissism is  at  the  basis  of  personal  ideals;  exhibitionism  is  seen  socialized 
in  the  public  speaker  and  the  actor.  These  instincts  produce  patho- 
logical results  only  when  sublimation  has  failed. 


INDEX. 


Abderhalden  reaction,  156 
Abdominal  muscles,  test  of,  88 
Abducens  nerve  palsy,  601 
Abductors  of  thigh,  test  of,  89 
Aberrant  fibers,  49 

medullary,  49 
meduUopontine,  49 
pontine,  49 
proper,  49 
subthalamic,  49 
Abscess  of  brain,  629 

advance  stage  of,  631 

course  of,  631 

diagnosis  of,  632 

etiology  of,  629 

forms  of,  631  ' 

history  of,  629 

otitic,  631 

primary  stage  of,  630 

prognosis  of,  632 

remission  or  latency  stage  of, 

630 
rhinogenic,  631 
sjTnptoms  of,  630 
traumatic,  late,  631 
treatment  of,  632 
cerebellar,  600 

diffuse    purulent    labyrinthitis 
and,  345 
Accessorius,  lesions  of,  352 
chnical,  353 
treatment  of,  353 
Achilles-jerk,  92 
Achondroplasia,  282 
micromelia,  281 
Acoustic  nerve,  receptor  topography,  337 
oculorotary  pathway,  319 
paths,  central,  337,  338 
rotary  pathway,  319 
Acromegaly,  242,  243.     See  Hyperpitui- 
tarism, 
characteristic  hand  of,  245 
Acroparesthesia,  401 
Actinomycosis  of  brain,  667 
Actual  neuroses,  787 
Acute  ascending  form  of  polioencephalo- 
myeiitis,  452 
circumscribed  edema,  186 
disseminated  sclerosis,  519 
hallucinatory  confusion,  922 
poUoencephalomyelitis,  441 


Addison's  disease,  256 

Adductor  pollicis,  examination  of,  81 

Adenoids,  29 

Adenoma  in  parathyroid,  236 

sebaceum,  970 
Adenomata  of  bram,  667 
Adiadokokinesis,  97 

in  cerebellar  disorder,  587 
Adiposis  dolorosa,  279 

treatment  of,  280 
Adler,  organ  inferiority,  25 
Adrenal  content,  increased  in  exophthal- 
mic goiter,  232 
Adrenalin,  254 

effect  of,  59,  137 

of  fear  upon,  137 
Adult  diseases,  29 
Affect  activities,  122 
Agenitalism,  258,  259 
Agnosia,  653 
A*gonists,  18 
Agoraphobia,  804 
Agraphia,  367 
Albuminuric  retinitis,  296 
Alcoholic  coma,  658 
epilepsy,  934 
hallucinosis,  932 
multiple  neuritis,  404 
course  of,  405 
treatment  of,  406 
pseudoparanoia,  933 
pseudoparesis,  933 
Alcoholism,  27,  925 
chronic,  927 

constitutional  inferiority,  928 
deUrium  tremens,  929 

symptoms  of,  929 
treatment  of,  930 
dipsomania,  934 
dream  states,  934 
drunkenness,  927 
Korsakow's  psychosis,  930 

acute    hemorrhage,    polio- 
encephalitis of  Wernicke 
931 
associated    with    alcoholic 
polyneuritis,  9:^0 
with   other   disorders, 
.  930,  931 
pathology  of,  931 
symptoms  of,  931 
treatment  of,  932 
mental  changes  in,  928 

(989) 


990 


INDEX 


Alcoholism,  mental  conflicts  in,  926 

exaggerated     reaction     to 
alcohol,  926 
neurotic  or  psychotic  symptom  of, 

926 
psj'chology  of,  925 
psychoses  due  to  alcohol,  927 
role  of,  in  nervous  diseases,  30 
somatic  effects  of,  928 
as  a  symptomatic  manifestation,  27, 

926 
unconscious  homosexual  conflict  in, 
928 
Alexia,  367 
Algolagnia,  active,  987 

passive,  987 
Allo-erotism  in  epileptics,  874 
Alternating  insanity.    See  Manic-depres- 
sive psychosis. 
Alzheimer's  disease,  958 
Amaurosis.     See  Retinitis. 
Amaurotic  family  types  of  defectives,  969 
Ambitendency,  844 

Ambivalence  or  ambivalency,  18,  844, 891 
Amblyopia,  crossed,  in  hysteria,  43 
Amentia,  922 
Amnesic  aphasia,  338 
Amsden  and  Hoch,  psycliical  examina- 
tion, 114 
Amyotonia  congenita,  atrophy  and  hypo- 
tonus,  76 
hypotonus,  77 
conjuncta,  277 
Amyotrophic  lateral  sclerosis,  469 

course  and  duration  of,  473 
diagnosis  of,  473 
etiology  of,  470 
history  of,  469 
pathology  of,  470 
symptoms  of,  471 
treatment  of,  473 
Anal,  erotic.    See  Psychoanalysis. 

reflex,  significance  of,  152 
Analgesia,  test  of,  101 
Anamnesis  in  mental  examination,  110 
Anarthfia,  74 
Ancestry,  definite  nervous  disorders  in, 

25 
Anemia,  cerebral,  635 

cord,  162 
Anesthesia  of  ulnar,  a  tabetic  symptom, 

102 
Aneurism  of  basilar  artery,  669 
Aneurismal  tumors  of  brain,  668 
Angina  pectoris,  159 
Angiomata  of  brain,  667 
Angioneurotic  edema,  184 

inheritance  chart,  185 
locaUzation  of,  187-189 
mucous  membranes  in,  187 
occurrence  of,  184 
pathogenesis  of,  188 
prognosis  of,  188 
symptoms  of,  186 
transition  forms  of,  188 
treatment  of,  189 


Ankle-clonus,  92 

Ankle-joint  movements,  90 

Anosmia,  292 

Antagonistic  actions  of  sympathetic  and 

autonomic  systems,  136,  137 
Antagonists,  18 
Anxietas  presenilis,  952 
Anxiety  hysteria,  805 
neurosis,  806,  952 

accompanying  and  substituted 

symptoms  of,  811 
acquired,  812 
anxiety  attack  in,  808 
anxious  expectation  in,  807 
auditory  hyperesthesia  in,  807 
etiology  and  occurrence  of,  812 
non-sexual,  814 
sexual,  812,  813 
general  irritability  in,  807 
hereditary,  812 
localization  of,  186-188 
mucous  membranes  in,  187 
occurrence  of,  184 
paresthesias  in,  811 
pathogenesis  of,  188 
pavor  nocturnus,  809 
phobias,  810 
prognosis  of,  188 
symptoms  of,  186,  807 
syncope  in,  809 
vertigo  in,  809 
visceral  disturbances  in,  811 
Aortic  aneurism,  neuralgic  pains  and,  376 
Aphasias,  361,  656 
amnesic,  338 
areas,  360 
auditory,  366 
in  brain  tumors,  675,  677 
clinical  forms  of,  365 
history  of,  362 
in  migraine,  172 
motor,  366 

and  sensory,  366 
cortical,  366 
subcortical  or  pure,  366 
productive,  361 
receptor,  361 

regions  in  left  hemisphere,  361 
in  syphiHs,  703 
treatment  of,  367 
visual,  367 

word-bhndness,  366,  367 
word-deafness,  366 
Aphasic  disturbances,   areas  and  path- 
ways, 362 
status,  74 
Aphemia,  363,  364 
Aphonia,  336-339 
Aplasias  of  cerebellum,  591 

and  brain,  combined,  593 
and  cord,  atrophies  or,  593 
Apoplectic  attack,  638 
Apoplectiform  attacks  in  multiple  sclero- 
ses, 517,  518 
Apoplexies,  940 
aphasia,  656 


INDEX 


991 


Apoplexies,  apraxia,  653 
attack,  638 
cerebral,  633,  637 

syndrome,  anterior,  639 
middle,  641 
coma  in,  638 
diagnosis  of,  657 

alcoholic  coma,  658 

diabetic  coma,  658 

epileptic  and  sjTicopal  attacks, 

658 
hysterical  hemiplegias,  657 
ophthalmoscopic     examination 

in,  657 
paretic  apoplexy,  658 
in  terms  of  causation,  657 
uremic  coma,  658 
distribution  and  causation  of,  637 
hemianesthesia  in,  642 
hemianopsia  in,  656 
hemiplegia  in,  641 

late  treatment  of,  661 
irritative  complexes  in,  641 
meningeal,  604 
prognosis  of,  659 
thalamic  syndrome  in,  642 

affective  reactivity,  644 
chief  features,  642 
loss  Of  sensibility,  644 
treatment  of,  660 
in  attack,  660 
prophylaxis,  660 
surgical,  661 
vascular  instabihty  of  cerebral  ves- 
sels, 635 
Apraxia,  97,  103,  653 
facial,  331 

locaUzation  of,  654,  655 
Arachnoid,  diseases  of,  611 
Aran-Duchenne  atrophy  in  radicuhtis,400 
type  of  progressive  muscular  atro- 
pliies,  462 
Archaic  type  of  reaction,  892 
Areas  involved  in  lesions  of  spinal  cord, 

440 
Argyll-Robertson  phenomenon  in  tabes, 
64,  743 
pupil,  56,  138 

syndrome  other  than  syphilitic,  696 
in  syphihs,  696 
Arm,  examination  of,  80 
Arsenical  neuritis,  407 
Arsphenamine,  768 
Arteriosclerosis,  cerebral,  636 
Arteriosclerotic  brain  atrophy,  959 
psychoses,  947,  958 
diagnosis  of,  962 
late  epilepsies,  961 
pathology  of,  961 
perivascular  gliosis,  959 
senile  cortical  devastation,  959 
subcortical  encephahtis,  959 
symptoms  of,  960 
treatment  of,  964 
softening,  962,  963 
Arthritic  disturbances  in  ancestors,  27 


Arthritides,  psychogenic,  283 
Arthritis,  30 

rheumatoid,  283 
Arthropathies,  280,  745 
neurogenic,  283 
psychogenic,  283 
Articular  rheumatism,  acute,  923 
Associated  movements,  97 
Association,  free,  118,  819,  820 
Astereognosis,  103 
Asthenia,  590 
Asthma,  157 
Asymboha,  653 
Asynergia,  cerebellar,  68,  590 

test  for,  68 
Ataxia,  Friedreich's,  594 

in  lower  extremities,  test  for,  97 
in  multiple  sclerosis,  514 
tabetic,  742 
test  for,  97 
Ataxic   form   of    acute    poUoencephalo- 
myeUtis,  453 
paraplegia  types  of  combined  sclero- 
ses, 491 
Athetoid  movements,  97 
Athetosis,  982 

Atrophies,  neural,  neuritic  or  spinal  neu- 
ritic,  453,  465 
peroneal  forearm  type,  466 
tabetic  tjTie,  469 
progressive  muscular  primary,  453 
nuclear,  453 
Atrophy  of  tongue,  356 

in  occlusion  of  cerebellar  arterv, 
76 
of  upper  extremities,  77 
Atropine,  action  of,  on  pupil,  50 
Auditory  disturbances,  339 

hjT)eresthesia  an  anxiety  symptom, 

807 
nerve,  336 

test  of  cochlear  portion,  63 
of  vestibular  portion,  64 
pathways,  diseases  in,  336 
symptoms  in  multiple  scleroses,  513 
Aura  in  epilepsy.     See  Epilepsy. 
Autistic  thinking,  888 
Auto-erotic  introversion  in  neurasthenia, 

815 
Auto-erotism  in  epileptics,  874 

in  masturbation,  814 
Autonomic  division  of  vegetative  ner- 
vous system,  134 
nervous  system,  121 
and  sympathetic  systems,  antagon- 
istic actions,  13(> 
Autonomotonic  drugs,  134 
AveUis,  syndrome  of,  527,  529 
Axial  neuritis,  298 
acute,  298 
chronic,  299 
etiology  of,  300 
hereditary  forms  of,  300 
pathologv  of.  300 
scotomata.  298,  299 
visual  fields  in,  301 


992 


INDEX 


B 


Babinski  and  Nageotte  syndrome,  530 
asjTiergic  test,  67,  68 
Chaddock  modification,  93 
dysmetria,  67 
hand  sign,  572 
great-toe  extension,  573 
plantar  extension  phenomenon,  93 
reflex,  93 

thigh  sign,  93,  573 
Bab}^  mental  life  of,  773 
Barany  tests,  69,  343 
Basedow's  disease.     See  Hyperthyroid- 
ism. 
Beard's  disease.     See  Neurasthenia. 
Bechterew,  132 
Bell,  nerve  of,  427 
Bell's  palsies,  332 
Benedict  svndrome,  543,  545 
Beriberi,  402 

Bernard-Homer  syndrome,  140,  144 
Bestiahty,  988 
Bezold's  methods,  64 
Biceps,  examination  of,  80 
Binet-Simon's  tests,  50 

criticism  of,  113 
Bipolar  receptors  in  olfactory,  289 
Birth  palsy,  cerebral  tjT^es  of,  697 

Erb's,  409,  411 
Bjerrum  test,  50 
Bladder,  mechanism  of,  153 

in  multiple  sclerosis,  516 
Blindness,  mind,  307 

word,  367 
Blood,  examination  of,  trophic  changes 
in,  41 
glands,  endocrinous  glands,  122 
serum  reaction  in  tabes,  745 
sjTidromes,  196 
test  in  syphilis,  687 
Bloodvessels,  160 
Bones,  examination  of  trophic  changes 

in,  41 
Bonnier  sjTidrome,  344,  744 
Bony   hypertrophies,    causing   compres- 
sion of  cord,  485 
sensibiHty,  100 
test  of,  102 
syndrome,  195,  280 
Bourdon  test,  113 
Brachial  neuralgia,  384 

diagnosis  of,  386 
painful  points  of,  385 
symptoms  of,  385 
treatment  of,  386 
palsy,  total,  410 

traumatic,  411 
plexus  palsies,  409 

inferior,  412 
superior,  412 
plan  of,  384 

sensory  disturbances  in  seven 
cases  of  gunshot  wounds  of, 
410 
Bradycardia,  159 


Bradvlalia,  74 
Brain,  abscess  of,  624,  629 
anemia  of,  635 
arteriosclerosis  of,  636 
blood  supply  of,  633-635 
diseases  of,  624 
hyperemia  of,  635 
multiple  sarcoma  of,  676 
stem,  lesions  of,  523 
tumors  of,  665 

vascular  disturbances  of,  633 
instabiUty  of,  635 
Breathing,  innervation  of,  157 
Bromides  poisoning,  935 
Brown-Sequard  syndrome,  482 

localization  and  symptoms  of, 
483 
Budge's  symptom  complex,  58 
Bulbar  autonomic  system,  134 

form  of  acute  poUoencephalomyeli- 

tis,  452 
palsies,  chronic  progressive,  464 
region  reflex  paths,  129 
sj^mptoms  in  multiple  sclerosis,  518 
in  sjTingoencephalomyelitis,499 
syndrome,  antero-internal,  527 

retro-oUvary,  529,  530 
types    of   s^Tingoencephalomyehtis, 
501 
Bulbopontine  types  of  progressive  nuclear 
atrophies,  464 


Cachexia  thyreopriva,  205 
Caisson  disease,  475 
Calcarine  fissure,  bullet  wound  of,  52 
Calcium  therapy  in  tetany,  240 
Cannon,  studies  in  reactions  of  vegeta- 
tive    nervous     system     and     mental 
stimuM,  122 
Carbon  bisulphide  poisoning,  408 
monoxide  poisoning,  408,  936 
Carcinomata  of  brain,  666 
Cardia  innervation,  158 

nerves,  144 
Cardiovascular  symptoms  in  exophthal- 
mic goiter,  227 
Caries,  485  5 

Catalepsy,  899,  921 
Cataleptic  rigidity,  68 
Cata^tonia,  898 
Catatonic  rigidity,  892 
Central  connections  vegetative  systems, 
125 
lesions,  chart  for  differentiation  of, 
338 
Cephalogyric  fibers,  311 
Cerebellar  abscess,  600 

diffuse    purulent    labjTinthitis 
and,  345 
asthenia,  590 
asynergia,  590 
ataxia,  585,  586 
atrophies,  Marie's  hereditary,  593 


IXDEX 


993 


Cerebellar  conditions,  treatment  of,  603 
disease,  symptoms  of,  584 
disorder,  adiadokokinesis,  587 
chief  sjTidromes  of,  590 
forced  movements,  590 
nystagmus,  588 
speech  disturbances,  590 
vertigo,  588 
dysmetria,  587 
fits,  590 
gaits,  585,  586 
hypotonus,  589 

labyrintliine  disturbances  and,  diff- 
erential diagnosis  of,  340,  345 
localization,  595 

peduncle,    afferent   tracts   through, 
580 
inferior,  580,  590 
lesions,  590 
paths,  580 
middle,  581 

lesions,  591 
paths,  581 
posterior,  582 

fiber  tracts  of,  582 
superior,  581 
lesions,  591 
paths,  581 
pontine  tracts,  and  descending,  584 
signs  in  chorea  minor,  573 
symptoms,  584 
syndromes,  579 

occlusion  of  artery,  76 
posterior-inferior,  541 
tracts,  580 

afferent,  580 
efferent,  581 

of  spinal  cord,  ascending,  583 
descending,  584 
tremor,  chronic  progressive,  598 
tumors,  .595,  596,  597 
diagnosis  of,  603 
symptoms  of,  596 
vertigoes,  345 
Cerebellopontine  angle  tumors,  603 
Cerebellum,  579 

afferent  tracts  of,  581 
aplasias  of,  591 

and  brain,  combined,  593 
pure  bilateral  agenesis,  591 
unilateral  loss,  591 
bilateral  softening  of,  596 
connections  with  forebrain,  341 

spinal,  341 
cord  atrophies  or  aplasias  and,  593 
cysts  of,  595 
function  of,  579,  581 
hemorrhage  of,  599,  600 
an  important  sensorimotor  station, 

581 
lesions,  591 
olivo-ponto-cerebellar    atrophy    of, 

593 
position  of,  579 

primary  parenchymatous  degenera- 
tion of,  595 
63 


Cerebellum,  spinal  connections  of,  chief, 
341 
tumor  ^\ith  atrophy  of,  680 
unilateral  loss  of  lobe,  591 
wounds  of,  599 
Cerebral  apoplexies,  633,  637,  940 
arteriosclerosis,  636 

chief  syndromes  of,  636 
symptoms  of,  636 
form  of  poUoencephalomyelitis,  453 
hemisphere,  derivation  of,  287 
palsy,  infantile,  626,  629 
paths  of  vestibular,  344 
peduncles,  48 

fossa  complications  of,  601 
symptoms  in  multiple  sclerosis,  518 
syndrome,  anterior,  639 
syphilis,  705,  709 

atrophy  of  tongue  and,  356 
external  rectus  palsy,  53,  314 
ptosis,  310 
Cerebrospinal  fluid,  cytological  examina- 
tion of,  688 
in  diagnosis  of  syphilis,  688 
normal,  694     ■ 
in  paresis,  720 
pathological,  695 
in  poUoencephalomyelitis,  447 
meningitides,  611 
meningitis,  615 

blood  curve  in,  616 
syphilis,  trochlearis  palsy  and,  317 
Cervical  rib,  414 

double,  413 
sympathetic,  143 

oculopupiUary  fibers,  139 
tj'pe  of  sjTingoencephalomyeha,  501 
Cervico-occipital  neuralgia,  383 
Chaddock  great-toe  extensor  sign,  94 
modification  Babinski,  93 
reflex,  93 
Characterological  defect  groups,  965,  984 
Charcot  joint,  747 
Charcot-Marie-Tooth  disease,  466 
Charcot  study  in  hysteria,  788 
Chemoregulatory,  examination  of  trophic 

changes  in,  41 
Cheyne-Stokes  respiration,  158 
Chiasm,  disease  at  or  about  the,  304 
Children's  diseases  in  patient's  history, 

29 
Chloromata  of  brain,  666 
Chlorosis,  196,  261 
Cholesteomata  of  brain,  667 
Chordoma  of  base  of  brain,  667 
Chordomata  of  brain,  666,  667 
Chorea,  570 

acute,  923,  943 
chronic,  571 

psychotic  disturbances  and,  944 
degenerans  of  Brissaud,  571 
electrica  of  Bergeron  and  Henoch, 

571 
epileptica,  Dubini,  571 
Huntington's,  571,  575 

psychotic  disturbances  and,  944 


994 


INDEX 


Chorea  insaniens,  923 

psychotic  disturbances  and,  944 
minor,  570,  571 

diagnosis  of,  574 

etiology   and   pathogenesis   of, 

573 
symptoms  of,  571 
treatment  of,  574 
postapoplectica,  571 
Syderiham's,  571 
tabica,  571 
thalamica,  571 
varieties  of,  570,  571 
Choreas  of  cerebellar  origins,  571 

of   congenital  or  infantile   cerebral 

palsies,  571 
of  general  paresis,  571 
posthemiplegic,  571 
psychogenic,  571 
of  psychoses,  571 

of  superior  cerebellar  peduncles,  571 
Choreic  movements,  97 
Chromaffin  bodies,  131 

Circle  of  Wilhs,  524,  633,  634 
Circular    insanity.     See    Manic-depres- 
sive psychosis. 
Circumflex  nerve,  427 
Claudication,  intermittent,  164,  376 
Clonus,  ankle,  92 

false,  92 
Cocain,  59,  935 

Coccygeal  plexus,  neuralgias  of,  395 
Cochlear  nerve,  test  of,  63 

oculorotary  pathway,  319 
Collapse  delirium,  921 
Colliculus  inferior,  548 

superior,  546 
Color  vision,  test  of,  43,  50 
Combined  degenerations,  490 
scleroses,  490,  491 
in  paresis,  491 
senile  forms  of,  491 
spastic  ataxic  type  of,  491 
toxic  anemic  forms  of,  491 
Complex,  the,  114 
indicators,  891 
interferences,  909 
Complexes,  spUt-off,  789,  790 
Compression  of  cord,  485 

caused  by  bony  hypertrophies, 
485 
by  spinal  cord  tumor,  486 
by  tuberculosis,  485 
syndrome   of,    in   peripheral   nerve 
injury,  425 
Compulsion  neurosis,  788,  796 
ceremonials,  801 
compromise  formations,  805 

symptoms,  800 
compulsive  action,  801 

reasoning,  801 
a  defense  neurosis,  797 
distortion  through  substitution, 

803 
doubting  mania,  801,  808 


Compulsion  neurosis,expiatory  for  sexual 
aggression,  797 
failure  of  defense,  800 
mechanism  of,  796 
obsessional  manias,  805 
obsessions  of  doubt,  804 
phobias,  802,  804 
reproaches  in,  798 
return  of  repressed  material,  799 
sadistic  component,  804 
secondary  defense,  800 
symptoms  of,  804 
tics,  802 

transformation  of  reproach,  800 
unconscious  hate  in,  803 
Concussion  syndromes,  484 

in  primary  progressive  muscu- 
lar atrophies,  473 
Conflict,  776 

in  psychoneuroses,  789 
Confusion,  acute  hallucinatory,  922 
Congenital  syphilis,  757 
Conjugate  deviation,  316 
Consanguinity,  23 
Consensual  test,  56 
Constitution,  general,  31 
Constitutional  defect  states,  122 

inferiority,  985,  986 
Convergence,  paralysis  of,  320 
Conversion  of  conflict,  115 

a  hysterical  mechanism,  790 
Convulsive  movements  localized,  97 
seizures  in  dementia  precox,  893 
importance  of,  31 
types  of  reaction  in,  865 
Cord,  atrophies  or  aplasias,  cerebellum 
and,  593 
reflex  paths  of,  126 
Corpora  quadrigemina,  546,  548,  601 

syndromes  of,  546 
Corpus  restiforme,  590 
Cortex,   disease  of,  in  lesions  of  optic 
tract,  307 
organ  of,  local  attention  of,  649 
thalamus  and  functional  relations  of, 
649 
Cortical  control  of  thalamic  activity,  649 
focus  of  attention,  649,  651 
lesions,  sensory  changes  in,  646 

compass  test  in,  648 
localization  of,  647 
posture    and    passive 
movements  in,  647 
tactile,  646 
oculorotary  pathway,  318 
palsies,  329 

representation  of  retina,  294 
sensory  disturbances,  651 
stations  of  vegetative  pathways,  132 
Corticonuclear  portion  pyramidal  tract, 

48 
Corticospinal  portion  pyramidal  tract,  48 
Corticothalamic  fibers,  643 
impulses,  sensory,  648 
Cranial-cervical    portion    of    vegetative 
nervous  system,  123 


INDEX 


995 


Cranial  nerves,  285 

examination  of,  42 

nuclei  of,  525 
Cranium,  examination  of,  32 
Cretinism,  211,  212,  971 

bony  changes  in,  972 

diagnosis  of,  971 

endemic,  212,  216 

mongolism  and,  973 

sporadic,  213-214 

treatment  of,  973 
Cretinoid  degeneration,  215 
Crises.     »See  Tabes,  Hypothyroidism. 
Crural  neuralgia,  393 
Crjang  in  multiple  sclerosis,  involuntary, 

517 
Cutaneous  anesthesia,  429 

reflex  zones,  103,  104 

of  hyperalgesia,  372,  373 
Cyclothymias,  840 
Cystic  formation  in  spinal  cord,  505 
Cysticercus  of  brain,  668 
Cysts  of  cerebellum,  595 


Dalrymple  sign,  227 
Day-dreaming,  79,5 
Deaf-mutism,  360 
endemic,  219 
Deafness,  336,  360 

word,  366 
Decompression  in  brain  tumors,  682 
Deep  reflexes,  95 
sensibiUty,  105 

test  for,  105,  106,  107 
bony,  106 
Lasegue's,  107 
muscle  and  joint,  105 
nerve  trunks,  102 
Defect,  mental,  due  to  physical  ill  health, 
983 
mild  grades  of,  982 
moral,  due  to  neurosis  or  psycho- 
neurosis,  984 
muscular,  congenital  and  acquired, 

417 
states,  classification  of,  966 
etiology  of,  966 
feeble-mindedness,  967 
idiocy,  967 
idio-imbeciUty,  967 
imbecility,  967 
moral,  967 
Defectiveness,  meaning  of  term,  965 
Defectives,  amaurotic  family  types,  969 
American  Indian  types,  982,  983 
apathetic,  982 
clinical  varieties  of,  969 
cretinism,  971 
epileptic  types  of,  979 
excitable,  982 

hydrocephalic  types  of,  974,  977 
idiot-savants,  982 
infantilism,  983 


Defectives,  inflammatory  tji^es  of,  980 
juvenile  general  paresis,  981 
microcephalic  tj^jes  of,  974 
mongolism,  973 
Negroid  types  of,  982,  983 
paralj-tic  types  of,  978 

porencephalus,  979 
rhythmic  idiots,  982 
sclerotic  types  of,  969 
sensorial  types  of,  980,  981 
syphilitic  types  of,  980 
traumatic  types  of,  979 
Defense  mechanisms  of  dream,  117 
Degenerations  of  spinal  cord,  combined, 

490 
Dejerine-Sottas  type  of  muscular 

atrophy,  469 
DeUre  de  toucher,  805 
Deliria,  drug,  935 
DeUrium  acutum,  919 
collapse,  921 
fever,  919 
grave,  921 
infection,  919 
senile,  955 
tremens,  929 
Deltoid,  test  of,  80 
Dementia,  paranoides,  901 
precox,  885 

alcohoKsm  and,  913 
ambi valency  of  ideas,  819 
"April      weather"      behavior, 

889 
archaic  type  of  reaction,  892 
autistic  thinking,  888 
catalepsy,  899 
catatonia,  898 

catatonic  excitement,  898,  900 
rigidity,  892 
stupor,  898 
command  automatism,  899 
complex  indicators,  891 
congenital  defect  and,  914 
continuous  bath  in,  915 
conversion    into   bodily  symp- 
toms, 910 
convulsive  seizures,  893 
course  and  progress  of,  910 
delire  chronique,  901 
delusional  formation,  890 

system  as  compromise  and 
wish-fulfilling,  901 
as  defense,  897 
dementia  simplex,  894 
diagnosis  of,  912 
dilapidation  of  thought,  890 
disorders  of  memory,  889 

of  seasorium,  890 
displacement  and  compromise, 
896 
of  emotions,  896 
disturbances  of  orientation,  889 
echolalia,  899 
echopraxia,  899 
emotional  deterioration,  890 
dulness,  888 


996 


INDEX 


Dementia  precox,  encapsulation  of  con- 
flict, 909,  910 
etiology  of,  886 

heredity,  886 

metabolic  disturbances,887, 
893 

psychological,  887 

shocks  as  exciting  causes, 
887 

toxic,  887 
failure  of  voluntary  attention, 

889 
fetal  attitude  in  regression,  906 
flexibilitas  cerea,  899 
formes  trust  es,  894 
forms  of,  894 

hallucinatory  experiences,  890 
hebephrenia,  895 
history  of,  885 
incestuous  ideas,  916 
industrial  training  in,  915 
interrelation    between    mental 

and  physical,  904 
intrapsychic  ataxia,  888 
introversion,  888,  907 

of  libido,  983 

psychosis,  893 
lack  of  interest,  889 
latent  period,  894 
law  of  anticipation,  917 
looseness  of  train  of  thought, 

896 
malignancy  of  reaction,  911 
mannerisms,  891,  901 
mechanism  of  displacement,  889 
mental  symptoms  of,  887 
mild  abortive  forms  of,  894 
mixed  and   atypical  states  of, 
902 

with  other  psychoses,  912 
mode  of  onset  of,  894 
muscular  tension,  899 
nature  of,  903 
negativism,  891,  898 
neologisms,  891 
organic  inferiority,  893 
paranoid  forms  of,  901 
pathology  of,  903 
perseveration  of,  899 
physical  changes  in  secondary, 
908 

symptoms  of,  893,  900 
poverty  of  ideas,  896 
prophylaxis  of,  917 
psychoanalysis  of,  916 
psychological  interpretation  of, 

906 
remissions  in,  911 
residuals,  911 
saltatory  associations,  909 
schizophreiiia,  888 
shallowTiess  of  tliinking,  890 
somatic   processes   and  regres- 
sion and,  904 
splitting  of  personality,  888 
status  catatonicus,  900 


Dementia  precox,  stereotypies,  891 
stupor,  892 

suggestibiUty,  892,  899 
surface  indications  of,  889,  890 
symbolisms    of     the     conflict, 

890 
symptom  activities  in,  891 
symptomatology  at  vegetative 

level,  894 
symptoms  of,  887 
thought  deprivations,  909 
toxic,  904 
treatment  of,  914 

mental  higher  level,  915 
lower  disturbances, 
915 
physical  conditions,  914 
social  relations,  916 
visceral  disorders,  893 
waxy  flexibility,  899 
ways  of  getting  well,  904 
withdi'awal  from  reality,  889 
word  salad,  897 
Depressio  apathetica,  952 
Dermographia,  103 
Dermoids  of  brain,  637 
Development,  general,  40 

genital,  40 
Deviates,  985 
Diabetes,  hereditary,  27 

melhtus,  266,  937 
Diabetic  coma,  658 
Diadokokinesis,  68,  97 
Diaphragmatic  neuralgia,  383 
Diffuse  neuritis,  303 

Digestive  disturbances  in  exophthalmic 
goiter,  228 
tract,  innervation  of,  143 
Diphtheria,  neuritis  due  to,  408 
Diplococcus  intracellularis,  611 
Diplopia,  monocular,  54 

test  for,  54 
Dipsomania,  934 

compulsive  activity  in,  805 
epileptic,  876 

as  manic-depressive  phase,  841 
Dislocation  syndromes  in  primary  pro- 
gressive muscular  atrophies,  473 
Displacement  in  dream,  116 
of  emotion,  115 
mechanism  of,  889 
Distortion  in  dream,  116 
DistractibiUty,  831,  833 
Dorsiflectors  of  foot,  test  of,  90 
Dorsolumbar  type  of  syringoencephalo- 
myelia, 501 
Doubts.     See  Anxiety  and  Compulsion 

neurosis. 
Drawing  diagrams  in  mental  examina- 
tion, 111 
Dreams,  115 

analysis  of,  115,  822 
mechanisms  of,  116 
Drug  deliria,  935 
Drugs,  habit-forming,  936 
Drunkenness,  927 


INDEX 


99- 


Dualistic    hypothesis    of    mental    and 

physical,  904 
Dura,  inflammation  of,  608 
Diiral  disease,  604 
Dysarthrias,  75,  314 

in  cerebellar  disease,  .590  I 

Dysgenitahsm,  261 
Dysgonadal  syndrome,  262 
Dysmetria,  97,  586 

Babinski,  67 
Dyspepsia,  nervous,  148 
Dyspituitarism,  249 

syndrome  of  Renon-Delille,  250 

treatment  of,  250 
Dyssynergia  cerebellaris  progressiva,  598 
Dystonia  musculonim  deformans,  568 
Dystrophia  adiposogenitaUs,  246 
Dystrophies,    muscular.     (See    Mxiscular 
dystrophies. 

E 

Ebbinghatts's  test,  113 
Eccentric  character,  985 
Edema,   chart  of  inheritance  in  angio- 
neurotic, 185 
Education  in  mental  examination,  29 
Egocentricity  of  epileptic,  873,  878 
Eighth  nerves,  336 
test  of,  63 
Ejaculation,  156 
Elbow  or  triceps-jerk,  91 
Electrotherapy  in  neuralgias,  378 
Eleventh  nerve,  test  of,  75 
Emancipation,  786 
EmboUsm,  637 
Emotional  factors  in  disease,  123 

status  in  mental  examination,  110 
Emotions,  three  types  of  response  to,  123 
Encephalic  trunk,  48 
Encephahtic  form  of  polioencephalomye- 

litis,  453 
Encephalitis,  624 
acute,  625 

influenza,  625 
polioencephahtis  hemorrhagica 

superior,  626 
pohoencephalomyelitis,  626 
pyogenic  types,  637 
symptoms  of,  625 
diagnosis  of,  628 
etiology  of,  624 
history  of,  624 
lethargic,  625,  627 
subcortical,  959 
therapy  of,  629 
Enchondromata,  667 
Endemic  cretinism,  216 

deaf-mutism,  219 
Endocrine  status,  34,  40 

system,  examination  of,  121 
Endocrinopatliic  syndrome,  scleroderma 

as  an,  191 
Endocrinopathies,  197 
polyglandular,  200 
uniglandular,  199 


Endocrinous  chsturbances,  lanugo  hair,39 
glands,  122 

examination  of,  41 
interrelationship  of,  200 
product,  pseudoepiphysis,  222 
stigma,  pseudoepiphysis,  282 
structures  influence  on  one  another, 
201 
Endotheliomata  of  brain,  666 
Energy'  distribution  in  epilepsy,  865-869 
Enteritis,  152 
Enuresis,  29 
Eosinophilia,  196 
Epicritic  sensibility,  99,  100 
test  of,  100 
thermal,  101 
Epilepsies,  865 

anomalous   and   borderland   condi- 
tions in,  870 
aura,  the,  874 

borderland  conditions  in,  870 
of  Bratz  and  Leubuscher,  877 
classical,  872 

constitution  of,  873 
heredity  in,  872 
seizure  in,  874 

psychic  disturbances  of,875 
course  and  prognosis  of,  880 
crimes,  876 
depth  of  reaction,  879 
diagnosis  of,  881 

due  to  faulty  energy  distribution, 
■    865-869 
early,  869 
essential,  869 
genuine,  871,  872 
grand  mal,  874 
of  gross  brain  disease,  869 
Jacksonian  type,  868,  869,  880 
late,  869,  961 
law  of  avalanche,  867 
meaning  of  attack  of,  876 
nocturnal  attacks  of,  881 
paranoid  psychic  states  of,  876 
pathological  groups  of,  871 

associated  with   arrest  of 
development,  872 
arteriosclerosis,  871 
external  poisons,  871 
focal  disease,  872 
structural  changes,871 
sypliilis,  871 
petit  mal,  875 
prophylaxis  of,  881 
psychic,  875 

symptom  groups  of,  879 
of  toxic  and  infectious  origin,  879 
transitory  conditions  in  states  of  ill- 
humor,  873,  876 
treatment  of,  881 
of  attack,  881 
between  attacks,  882 

antisyphilitic,  882 
internal  secretions,882 
middle-ear  discase,882 
pharmacotherapy,  883 


998 


INDEX 


Epilepsies,    treatment   of   between    at- 
tacks, surgical,  882 
colony,  883,  884 
psychoanalysis,  883 
of  status,  882 
varieties  of  attack,  880 
continuous,  880 
isolated,  880 
myoclonus,  880 
serial,  880 

status  epilepticus,  880 
Epilepsy,  heredity  and,  26 

lesions,  867-870 
Epileptic  attacks  in  compulsion  neurosis, 
804 
in  multiple  sclerosis,  517 
automatism,  875 
confusion,  876 
constitution,  873 
delirium,  876 
dementia,  874,  880 
depression,  876 
deterioration,  879 
dipsomania,  876 
discharge,  868 
dream  states,  875 
ecstasy,  876 
equivalent,  875 
excitement,  876 
fugues,  876 
furor,  875 
scars,  871,  872 
sclerosis  of  cortex,  866 
stupor,  876 

types  of  defectives,  979 
voice  sign,  874 
Epileptics,  egocentricity  of,  873,  878 
polyvalent  sexuality  of,  873 
social  position  of,  884 
Epileptoid  types  of  reaction,  877 
Epiphysis,  122,  252 
Equilibration,  tests  for,  68,  69 
Erb's  birth  palsy,  409,  411 
Erb-Westphal  sign,  92 
Erb-Zimmerlin   type   of   muscular   dys- 
trophy, 460 
Erection,  156 

Erector  muscles,  test  of,  88 
Eroticism,  987 
Erysipelas,  923 
Erythromelalgia,  161 
course  of,  162 
symptoms  of,  162 
therapy  of,  162 
Esophagus,  147 

vagotonic  contraction  of,  146 
Ethical  questions  in  mental  examination, 

111 
Eunuchoid,  260,  263 
Eunuchs,  259 

Examination  of  endocrinous  glands,  41 
of  larynx,  73 
methods  of,  22 

mental,  110 
objective,  32 
physical,  32 


Examination,  sensorimotor,  42 
of  sensory  nervous  system,  98 
of  speech,  73 
of  taste,  73 
varieties  of,  22 
of  vegetative  system,  33,  38 
Exanthems,  923 
Exhaustion  psychoses,  918,  920 

acute  hallucinatory  confusion, 
922 
amnesic  variety,  923 
hyperesthetic       emo- 
tional state  of  men- 
tal weakness,  922 
amentia,  922 
coUapse  deUrium,  921 

acute  delirious  mania, 

921 
grave,  921 
treatment  of,  923 
Exhibitionism,  988 
Exophthalmic  goiter,  226,  229 

Bernard-Horner   syndrome   of, 

140 
course  of,  232 
diagnosis  of,  231 
etiology  of,  230 
extirpation  of,  226 
forms  of,  231 
occurrence  of,  232 
pathology  of,  230 
symptoms  of,  226 

physical,  227-230 
psychical,  230 
treatment  of,  232 
surgical,  232 
Explosive  diathesis,  941 
Extensors  of  foot,  test  of,  90 
of  knee,  test  of,  90 
of  thigh,  test  of,  89 
of  wrist,  examination  of,  80 
External  rectus  palsy  in  tabes,  320 
Extremities,  lower,  examination  of,  89 

upper,  examination  of,  77 
Extroversion  type  of  psychosis,  843 
Eye  muscle  palsies  in  multiple  sclerosis, 
512 
palsies,  55 

central  origin  and  syringomye- 
lic dissociation,  313 
reflex  paths,  57 
reflexes  in  syphilis,  696 
sympathetic,  138 
symptoms  in  exophthalmic  goiter, 

227 
trophic  ulcer  of,  323 
Eyeball  apraxias,  322 
Eyes,  test  of,  42 


Face,  anesthesia  of,  498 
Facial  fibers,  course  of,  330 

lesions,  pontine,  332 

nerve,  331 

diagram  of,  63,  333 


INDEX 


999 


Facial  nerve,  diseases  of,  329 
injuries  to,  426 
palsies,  peripheral,  332 
progressive  hemiatrophy  of,  328 
secretory  and  taste  fibers  of,  333 
test  of,  61 
palsies  in  multiple  sclerosis,  513 
palsy,  62 

peripheral,  334 
in  tabes,  744 
tics,  331 
Facies,  types  of,  40 
Fantasies,  wish-fulfilUng,  777 
Fantasy  thinking,  777 
Fasciciilation,  showTi  in  transverse  sec- 
tions of  median,  ulnar  and  musculo- 
spiral  nerves,  433 
Fatigue  neurosis,  814 
Fatty  syndrome,  279 
Febrile  psychoses,  918 
Feeble-mindedness,  965,  967 

in  congenital  syphiUs,  762 
Femoral  neuralgia,  394 
Fetal  adenoma  of  thyroid,  217 
Fetichism,  988 
Fever  deUrivun,  919 
Fibromata  of  brain,  666 
Fibrosis  in  scleroderma,  192 
Fifth  nerve,  322. 

involvement,  chief  sensory 

symptoms  of,  325 
paralysis  of,  323 
test  of,  motor,  60 
sensory,  61 
Finckh  mental  examination,  111 
Finger-finger  test,  97 
Finger-nose  test,  97 
Fingers,  test  of,  80 

Fixed  ideas.     See  Compulsion  neurosis. 
Flexner  serum,  617 
Flexors  of  fingers,  examination  of,  80 
of  knee,  test  of,  89 
of  thigh,  test  of,  89 
of  wrist,  examination  of,  80 
Flight  of  ideas,  829,  831,  832 
Flushing.    See  Psychoneuroses,  Vascular 

sjTidromes. 
Folie  de  doute,  824 
Foot-drop,  90 
Forced  movements,  590 
Forebrain,  cerebellum  connections  with, 

341 
Foreconscious,  777 
Forward  and  backward  associations  in 

mental  examination,  111 
Fossa  complications,  601 

associated  posterior,  601 
cerebral  peduncles,  601 
corpora  quadrigcmina,  601 
crossed  hemiplegia  and  abdu- 

cens  palsy,  601 
occipital  lobes,  601 
pons,  601 

tumors  of  fourth  ventricle,  601 
Four  reactions  in  syphilis,  694 
Fourth  nerve,  309 


Fourth  nerve,  palsy  of,  315 
paralysis  of,  53,  315 
test  of,  53 
Foville  syndrome,  534,  537 

syndromes,  311 
Fracture  and  dislocation  sjTidromes  in 
primary  progressive 
muscular  atrophies, 
473 
diagnosis  of,  481 
prognosis  of,  482 
s>Tnptoms  of,  474 
treatment  of,  484 
of  skull,  604 
Franz's  esthesiometer,  100 
Free  association  in  psychoanalysis,  118, 

819,  820 
Freud  repression  theory  of  hysteria,  789 
Friedmann's.  complex,  941 
Friedreich's  ataxia,  594 

disease,  595 
Frijridity,  987 
Frontal  lobe  tumors,  673 


G.MT  in  equilibration  tests,  69 

in  multiple  sclerosis,  575 
GangUa,    inconstant   locaUzation   of,   in 

head, 127 
GangUon,  cehac,  129 

Gasserian,  127,  129,  324,  328 
inferior  cervical,  128 
mesenteric,  129 
upper  cervical,  128 
Ganglionic  system  in  man,  location  of, 

125 
Gangrene  of  skin,  multiple  neuritic,  193 
sweat    secretorv    mechanisms, 
193 
Gasserian  gangUon,  127,  129,  324,  328 
Gastro-intestinal  diseases,  937 

syndromes,  145 
Geniculate  ganglion,  128 

hemorrhage  degenerations  in  optic 
radiations,  306 
hemianopsia  due  to,  304 
lesion  giving  rise  to  hemianopsia,  305 
Genital  development,  40 

glands,  examination  of,  42 
syndromes,  258 
Genito-urinary  system,  153 

autonomic  reflexes,  153 
bladder,  153 
sexual  organs,  154 
Gigantism,  242 

Glands  of  internal  secretion,  122 
non-nervous,  122 

related  to  nervous  structures,  122 
Glaucoma,  141 

Ghoma  of  temporal  lobes,  678 
Gliomata  of  brain,  665 
Gliomatous  tumor  cavity,  493 
Gliosis,  59 

Globus  pallidus,  progressive  atrophy  of, 
564 


1000 


INDEX 


Glossopalatine  nerve,  347 
Glossopharyngeal  nerve,  347 

test  of,  75 
Goiter,  212,  216 

exophthalmic,  225 

heart,  212,  232 
Gonadal  systems,  diseases  of,  258 
Gonorrhea,  30 
Gonorrheal  neuritis,  30 
Gordon  great-toe  extension  sign,  573 
Gower's  tract,  581,  595 
von  Graefe's  sign,  60,  140,  227 
Grand  mal,  874 

Graves's  disease.     See  Hyperthyroidism. 
Gubler- Weber  syndrome,  542 
Gumma  of  brain,  668,  700 


Habits,  nervous,  29,  30 

sexual,  30 
Hair,  examination,  of  trophic  changes  in, 

41 
Hallucinations  in    mental   examination, 

110 
Hand,  test  of,  80 

Head  and  neckmuscles,  nerve  supply  of,  75 
Headache  in  brain  tumors,  670 

in  migraine,  173 

in  paresis,  718 

in  syphilis,  707 

in  syphilitic  meningitis,  703 
Hearing.     See  Auditory  nerve. 
Heart,  159 

disease,  psychotic  disturbance  of, 946 

non-nervous  gland  of,  122 
Heatstroke,  623 
Hebephrenia,  895 
Heine-Medins'  disease,  626 
Hematomyeha,  59,  477 
Hemianesthesia,  642 
Hemianopsia,  304,  656 

due  to  geniculate  hemorrhage,  304 

right  homonymous,  43,  307 

test  for,  50 
Hemi  crania,  165 

Hemifacial  atrophy  syringomyelia,  328 
Hemiopic  pupils,  56 
Hemiplegia,  652 

cerebral,  Fovilles's  syndromes,' 311 

crossed,  601 

late  treatment  of,  661 
Hemorrhage  of  cerebellum,  599,  600 

cerebral,  637,  639 

extensive  ventricular,  640 

of  pia-arachnoid,  605 

supradural,  606 

traumatic  meningeal,  604 
Hemorrhages,  most  frequent  sites  for,  634 
Hemorrhagic  retinitis,  297 
Henneberg's  reflex,  61 
Hereditarj^  dominancy  of  distinct  types, 
26 

tendencies,  24 
Heredity,  24 


Hermaphroditism,  259 
Herpes  cornse,  141 

ophthalmicus,  141 
zoster,  395,  396 
Heterosexual  stage,  780 
Hip-joint  movements,  88 
History  of  family,  22,  23 
menstrual,  31 
objective,  22 
of  patient,  22,  27 
of  present  illness,  22,  31 
subjective,  31 
Hoch  and  Amsken  psychical  examina- 
tion, 114 
Holmes's  compass,  100 
Homosexual  conflict  in  alcoholism,  928 
fixation  cause  of  drug  addiction,  936 

in  paranoia,  860 
narcissistic  stage,  780 
Homosexuality,  987 
Hoover  procedure  in  chorea,  572 
Hormone,  18,  21 

Hormones  not  independent  activities,203 
products  of  endocrinous  glands,  134 
Horner's  symptom  complex,  58 

syndrome,  139 
Huntington's  chorea,  575 
course  of,  577 
etiology  of,  575 
pathology  of,  577 
pedigree  chart,  26.    See  Chorea, 

Huntington's, 
psychotic  disturbance  of,  944 
symptoms  of,  525 
therapy  of,  578 
Hutchinson  teeth,  206 
Hydrocephalic  types  of  defectiveness,  974 
Hydrocephalus,  621,  976 

causes  for  hypopituitarism,  247 
in  congenital  syphilis,  761 
due  to  tumors  of  fourth  ventricle,  601 
symptoms  of,  622 
therapy  of,  623 
Hydrotherapy  in  dementia  precox,  915 
Hyperadrenalemia,  256,  263 
Hyperalgesia,  cutaneous  reflex  zones  of, 
103,  104,  372,  373 
reflex,  103 
test  for,  101 
j  Hyperemia,  635 
I  Hyperemias,  tonic,  161 
I  Hypergenitalism,  258 
I  Hyperorchismus,  264 
Hyperosmia,  292 
Hyperovarian  signs,  262 
Hyperpituitarism,  137,  242 
acromegaly,  242 
prognosis  of,  246 
symptoms  of,  243 
Hyper thyreoses,  225 

mental  symptomatology  of,  230 
surgical  treatment  of,  232 
Hyperthyroid,  33 

Hypertrophy  of  upper  extremities,  77 
Hypoadrenalemia,  255 
Hypochondriasis.    See  Psychoneuroses. 


INDEX 


1001 


Hypogenitalism,  258 
Hypoglossal  nerve,  disease  of,  352 

test  of,  75 
Hypoglossus,  354 

cUnical,  356 
Hypomanic  facial  expression,  829 
Hypo-ovarian  signs,  262 
Hypo-ovarism,  41,  261 
Hypophysis  changes  in  cretinism,  217 

diseases  of,  241 

endocrinous  gland  of,  122 

examination  of,  41 
Hypopituitarism,  137,  246,  250 

adipose  genital  dystrophy,  251 

definition  of,  246 

dystropliia  adiposogeni talis,  246 

etiolog}'  of,  247 

pathogenesis  of,  247 

post-traumatic,  248 

symptoms  of,  248 

tapering  hand  of  adolescent,  246 
Hyporchismus,  260,  263 
Hypothalamus,  287 
Hypothyreoses,  205 
Hypothyroid  constipation,  221 

disturbance,  lanugo  hair,  220 

states,  mild  and  mixed,  219 
pathology  of,  223 

stigma,  psejido-epiphysis,  282 

type,  220 
Hypothyroidism,  208,  209,  218,  221 

therapy  for,  223 
Hypotonus  of  upper  extremities,  77 
Hysteria,  788 

amnesias,  793 

anxiety,  805 

conversion,  790,  795 

disturbances  of  motility,  792 
of  sensibility,  791 
varied  somatic,  792 

episodic^  792 

history  of,  788 

dissociation  theories  of,  788 
repression  theory  of,  789 

mechanism  of,  789 

stigmata  of,  794 

symptoms  of,  791 
visceral,  792 
Hysterical  fantasies,  795 

hemiplegias,  657 
Hysteric  grande,  788 


Idiocy,  965,  967 

Idio-imbecility,  967 

Idiot-savants,  982 

Imbecile,  976,  984 

Imbecility,  965,  967 

Impotency.     See  Psycho  neuroses. 

Impulsive.     See  Compulsion  neurosis. 

Incest  complex  in  dementia  precox,  916 

fantasies  in  psychoneuroses,  779 
Incomplete  or  minor  form  of  acute  polio- 
encephalomyehtis,  454 


Incontinence.      See  (jenito-urinary  syn- 
dromes. 
Inequality  of  pupils,  56 
Infantile  cerebral  palsy,  626,  629 

hereditary  forms  of  spinal  progres- 
sive nuclear  atrophies,  463 
myxedema,  206 
Infantilism,  261,  983 
Infection  deUrium,  919 

psychoses,  acute  articular  rheuma- 
tism, 923 
chorea,  923 
complications  of,  924 
erysipelas,  923 
exanthems,  923 
fever  delirium,  919 
infection   and  initial   delirium, 

919 
influenza,  923 
malaria,  923 
pneumonia,  924 
postfebrile  psychoses,  920 
prefebrile,  febrile  and  postfebrile 

psychoses,  818 
treatment  of,  923 
Infection-exhaustion  psychoses,  918 
Infections,  role  of,  in  mental  pathologj', 

30 
Infectious   fever   delirium,   epileptiform 
excitement,  919 
infectious  dream  state,  920 
meningitis,  615 
psychoses,  typhoid  fever,  923 
Inferior  alternate  paralysis,  534,  537 
Influenza,  923 

encephalitis,  625 
neuritis  due  to,  409 
Infundibular  syndromes,  252 
Inheritance     in     angioneurotic    edema, 

chart  of,  185 
Innervation  of  breathing,  157 
cardiac,  158 
of  digestive  tract,  143 
of  external  muscles  of  lower  extremi- 
ties, radicular  and  peripheral,  86 
of  internal  muscles  of  lower  extremi- 
ties, radicular  and  peripheral,  87 
intestinal,  147 
of  lateral  muscles,  peripheral,  83 

radicular,  82 
of  liver,  spleen,  kidney,  267 
of  mechanism  of  swaUowing,  145 
of  muscles  of  leg  and  foot,  90 
of  shoulder  girdle,  81 

joint,  81 
which  turn  the  head,  318 
pelvic,  151 

pupillary,  clinical  conditions  depen- 
dent upon,  138 
of  salivary  glands,  142 
of  stomach,  147 
of  vasomotors,  158 
Inrotation  of  thigh,  test  of,  89 
Insanity,  meaning  of  term,  965 
!  not  an  entity,  26 

1  Insolation,  943 


1002 


INDEX 


Intelligence     tests,     relative     value     of 

limited,  113 
Intention  tremor,  97 

in  multiple  sclerosis,  514 
Intercostal  neuralgias,  387 
Intermedius  nerve,  331 
Intermittent  claudication,  169 
neuralgias  and,  376 
therapy  of,  165 
Internal  secretion,  glands  of,  122 
secretions,  122,  197 

action  on  muscle  and  connective 
tissue,  202 
on  spinal  reflex  arc,  202 
on  sugar  mobilization,  202 
Internuclear   fibers,    oculorotary   paths, 

318 
Interruption  syndromes,  motor  and  sen- 
sory recovery,  424 
in  peripheral  nerve  injuries,  419 
Interstitial  bodies,  non-nervous  glands, 
122 
optic  neuritis,  visual  fields  in,  302 
peripheral  neuritis,  301 
Intestinal  innervation,  148 

worms,  29 
Intoxicants,  miscellaneous,  935 
Intoxications,  408 

Intramedullary  lesions  of  spinal  cord,  477 
Introversion,  794 
of  Ubido,  893 
psychosis,  893 
Involution  melancholia,  843,  947 

endopsychic  conflict,  949,  950 
internal  secretions  of,  949 
manic-depressive  psychosis  and, 

949 
pathology  of,  951 

central  neuritis,  951 
symptoms  of,  947 
treatment  of,  951 
lodothyrin,  thyroid  hormone,  135 
Irritation,  syndrome  of,  425 


Jackson,  syndrome  of,  528,  529 
Jacksonian  epileptic  movements,  97 

epilepsy,  868,  869,  880 
Jacobsohn's  reflex,  91 
Janet  dissociation  theories  of  hysteria, 

788 
Joint  sense,  test  of,  101 
Juvenile  paresis,  728,  731 

congenital  disorder,  759 
tabes,  748 

congenital  disorder,  759 


Kat,muc  type  of  defectiveness,  973 
Kempf,  mechanistic  classification  of  neu- 
roses and  psychoses,  784,  785,  786 
Keratitis  neuroparalytica,  141 


Kidney,  innervation  of,  267 

Kleptomania  a  compulsive  activity,  805 

Klumpke  palsy,  412 

Knee-heel  test,  97 

Knee-jerk,  89 

Knee-joints,  movements  of,  89 

Korsakow's  psychosis,  930 

Kraepelin  definition  of  paranoia,  856 

Krafft-Ebing  description  of  paranoia,  855 


Labyrinth,  destruction  of,  343 
Labyrinthine  and  cerebellar  disturbances, 
differential  diagnosis  of,  340,  345 
oculorotary  pathway,  319 
tonus,  340 
Labyrinthitis,  circumscribed,  345 
diffuse,  345 

purulent  and  cerebellar  abscess, 
345 
Lacunar  syndromes,  658,  659 
Landouzy-Dejerine  myopathy,  278 
Landry's  paralysis,  452 
Lange's  colloidal  gold  test,  696 
Lanugo  hair,  40 

hypothyroid  disturbance,  220 
Laryngeal  disorders,  350 
nerves,  diseases  of,  350 
paralyses,  organic,  72,  351 
psychogenic,  351 
Larynx,  test  of,  73 
Lasegue's  phenomenon,  390 

test,  107 
Latent  content  of  dreams,  116 
Lateral  sclerosis,  488 

congenital  type  of,  489 
diagnosis  of,  489 
forms  and  varieties  of,  489 
hereditary  familial  types  of,  489 
history  of,  488 
infantile  types  of,  489 
mixed  types  of,  489 
pathology  of,  488 
symptoms  of,  488 
treatment  of,  489 
unilateral   ascending    and    de- 
scending, types  of,  489 
Latissimus  dorsi,  test  of,  80 
Laughing  in  multiple  sclerosis,  involun- 
tary, 517 
Law  of  anticipation,  917 

of  avalanche,  867,  868 
Lead  neuritis,  407 
palsy,  407 
poisoning,  936 
Legend  abbreviations  of  medullary,  pon- 
tine, peduncular   and   midbrain    syn- 
dromes, 526 
Lemniscus,  median,  48 
Lens,  examination  of  trophic  changes,  41 
Lenticular  degeneration,  progressive,  567 
Leptomem'ngitides,  acute,  611 
causes  of,  611 
course  and  prognosis  of,  611 


INDEX 


1003 


Leptomeningitides,  acute,  cranial  nerve 
signs  of,  613 
headache  in,  612 
hyperesthesia  in,  612 
irregular,  614 
lumbar  puncture  in,  614 
paralytic  phenomena,  613 
reflexes  of,  614 
stiffness  of  neck  in,  612 
symptoms  of,  612 

mental,  612 
temperature  in,  613 
treatment  of,  617 
tuberculous  meningitis  in,  617 
Leptomeningitis,  611 
chronic,  620 

differential  diagnosis  of,  619 
epidemic   cerebrospinal   meningitis, 
617 
treatment  of,  617 
infectious,  617 

motor  irritation  or  paralytic  phe- 
nomena, 613 
nausea  and  vomiting  in,  612 
serous  meningitis,  618 

of  alcoholic  origin,  618 
syphilitic,  704,  730 
meningitis,  619 
treatment  of,  619 
types  of,  615 
Lesions,  central,  70 

of   medulla,  pons,  brain -stem   and 

midbrain,  523 
peripheral,  69 
Lethargic  encephalitis,  625,  627 
Levels  of  nervous  activity,  18 
Libido,  154 

introversion,  893 
nutritive,  778 
sexual,  778 
sublimation,  778 
Ligamentous  syndromes,  280 
Ligaments,     examination     of     trophic 

changes  in,  41 
Light  touch,  100 
Lipomata  of  brain,  667 
Lissauer  type  of  paresis,  727 
Little's  disease,  489,  608 
Liver,  innervation  of,  627 

non-nervous  gland,  122 
Locomotor  ataxia.     See  Tabes. 
Long  thoracic  nerve,  427 
Loss  of  sensation,  tests  for,  104 
Love,  infantile,  for  family,  779 
Lower  extremities,  reflexes  of,  92 

test  of,  89 
Lowi's  sign,  60,  140,  227 
Luetin  test,  696 
Lumbar  plexus,  388 

neuralgias,  387 
puncture  in  acute  leptomeningitides, 
614 
in  multiple  sclerosis,  516 
root  syndrome,  415 
Lumbo-abdominal  neuralgias,  393 
Lumbosacral  plexus  palsy,  415 


Lungs,  non-nervo\is  glands,  122 
Lymphocytes  in  cerebrospinal  fluid,  688 
Lymphocvtosis  of  cerebrospinal  fluid  in 
syphilis,  690 


M 


Magendie-Hertwig  syndrome,  591 
Magnan  description  of  paranoia,  854 
Malaria,  923 
MaUngering,  tests  for  deafness,  64 

ocular,  50 
Mammary  neuralgia, ,  387 
Mania.    See  Manic-depressive  psychosis, 
acute,  829,  833 

dehrious,  829,  834,  835,  921 
chronic,  835 
Manic-depressive  psychosis,  827 
acute  mania,  833 
ambivalency      and      ambiten- 

dency,  844 
chronic  mania,  835 
compromise  and  defense,  844 
continuous  bath  in,  849 
course  and  prognosis  of,  847 
cyclothjTnias,  840 

dysthymic  types  of,  841 
hyperthymic  types  of,  841 
paranoid  types  of,  842 
visceral    disturbances    in, 
841 
delirious  mania,  834 
acute,  835 
delusions,  834 
depressive  phase,  835 

acute  melanchoha,  836 
chronic  depression, 837 
depressive  stupor,  837 
simple  retardation,835 
differential  diagnosis  of,  847 
distractibihty,  831,  833 
emotional  exaltation,  832 
etiolog)^  of,  823 
extroverted  type  of,  843 
flight  of  ideas  in,  829,  831,  832 
hypnotics  in,  849 
hypomania,  829 
involution  melancholia  as,  843, 

949 
levelling  of  ideas,  834 
manic  phase,  829 
mixed  states,  842 
nature  of,  843 
pathology  of,  843 
periodical  types  of,  837 
physical  conditions  of,  846 
pressure  of  activity  and  speech, 

832 
psychoanalysis  of,  850 
psychomotor  activity  of,  834 
suicide,  850 
treatment  of,  848 
Manifest  content  of  dream,  116 
Marie's  hereditary  cerebellar  atrophies, 
593 


1004 


INDEX 


Masochism,  987 

Masselon,  mental  examination,  111 
MastodjTiia,  387 
Mastm-bation,  30,  987 

auto-erotic  phenomenon  of,  814 
cause  of  neurasthenia,  815 
function  of,  815 
genital,  not  only  type,  31 
Mechanistic    classification    of    neuroses 
and  psychoses  produced  by  distortion 
of  autonomic-affective  functions,  784, 
785 
Median  lemniscus,  48 

nerve,  429 
MeduUa,  48 

lesions  of,  523 
Medullary  syndromes,  524 

abbreviations  of,  526 
Melancholia,  acute,  836 
involution,  843,  947 
vera,  951 
Memory  in  mental  examination,  general 

and  special,  110,  111 
Mendel-Bechterew  reflex,  94 
MendeUan  laws,  24 
Meniere-Uke  attacks,  343 
Meniere's  syndrome,  342 

apoplectic  form,  346 
Meningeal  apoplexy,  604 

diagnosis  and  therapy  of,  607, 

608 
etiology,  pathology  and  symp- 
toms of,  604 
hemorrhage,  traumatic,  604 
Meninges,  disease  of,  604 
Meningitic  forms  of  acute  polioencephalo- 

myefitis,  454 
Meningitides,  cerebrospinal,  611 
Meningitis  of  base,  syphilitic,  700 
cerebrospinal,  epidemic,  615 
of  convexity,  syphilitic,  703 
hydrocephalus,  621 
infectious,  615 
serous,  618 

syphilitic,  619,  706,  730 
tuberculous,  617 
Meningococcus  intracellularis,  615 
Meningomyelitis,  syphiUtic,  753 
Menstrual  history,  31 
Mental  defect  as  failure  in  sublimation, 
778 
disorders  in  ancestry,  25 
examination,  110 

methods  of,  110 
status  questionnaire,  106 
symptoms  in  multiple  sclerosis,  517 
in  sjrringoencephalomyelia,  501 
Mercury  poisoning,  936 
Metabolism,  neurology  of,  121 
Metameric  system,  remnant  of,  123 
Metro-erotism  of  epileptic,  878 
Microcephalic  brain,  978 

types  of  defectives,  974,  977 
Micromelia,  281 

in  achondroplasia,  281 
Midbrain,  lesions  of,  523 


Midbrain,  synapses  of  vegetative  path- 
ways, 131 
syndromes  of,  636 
Midpeduncle  syndromes,  542 
Migraine,  abortive  attacks  of,  166 
classical,  167 
diagnosis  of,  179 
etiology  of,  166 
facioplegic,  182  •*■ 

headache  in,  173 
hemiplegic,  182 
heredity  in,  27 
history  of,  165 
motor  disturbances  in,  172 
ophthalmic,  165 
ophthalmoplegic,  182,  183 
paralytic  phenomena  in,  173 
psychic  disturbances  in,  176 
scotomata  while  reading,  171 
secretions  in,  175 
severe,  169 

symptoms  of,  cerebellar,  173 
early,  168 
sensory,  169 
treatment  of,  178 

of  attack  itself,  180 
trophic  disturbances  in,  176 
vasomotor  disturbances  in,  175 
visual  phenomena  in,  170 
Migraines,  symptomatic,  177 
Miliary  plaques  in  senile  dementia,  957 
Millard-Gubler-FoviUe    sjTidrome,    536, 

539 
Millard-Gubler  sjTidromes,  534,  537,  601 
Mobius  sign,  60 

syrnptom,  320 
Mongolism,  973 

cretinism  and,  973 
Moral  imbeciles,  985 

imbecihty,  967 
Morbid  fears.    See  Compulsion  neurosis. 
Moron,  984  . 

Morvan's  disease,  syringomyelia,  328 
Motor  disturbances  in  multiple  sclerosis, 
513 
reflex,  95 
Mott  law  of  anticipation,  917 
Movements  of  ankle-joint,  90 

of  toes  at  interphalangeal  joints,  91 
at  metatarsophalangeal  joints, 
91 
Mucous  glands,  141 

membranes,  examination  of  trophic 
changes  in,  41 
involved       in       angioneurotic 
edema,   187 
Miiller's  muscle,  138,  145 
Multiple  neuritis,  402 
alcohoUc,  404 
neurotic  gangrene  of  skin,  193 
sclerosis,  506 
age  in,  509 
apoplectiform  and  epileptiform 

attacks,  517 
ataxia  in,  514 
Babinskis  phenomenon  in,  457 


INDEX 


1005 


Multiple  sclerosis,  bladder  in,  516 
characteristic  forms  of,  517 
diagnosis  of,  519 
etiology  of,  509 
eye  muscle  palsies  in,  512 
facial,  513 
gait  in,  515 
hearing  in,  513 
history  of,  506 
intention  tremor  in,  514 
involuntary  laughing  and  cry- 
ing in,  517 
lumbar  puncture  in,  516 
motor  disturbances  of,  513,  514 
muscular  atrophies  of,  515 
non-characteristic  forms  of,  518 
pathology  and  pathogenesis  of, 

520 
pneumogastric,  513 
primarj',  508 
prognosis  of,  522 
sclerotic  patches  in,  507 
sensory  phenomena  in,  515 
sex  in,  509 
skin  reflexes  in,  515 
spastic  paresis  in,  515 
speech  disturbances  in,  513 
sjTuptoms  of,  509 
mental,  517 
ocular,  512 
olfactory,  510 
optic,  510 
trigeminus,  512 
taste  in,  513 
tendon  reflexes  in,  516 
therapy  of,  522 
vasomotor  and  trophic  signs  of, 

516 
writing  in,  514 
Muscle  and  connective  tissue,  action  of 
internal, secretions  upon,  202 
sense,  test  of,  101 
syndrome,  267 
Muscles,    anterior,    peripheral   innerva- 
tion of,  79 
radicular  innervation  of,  78 
of  arm,  84 

pronation  of,  85 
spinal  sjTiapse  segments  of,  84 
supination  of,  85 
diseases  of,  267 
examination  of  trophic  changes  in, 

41 
of  finger,  85 
of  foot,  test  of,  90 
of  hand,  85 

lateral,  peripheral  innervation  of,  83 
radicular  innervation  of,  78,  82 
of  leg  and  foot,  innervations  of,  90 
of  lower  extremities,  external,  inner- 
vations of,  88 
internal,  innervations  of,  87 
rotary,  of  head,  innervation  of,  318 
of  shoulder  girdle  and  joint,  81 
of  thigh  and  buttocks,  spinal  syn- 
apse segments,  88 


^Muscles  of  thumb,  85 
of  trunk,  test  of,  88 
of  wTist-joint,  85 
Muscular  atrophies  in  multiple  sclerosis, 
515 
in  s\Tingoencephalomyelia,  495 
dystrophies,  271,  460 
forms  of,  274 
pathology   and   pathogeny   of, 

273 
symptoms  of,  277 
treatment  of,  278 
power  of  upper  extremities,  77 
tests  of,  80 
Masculocutaneous  nerve,  429 
Musculospinal  nerve,  431 
Mutism,  catatonic,  898 
Myalgia,  375 

Myasthenia  gravis,  19,  267 
Myatonia  atrophica,  270 

congenita,  269 
Mydriasis,  134 
adrenalin,  59 
cocain,  59 
Myelitis.     See  Meningomyehtis,  Tumor 
of  spinal  cord,  Sj'philis  of  spinal  cord. 
Compression  of  cord.  Poliomyelitis. 
Myoclonus  epilepsy,  871,  880 
Myogenic,  types  of  progressive  muscular 

atrophies,  460 
•Myopathies,  271 

distal  type  of,  278 
facio-scapulo-humeral  type  of,  276 
juvenile  form  of,  276 
pseudohypertrophic  type  of,  274 
Myopathy,  Landouzy-Dejerine,  278 

pseudohypertrophic,  272-277 
Myosis,  134 
Myotonia  atrophica,  270,  271 

congenita,  269,  277 
Myxedema,  205 

diagnosis  of,  211 

failure  of  ossification  in,  211 

infantile,  206 

skin  in,  205 

symptoms  of,  205 

types  of,  210    * 


N 


Nageotte,  syndrome  of  Babinski,  530 

Nail-biting,  29 

Nails,  examination  of  trophic  changes  in, 
41 

Narcissism,  987 

Narcissistic  fixation,  cause  of  drug  addic- 
tion, 936 

Narcolepsy,  870 

Narcotics,  use  of,  30 

Neck,  75 

sympathetic,  143 

Necrophilia,  988 

Negativism,  891,  898 

Neosalvarsan,  768 

Nerve  of  Bell,  427 


1006 


INDEX 


Nerve,  facial,  331 

secretory   and   taste   fibers  of, 
333 

intermedius,  331 

supply  of  muscles  of  head  and  neck, 
75 

trunks,  test  of  sensibility  of,  102 
Nervous  disorders  in  ancestry,  26 

dyspepsia,  148 
Nervus  vestibularis,  340 
Neural,  neuritic  or  spinal  neuritic  atro- 
phies, 465 
Neuralgia,  brachial,  384 

painful  points  in,  385 

cervico-occipital,  382 

chronic  neuritic,  374 

crural,  393 

diaphragmatic  or  phrenic,  383 

femoral,  394 

intercostal,  387 

lumbo-abdominal,  393 

mammary,  387 

obturator,  394 

subacute  neuritic,  374 

testicular,  393 

trigeminal,  379 
Neiu^algias,  369 

coccj^geal  plexus,  395 

course  of,  374 

definition  of,  369 

diagnosis  of,  374 

etiology  of,  370 

lumbar  plexus,  387 

neurasthenic  pains  in,  375 

prognosis  of,  376 

pudendal  plexus,  394 

sciatic,  387 

special  locaHzed  forms,  378 

symptoms  of,  371 

treatment  of,  376 

\mdulating,  320 
Neuralgic  neurosis,  374 
Neurasthenia,  806,  814 

avto-erotic  fixation,  814 

causes  of,  30,  816 
Neurasthenic  pains,  375 
Neuritic  form  of  muscular  atrophy,  467, 

468 
Neuritides,  sciatic,  387 
Neuritis,  375,  401 

alcoholic,  405 

arsenical,  407 

axial,  298 

scotomata  in,  298,  299 
visual  fields  in,  301 

central,  951 

diffuse,  303 

due  to  diphtheria,  408 
to  influenza,  409 
to  multiple  sclerosis,  visual  field, 
303 

etiology  of,  401 

infectious  disease  types  of,  408 

interstitial  peripheral,  301 

visual  fields  in,  302 

lead,  407 


Neuritis,      miscellaneous     intoxications, 
general  toxic,  408 
multiple,  402 

peroneal-forearm  tj^^e  of,  466 
symptoms  of,  402 
tabetic,  type  of,  469 
trigeminal,  325 
Neurofibromata  of  brain,  666 
Neurological  history,  22 
Neurologj'  of  metabolism,  121 
Neuronic     mechanistic     alterations    of 
physical    structures,   interrelationship 
of  psychogenic  emotional  factors  and, 
123 
Neurons,  central  motor,  316 

central  nystagmus,  320 
conjugate  deviation,  316 
irregular  palsies,  320 
lateral  associated  palsy,  317 
paralysis   of    convergence, 

320 
verticd,l    associated    palsy, 
317 
Neuropathic  tendency  in  consanguinity 

of  relatives,  25 
Neuroses,  actual,  783,  806 

as  failure  in  sublimation,  778 
mixed,  817 
treatment  of,  818 

psychoanalysis  in,  818 
secondary,  818 
Neurosis,    a   wish-fulfiUing    mechanism, 

780 
Neurosypliilis,  683 
Neurovisceral  system,  121 
Night-blindness,  42 
Ninth  nerve,  test  of,  75 
Noci-association,  635 
Non-nervous  glands,  122 
Norme's  findings  in  syphilis,  695 
Nyctalopia,  42 
Nystagmus,  central,  320 

in  central  and  peripheral  lesions,  70 
in  cerebellar  disorder,  588 
in  mvdtiple  sclerosis,  512,  517 
rotatory,  53,  54 
test  for,  53 
undulating,  320 
vertigo  and,  342,  343 
vestibular,  588 


Obesity,  279 
Obsessional  manias,  805 
Obsessions  of  doubt,  804 
Obsessive  thinking,  797 
Obturator  neuralgia,  394 
Occipital  lobes,  601 

left  hemianopsia,  43 
Occupation   neuroses.    *See   Psychoneu- 
roses. 


toxemias,  30 
Ocular  malingering,  50 
perves,  309 


INDEX 


1007 


Ocular  nerves,  external  disturbances  of 
external,  site,  cause,  clinical  pic- 
ture of,  321 
palsies  in  tabes,  744 
paralyses,  Werner's  artificial  mem- 
ory, 54 
symptoms  in  multiple  sclerosis,  512 
Oculog>Tic  fibers,  311 
Oculomotor  nerves,  diseases  of,  309 
nuclei,  basal  projection  of,  312 
sagittal  projection  of,  312 
Oculopupillary  fibers,  cervical  sympathe- 
tic, 139 
Oculorotary  paths,  318 
acoustic,  319 
central  sensory,  319 
trigeminal,  319 
cochlear,  319 
cortical,  318 
intemuclear  fibers,  318 
labyrinthine,  319 
tactospinal,  319 
tegmentum,  318 
vestibular,  319 
visual,  319 
(Edema.     See  Edema. 
Olfactorius  neurons,  290 
Olfactory  disturbances,  general  summary 
of,  292 
paths,  291  ' 

receptors,  extent  of,  289 
symptoms  in  multiple  sclerosis,  510 
tract,  diseases  of,  287 

treatment  of,  293 
psychogenesis  of,  293 
Olivo-ponto-cerebellar  atrophy,  593 
Ophthalmic  migraine,  165 

periodic  palsies  and,  182,  183 
psychosis,  943 
Ophthalmoplegia,  57 

chronic  progressive,  465 
Ophthalmoplegias  in  encephahtis,  626 
Ophthalmoplegic  migraine,  182,  183 
Opium,  934 

Oppenheim  great-toe  extension  sign,  94, 
573 
reflex,  94 
Opponens  pollicis,  81 
Optic  agnosia,  308 

atrophy  in  tabes,  303 
nerve,  297 

changes  in  brain  tumors,  672 
in  tabes,  744 
paths  and  chief  connections  of,  295 
radiation,  degenerations  of,  due  to 

geniculate  hemorrhage,  306 
symptoms  in  multiple  sclerosis,  510 
thalamas,  position  and  relations  of, 
643 
Optical  disturbances,  summary  of,  308 
Organ  inferiority,  25 
Organic  inferiority,  893 
Orientation,  110 
Osteitis,  485 

deformans,  485 
Osteo-arthritis,  485 


Osteomalacia,  283 
Osteomata  of  brain,  687 
Osteopathies,  280 
Osteoporosis  congenita,  195 
Out  rotators  of  thigh,  test  of,  89 
Ovaries,  non-nervous  glands,  122 
Overdetermination  of  dream,  116 
Oxycephahc  skull,  978 
Oxycephaly,  283 


Pachymeningitis,  505,  608 
externa,  608 

hypertrophica  cervicalis,  757 
interna  hemorrhagica,  609 
course  of,  610 
diagnosis  of,  610 
history  of,  609 
occurrence  of,  609 
pathology  of,  609 
symptoms  of,  610 
therapy  of,  610 
simplex,  609 
syphihtic,  730 
Pain,  causes  of  reflex,  95 

test  for,  101,  105 
Paleopsychology,  774 
PaUiation  in  brain  tumors,  682 
Pallidal  system,  primary  atrophy,  564 
PalmOS,  557 
Palsies,  birth,  607 

brachial  plexus,  409 

bulbar,  chronic  jjrogressive,  464 

in  cerebral  syphilis,  708 

cortical,  329 

eye,  55,  315 

central  origin  and  syringomyelic 

dissociation,  55,  313 
chronic  progressive,  315 
isolated,  316 

muscle  in  multiple  sclerosis,  512 
in  pontine  syndrome,  498 
irregular,  320 
larj^ngeal,  352 
ocular  in  tabes,  744 
periodic,  182 
peripheral,  310,  417 

distinguished  from  nuclear,  312 
facial,  332 

war  injuries,  418 
plexus,  409 
third  nerve,  310 

and  fourth  nerve,  syphilitic 
702 
Palsy,  abdominal  nui.scle,  jioliomyelitis, 
449 
abducens,  601 
Erb's  birth,  409,  411 
external  rectus,  314,  316 

in  cerebral  syphilis,  53 
in  tabes,  320 
facial,  62 

in  tabes,  744 
fourth  nerve,  315 


1008 


INDEX 


Palsy,  infantile  cerebral,  626,  629 
inferior  root  type,  412 
Klumpke,  412 
lateral  associated,  317 
median  and  ulnar  nerve,  430 
mixed  types,  413 
peripheral  facial,  334 
pseudobulbar,  332 

from  syphiUtic  disease,  708 
sixth  nerve,  315 
superior  bracliial  plexus,  412 
third  nerve,  310 
trochlearis,  317 
unilateral,  312 

vertical  associated  lead,  317,  407 
Pancreas,  diseases  of,  266 
examination  of,  42 
non-nervous  gland,  122 
Pancreatic  infantilism,  267 

syndrome,  266 
Papillitis,  298 
Papillomacular  bundle,  297 

course  of,  300 
Paracusia,  338 
Paradoxical  reflex,  94 
Paraganglia,  130 
Paralyses,  organic  laryngeal,  72,  351 

psychogenic  laryngeal,  351 
Paralysis  agitans,  557 

attitude  in,  561 
course  and  progress  of,  563 
differential  diagnosis  of,  565 
disturbance  of  equilibrium  in, 

562 
etiology  of,  558 
history  of,  557 
juvenile,  564,  565 
pathology   and   pathogeny   of, 

565 
psychic  disturbances  in,  563 
psychotic  disturbance  in,  945 
reflexes  in,  563 
secretory,    vasomotor,    tropliic 

disturbances  in,  562 
sympathetic  or  dilator,  58,  59 
symptoms  of,  559 
sensory,  563 
therapy  of,  566 
tremor  in,  561 
of  convergence,  320 
of  external  rectus,  55,  498 
of  fifth  nerve,  323 
of  fourth  nerve,  315 
inferior  alternate,  534,  537 
of  median  nerve,  430 
of  right  spinal  accessory  nerve,  354 
of  seventh  nerve,  498 
atrophic,  55 
of  third  nerve,  314 
of  upward  eye  movement,  316 
Paralytic  types  of  defectives,  978 
Paranoia,  acquired,  855 
acute,  857 
attenuated  form  of  dementia  precox, 

901 
combinatoria,  858 


Paranoia,  delire  chronique,  854 
delirium  of  interpretation,  855 

of  revindication,  altruistic  type, 
856 
egocentric  type,  856 
delusion,  effort  at  adjustment,  858 
delusions  of  grandeur,  860 
of  jealousy,  860 
of  persecution,  860 
description  of,  854 
Kraepelin's,  856 
Ivrafft-Ebing's,  855 
Magnan's,  854 
S^rieux  and  Capgras',  855 
diagnosis  of,  861 
erotic,  855 
erotomania,  860 
expansive,  855 
fantastic,  905 
group,  852 
hallucinatoria,  855 
homosexual  fixation,  860 
interpretation  of,  857 

Freud's  conception  of,  859 
inventive,  855 
late,  855 
Mtigious,  855 

not  a  disease  entity,  853,  854 
origin  in  complexes,  858 
original,  855 

paraphrenia  confabulans,  856 
expansiva,  856 
phantastica,  856 
systematica,  856 
persecutory,  855 
projection  mechanism,  859 
psychoanalysis,  862 
querulous,  855 
reformatory,  855 
religious,  855 

suspici  jusness,  affect  of,  858 
treatment  of,  861 
Paranoid  states,  861 

types  of  manic-depressive  psychosis, 
842 
Paraphrenia,  856,  901.     See  Paranoia. 
Paraplegia.     See  Myelitis. 
Parasympathetic  system,  122 
Parasyphilitic,  disorders,  708 
Parathyreopriva,  239 
Parathyroid  pathology  in  tetany,  237 

syndrome,  234 
Parathyroids,  endocrinous  glands,  122 
ParenchjTnatous  types  of  syphilis,  708 
Paresis,  agitated  forms  of,  726 

alteration  of  emotional  activity  in, 
717 
in  reflexes  in,  720 
Ammon's  horn  in,  713 
b/ain  atrophy  in,  736 
cell  degeneration  in,  735 
cellular  changes  in,  715 
cerebrospinal  fluid  findings  in,  720 
character  alterations  in,  718 
convulsive  phenomena  in,  719 
cortex  in,  732 


INDEX 


1009 


Paresis,  cortical  changes  in,  716 
cutaneous  sensibiUtj^  in,  718 
delusions  in,  717 
dementing  form  of,  722 
depressed  form  of,  722 
disorders  in  speech  in,  718 
expansive,  723 
eye  symptoms  of,  719 
forms  of,  721 
general,  708 
groups  of,  714 

impoverishment  of  ideas  in,  716 
irregular  forms  of,  727 
Lissai^er,  727 
juvenile,  728,  731,  981 
with  Korsakow  sj-ndrome,  627,  716 
loss  of  judgment  in,  717 
motor  incoordinations  in,  718 
neurological  signs  of,  718 
remissions  in,  721 
retention   and   sensory    defects   of, 

715 
symptoms  of,  714 
taboparesis  in,  727 
vasomotor  and  trophic  disturbances 
in,  721 
Paretic  psychosis,  interpretation  of,  729 
Parkinson's  disease,  557.    See  Paralysis 

agitans. 
Parosmia,  292 
Parotid  gland,  141 
Patellar  tendon  reflex,  92 
Path  of  opposites,  844 
Pathological  drunkenness,  934 

liar,  985 
Pavor  nocturnus,  809 
Pawlow,  studies  in  reaction  of  vegetative 
nervous  system  to  mental  stimuh,  122, 
132 
Pectoral  muscles,  test  of,  80 
Pedigree  chart,,  23,  26 

of  congenital  syphilis,  28 
of  defective  inheritance,  23 
of  Huntington's  chorea,  26 
Peduncle  syndromes,  542 
Peduncular  lesions,  analysis  of  symptoms 
resulting  from,  552 
S,yndrome,  anterior,  544 
posterior,  545 
PeUagra,  938 
Pellagraphobia,  939 
Pelvic  innervation,  151 

nerve,  92 
Periodic  palsies,  182 

etiology  of,  182 

facioplegic  and  hemiplegic  types 

of,  183 
ophthalmoplegic    migraine    in, 

183 
symptoms  of,  182 
vasomotor  palsies,  184 
Peripheral  distribution,  vegetative  sys- 
tem, 125 
facial  palsies,  332,  334 
innervation  of  anterior  muscles,  79, 
83 
64 


Peripheral  lesions,  chart  of,  338 
differentiation  of,  338 
nerves,  affections  of,  369 
palsies,  417 

compression  syndrome  of,  425 
due  to  war  injuries,  418 
interruption  s\Tidrome  in,  419 
irritation  syndrome  in,  425 
restoration  syndrome  in,  425 
stages  of  recoverv'  in,  422,  423, 

424 
treatment  of,  425 
Perivascular  ghosis,  959 
Peroneal-forearm  type  of  neural  atro- 
phies, 466 
course  of,  468 
etiology  of,  466 
patholog>'  of,  468 
symptoms  of,  467 
Perseveration,  899 
Petit  mal,  875 
Pharrnacological     tests    of     vegetative 

nervous  system,  134 
Pharynx,  test  of,  72 
Phobias.     See    Anxiety     neurosis    and 

Compidsive  neurosis. 
Phrenic  neuralgias,  383 
Physical  examination,  32 

structures,  interrelationship  of,  123 
Physicochemical  activity,  19 

systems,  121 
Pia,  diseases  of,  611 
Pia-arachnoid  hemorrhage,  605 
Pilomotor  system,  194 
Pineal,  examination  of,  42 
organ,  diseases  of,  252 
syndrome,  252 
tumor,  252 
Pituitary  body,  derivation  of,  287 
gland,  241 

feeding     in      hypopituitarism. 
248 
Plantar  extension  phenomenon,  93 
flexors,  90 
reflex,  92 
Plexus,  brachial,  plan  of,  384 
lumbar,  388 
lumbosacral,  415 
palsies,  409 

course  of,  414 
diagnosis  of,  414 
I  inferior  root  type  of,  412 

mixed  types  of,  413 
treatment  of,  414 
I  pudendal,  plan  of,  389 

sacral,  plan  of,  389 
'  Pneumogastric  disturbances  in  multiple 

sclerosis,  513 
I  Pneumonia,  924 
Pohoencephalomyclitis,  441,  626 
acute,  441 

acute  stage  of,  457 
ascending  form  of,  452 
chronic  stages  of,  458 
diagnosis  of,  455 
etiology  of,  442 


1010 


INDEX 


Polioencephalomyelitis,  acute,  historical 
note  on,  441 
incomplete  or  minor  forms  of, 

454 
meningitic  form  of,  454 
polyneuritic  form  of,  454 
prognosis  of,  456 
prophylaxis  of,  456 
treatment  of,  456 
vegetative  types,  454 
atrophic  stages  in,  451 
bulbar  and  pontine  forms  of,  452 
cerebellar  forms  of,  453 
encephalitic  or  cerebral  form  of,  453 
hemorrhagica  superior,  626 
nervous  symptoms  of,  448 
pathology  of,  444 
sensory  disturbances  in,  451 
spinal  form  of,  448 
superior,  627 

Wernicke,  717 
vegetative  involvements  in,  451 
Poliomyelitis,  acute  posterior,  395 
anterior,  450 
chronic,  461 

comparative  incidence,  rates  of,  444 
contact  in,  443 

epidemic,  relative  severity,  456 
spinal  form  of  acute  polioencephalo- 
myelitis, 448 
Polymorphous  perverse,  987 
Polyneuritic  form  of  acute  poHoencephal- 

omyelitis,  454 
Polyneuritis,  402 
course  of,  404 
diagnosis  of,  404 
etiology  of,  402 
occurrence  of,  403 
psychotic  disturbance  of,  944 
symptoms  of,  403 
treatment  of,  404 
Pons,  48,  601 

hemorrhage  of,  537,  541 
lesions  of,  523,  531 
Pontine  and  cerebellar  tracts,  descending, 
584 
facial  lesions,  332 
form  of  acute  poUoencephalomyeli- 

tis,  452 
syndromes,  55 

abbreviations  of,  526 
alternate     hemianesthesia     of 

trigeminus,  326 
anterior,  534,  537 

and  posterior,  311 

internal,  533 
cephalic,  532 
eye  palsies  in,  313,  498 
middle,  543 

Millard-Gubler-Foville  type,537 
Millard-Gubler  type,  534 
posterior,  537 
posterolateral,  535 
Pontomesencephalic  forms  of  progressive 

nuclear  atrophies,  465 
Popliteal  nerve  brauch  of  sci9.tica,  434 


Popliteal    nerve,  external  and  internal, 

lesions  of,  434,  436 
Porencephalus,  979 
Postero-inferior  cerebellar  syndromes,  541 

longitudinal  fasiculus,  48 
Postfebrile  psychoses,  918,  920 
Postoperative  psychoses,  943 
Pott's  disease,  485 
Prefebrile  psychyses,  918 
Pregnancies,  history  of,  31 
Presbyophrenia,  925 
course  of,  975 
diagnosis  of,  955 
facies  of,  956 
pathology  of,  957 

similarity  of,  to  Korsakow's  psycho- 
sis, 930,  955,  956 
treatment  of,  975 
Presenile  psychoses,  947 

anxietas  prsesenilis,  952 
delusional,  951 
depressio  apathetica,  952 
involution  melancholia,  947 
melanchoha  vera,  951 
physical  changes,  952 
Prison  psychoses,  985 
Progressive  bulbar  palsies,  chronic,  464 
facial  hemiatrophy,  328 
muscular  atrophies,  459,  462 

amyotrophic  lateral  sclero- 
sis, 469 
bulbopontine  types,  464 
combined    sclerosis,    com- 
bined degeneration,  490 
compression  of  cord,  485 
fracture     and     dislocation 

syndromes,  473 
lateral  sclerosis  group,  488 
multiple  sclerosis,  506 
neural,   neurilic  or  spinal 

neuritio,  465 
pontomesencephalic  forms 

of,  465 
primary,  459 

syringoencephalorn  y  e  1  i  a, 
495 
nuclear  atrophies,  460 
primary,  462 
spinal,  460 
Pronation,  85 

Protopathic  sensibility,  99,  100,  101 
Provocative,  Wassermann,  696 
Psammomata  of  brain,  667 
Pseudobvdbar  palsy,  332 
Pseudoepiphysis,  222 

hypothyroid  stigma,  282 
Pseudohermaphroditism,  256,  259 
Pseudohypertrophic  myopathy,  272,  274 
Pseudologia  phantastica,  985 
Pseudotabetic  syndromes,  738 
Psychic  activity,  18 

disturbances  in  paralysis  agitans,563 
level,  function  of,  20 
Psychical  disturbances  in  migraine,  176 

or  symbolic  systems,  773 
Psychoanalysis,  20,  114 


INDEX 


1011 


Psychoanalysis,      analysis    of     fantasy 
formations,  822  ' 
dream  anah^sis,  821 
free  association,  820 
interference  of  repressed  complexes, 

821 
length  of  time  required  for,  1 18 
technic  of,  117,  819 
therapeutic  object  of,  119 
transference  phenomenon,  823 
Psychogenic  depression,  985 

emotional  factors  and  alterations  of 

physical  structures,  123 
maladjustment,  149 
Psychological  age,  classification  of,  967, 

968 
Psychoneuroses     and    actual     neuroses, 
787 
conflict  in,  789 
as  failure  in  sublimation,  778 
hysteria,  788 
Psychoneurosis  a  wish-fulfilling  mechan- 
ism, 780 
Psychopathic  constitution,  984 
depression,  985 
exaltation,  985 
Psychoreflexes,  133 

Psychoses  associated  with  organic  dis- 
eases, 940 
acute  chorea,  943 
chronic  chorea,  944 
heart  disease,  946 
multiple  sclerosis,  945 
paralysis  agitans,  945 
polyneuritis,  944 
as  failure  in  subhmation,  778 
prison,  985 
situation,  985 
syphilitic,  729 
Psychosexaal   development,   stages   of, 

780 
Ptosis,  57 

bilateral,  of  tabes,  58 
of  cerebral  syphilis,  310 
isolated,  314 
Pudendal  plexus,  neuralgias  of,  394 

plan  of,  389 
Pupillarj-  immobihty,  reflex  or  isolated,  58 
inequahties,  significance  for  imcon- 

scious  material,  138 
innervation,  138 
reflexes  in  tabes,  743 
Pupils,  inequality  of,  56,  320 

test  of,  54 
Pyramidal  fibers,  course  of,  330 
tract,  48,  49 

aberrant  fibers,  48 

medullary,  49 
medullopontine,  49 
pontine,  48 
proper,  49 
subthalamic,  48 
corticospinal  and  corticonuclear 

portions,  48,  49 
distm-bances  of,  in  chorea,  572 
PjTomania,  compulsive  activity,  805 


Qtjadrigeminal  body,  anterior,  546 

posterior,  548 
Questionnaire,  22,  44 
mental  status,  106 


R 


Rabies,  550 

pathology  of,  551 
symptoms  of,  550 
treatment  of,  551 
Radial  nerve,  diseases  of,  431 
symptoms  of,  432 
treatment  of,  432 
Radicxilar  innervation  of  anterior  mus- 
cles, 78 
of  lateral  aspect  of  head,  neck, 
trunk  and  upper  extremities, 
82 
Radicuhtis,  397 

acroparesthesia,  401 
diagnosis  of,  399 
sjTnptoms  of,  397 
topography  of,  in  syphilis,  398 
treatment  of,  401 
Radiculoganghonic  syndrome,  395 
Radius,  periosteal  reflex  of,  91 
Rami  communicates  albi,  162 

grisei,  126 
Raynaud's  disease,  162,  163 
symptoms  of,  164 
treatment  of,  164 
Reactions   used   in    psychophysiological 

studies,  133 
Reality  thinking,  777 
Rectum,  151 

Red  nucleus.     See  Midbrain. 
Reflex,  21,  153 
anal,  153 

arc  of  vegetative  nervous  system,  127 
bladder,  153 
genital,  153 

paths  in  bulbar  region,  129 
in  cord,  126 

in  tendon  in  multiple  sclerosis, 
516 
rectal,  153 
scrotal,  153 
uterus,  153 
zones,  cutaneous,  103 

of  hyperalgesia,  372,  373 
Reflexes,  91 

accommodation,  56 

Achilles-jerk,  92 

acute  leptomeningitides,  614 

auditory-orbiculuris,|,67 

closing  eyes  to  light,  56 

corneal,  56 

deep,  91 

elbow  or  triceps-jerk,  91 

erector  pilff,  diagnostic  importance 

of,  194 
eye  paths,  56 


1012 


INDEX 


Reflexes,  eye  paths,  in  syphilis,  696 

Jacobsohn's  radius,  91 

jaw,  sneezing,  pharyngeal,  324 

of  lower  extremities,  92 

motor,  99 

in  multiple  sclerosis,  515 

organ,  99  ■ 

in  paresis,  720 

pupillary,  56,  324 

radius  periosteal,  91 

superficial,  95 

supinator  jerk,  91 

sympathetic,  57 

in  syringoencephalomyelia,  499 

in  tabes,  pupillary,  743 
tendon,  743 

of  upper  extremities,  91 

vegetative,  99 

^vnnking,  324 
Remy  diploscope,  52 
Renon-Dehlle  sjTidrome,  250 
Repression,  781,  789 
Reproduction,  instinct  of,  154 
Respiratory  apparatus,  157 

symptoms  in  exophthalmic  goiter, 

228 
Restiform  body,  580 
Restoration     syndrome     in     peripheral 

nerve  injury,  425 
Reticular  formation,  48 
Retinal  neurones,  plan  of,  294 
Retinitis,  296 

pigmentosa,  297 
Rheumatism,  acute  articular,  923 
Rheumatoid  arthritis,  196,  283 
Rinne's  test,  64 
Romberg's  sign  in  tabes,  743 

test,  68 
Root  segment  distribution,  399 
Rubrospinal  syndrome,  312 


Sacral  autonomic  system,  134 

plexus  injury,  sensory  disturbances 
in,  owing  to  gunshot  wounds, 
417 
plan  of,  389 
portion  of  vegetative  nervous  sys- 
tem, 123 
root  syndrome,  416 
Sacrolumbar  types  of  syringoencephalo- 

myeUa,  502 
Sadism,  987 
St.  Vitus's  dance,  571 
Salivary  glands,  141 

innervation  of,  142 
Salvarsan,  768 
Sarcomata  of  brain,  666 
multiple,  676 
of  cord,  multiple,  486 
Scanning  speech,  74 
Schizophrem'a,  885,  888 
Schizophrenic  group,  885 
Schmidt,  syndrome  of,  528 


Sciatic  nerve,  434 
Sciaticas,  387 

clinical  forms  of,  391 
course  of,  391 
diagnosis  of,  391 
electrotherapy  in,  393 
etiology  of,  388 
history  of,  388 
symptoms  of,  389 
therapy  of,  392 
Scleroderma,  191 

treatment  of,  193 
Scleroses,  comh^ined,  490 
senile  forms  of,  491 
toxic  anemic  forms  of,  491 
Sclerosis,  acute  disseminated,  518,  519 
amyotrophic  lateral,  469 
of  brain,  tuberous,  970 
combined,  490 
lateral,  488 

multiple,  506.     See  Multiple  sclero- 
sis. 
primary  lateral,  757 
Sclerotic  tj^pes  of  defectives,  969 
Scotomata  in  acute  axial  neuritis,  central, 
298 
in  hearing,  337 

in  migraine  while  reading,  171 
in  multiple  sclerosis,  irregular,  511 
test  for,  50 

in  tobacco  or  alcohol  axial  neuritis, 
299 
Seasickness,  346 

Secondary  elaboration  in  dream,  116 
Secretions,  internal,  197 

in  migraine,  175 
Secretory  disturbances  in  paralysis  agi- 

tans,  562 
Segmental  spinal  sensory  areas,  98,  99, 

102 
Senile  cortical  devastation,  959 
involution,  normal,  953 
psychoses,  947,  953 

Alzheimer's  disease,  958 
intermediate  conditions,  953 
presbyophrenia,  955 
senile  deUrium,  955 
simple  senile  deterioration,  954 
Sensation,  loss  of,  104 

unit  of  psychological  experience,  774 
Sensations,  spontaneous,  104 
Sensibility,   changes  in,   after  complete 
division    of   periph- 
eral nerve,  421 
of   ulnar    nerve,  421, 
428 
deep,  105,  420 
epicritic,  100,  420 

of  glans  penis  to  measured  prick,  105 
of  nerve  trunks  to  direct  pressure, 

102 
protopathic,  101,  420 
scheme  for  testing,  103 

loss  of  sensation,  104 
i  spontaneous      sensations, 

I  104 


INDEX 


1013 


Sensibility,  testicular,  105 
Sensorial  types  of  defectives,  980,  981 
Sensorimotor  activity.  18 
examination,  42 
neurology,  285 
system,  285 

function  of,  19 
reflex  paths,  bulbar,  129 
in  cord,  126 
Sensory  dissociation  in  syringoencephalo- 
myelia, 494 
disturbances  in  gunshot  wounds  of 
brachial  plexus,  410 
in  paralysis  agitans,  563 
fibers,  three  systems  of,  419 
nervous  system,  examination  of,  98 

tests  of,  98 
oculorotary  pathway,  central,  319 
phenomena  in  multiple  sclerosis,  515 
reflex,  95 
Serieux    and     Capgras'     description    of 

paranoia,  855 
Serous  meningitis,  618 
Serratus  magnas,  examination  of,  80 
Seventh  nerve,  329 

atrophic  paralysis  of,  55 
syndromes    of,    etiology,    loca- 
tion, clinical,  335 
test  of,  61 
Sex  inheritance,  alternative,  987 
Sexual  anesthesia,  987 
hj-peresthesia,  987 
instinct,  anomalies  of,  987 
inversions,  987 
organs,  154 
perversions,  987 
Shell  shock,  806 

neuroses,  985 
Shingles,  395 
Shock,  635 
Shocks,  mental,  30 
Shoulder,  examination  of,  80 

girdle  muscles,  innervation  of,  81 
joint  muscles,  innervation  of,  81 
"Shut  in"  character,  985 
Simulation,  986 
Sinus  thrombosis,  662 
cavernous,  664 
lateral,  663 
longitudinal,  664 
middle-ear  disease  and,  663,  664 
symptoms  of,  662 
treatment  of,  664 
Sinuses,  venous,  of  head,  663 
Sixth  cranial  nerve,  309 

palsy  of,  315 
test  of,  53 
Skew  deviation,  316,  322 
Skin  disorders  as  sjTnbolic,  194 

multiple  neurotic  gangrene  of,  193 
reflexes  in  multiple  sclerosis,  515 
symptoms  in  exophthalmic  goiter, 

227 
syndromes,  191 
Skull,  fracture  of,  604 
Sleep-walking,  29 


Smell,  loss  of.  291     • 

tests  for,  29 
Softening  in  spinal  cord,  505 
Spasm  of  upper  extremities,  77 
Spasmodic  torticollis,  353 
Spastic  anemic  group,  162 

ataxia  ty\>es  of  combined  sclerosis, 

491 
paraplegia.     See  Myelitis,  Meningo- 

mj'elitis.  Spinal  cord  tumor, 
paresis  in  multiple  sclerosis,  515 
Speech  connections,  scheme  of,  359 
disorders  in  paresis,  718 
disturbances,  357 

in  cerebellar  disorder,  590 
clinical,  360 

deafness,  360 
dysarthrias,  360 
in  multiple  sclerosis,  513 
in  mental  examination,  73,  110 
Spinal  accessory  nerve,  427 
diseases  of,  352 
paralysis  of  right,  354 
test  o"f,  75 
centers  of  sweat  secretions,  193 
connections  of  cerel)ellum,  341 
cord,  areas  involved,  440 
chief  sjTnptoms  of,  441 
syndromes  of,  440 
general   diagnostic   svmlx)l  of, 

440 
lesions  of,  439 

location  of,  441 
localization    of    symptoms    at 

difi'erent  levels  of,  476 
surgical  treatment  of,  484 
sympathetic  nuclei,  125 
tumor,   causing  cord  compres- 
sion, 486 
pohomyehtis,  atrophic  stages,  451 
form  of,  448 

sen.sory  disturbances  of,  451 
vegetative  involvements  of,  451 
progressive  muscular  atrophies,  460 
Aran-Duchenne    type 

of,  462 
chronic   poUomyelitis, 

461 
infantile       hereditary' 
form  <jf,  463 
reflex  arc,  action  of  internal  secre- 
tions upon,  202 
root  segments  and  cutaneous  reflex 

zones,  103 
sensory  areas,  segmental,  98,  99,  102 
.symptoms  in  multij)le  sclerosi.s,  518 
synap^^e  segments,  arm  niiLsdes,  84 
miLscles  of  thigh  and  \m\- 
tocks,  88 
syphilis,  753 
Spirocha'ta  pallida,  685 
Spleen,  innervation  of,  267 
Spondylosis  rhizomeliquo,  485 
Spontaneous  sen.sations,  104 
Sf)oradic  cretinism,  213 
Stammering,  29 


1014 


INDEX 


Status  corporis,  105 

epilepticus,  880 

thymolymphaticus,  263 
Stellwag's  sign,  40,  227 
Stereognosis,  103 

Stomach,  inhibited  through  splanchnic 
nerve,  123 

innervation  of,  147 
secretory,  123 
tonic  motor,  123 
vagus,  123 

non-nervous  gland,  122 
Stories  in  mental  examination,  111 
StrtimpeU's  acute  encephalitis,  626 

reflex,  94 
Stuttering,  73 

Subjective  certainty  and  uncertainty,  952 
Sublimation  of  Ubido,  778 
Subungual  gland,  143 
Submaxillary  gland,  143 
Substitution  of  affect,  115 
Suicide   in   manic-depressive   psychosis, 

850 
Sunstroke,  623 
Superficial  reflexes,  91 
Supination,  85 
Supinator  longus  jerk,  91 

test  of,  80 
Suprarenal  body,  diseases  of,  253 
syndromes  of,  253 
therapy  of,  258 
Suprarenals,  endocrinous  glands,  122 

examination  of,  42 
Suprathalamic  pathways,  646 
SwaUowing  mechanism,  innervation  of, 

145 
Sweat  secretions  of  spinal  centers,  193 

secretory  mechanisms  of,  193 
Swindlers,  985 
Sydenham's  chorea,  571 

psychotic  disturbance,  943 
Symbol,  20,  21 

ambivalency  of,  776 

carrier  or  wishes,  115 
Symbohc  level,  disorders  at,  787 

systems,  773 
Symbolism,  115,  781 

in  dreams,  116 
Symbohsms  of  conflict,  781 
Sympathetic    and    autonomic    system, 
antagonistic  actions  of,  121 

division,  134 

nervous  system,  121 

nuclei  of  spinal  cord,  125 

paralysis,  139 

portion  of  vegetative  nervous  sys- 
tem, 134 

system  reflex  paths,  bulbar,  129 
Sympathicotonia,  38 
Sympathicotonic  activity,  tests  of,  139 

constitution,  38 

drugs,  134 

reaction,  33 
Sympathicotropic     substances,     experi- 
ments with,  59 
Symptom  activities,  891 


Symptomatic  migraines,  177 
Synapses,  central  or  spinal,  126 

inconstant  localization  of  distortion 

in  head,  128 
midbrain,  of  vegetative  pathways, 
131 
Syncope  in  anxiety  neurosis,  809 
Syphilis,  additional  tests,  eye  reflexes,696 
Lange's  coUoidal  gold  test, 

696 
luetin,  696 

provocative    Wassermann, 
696 
biological  or  serological  test  of  blood, 
687,  696 
of  cerebrospinal  fluid, 
687,  694 
cerebral,  705,  709 

abnormal  sleepiness  in,  707 

atrophy-  of  tongue  in,  356 

dizziness  in,  707 

external  rectus  palsy  in,  314 

headache  in,  707 

insomnia  in,  707 

psychical  distiu-bances  in,  707 

symptoms  of,  705 

local,  708 
vascular  types  of,  705 
cerebrospinal  fluid  changes  in,  689 

trochlearis  palsy,  317 
chemical  examination  in,  694 
cUnical  forms  of,  698 
congenital  or  hereditary,  757 
effect  of,  757 
feeble-mindedness  in,  762 
geneology  of,  758,  759 
hydrocephalus  in,  761 
pedigree  charts  in,  28 
of  cranial  bones,  699 
diagnosis  of,  686 
first  rank  in  adult  disease,  29 
heredity  in,  27 
history  of,  684 
intermediary  forms  of,  684 
laboratory  findings  in,  694 
meningitis  of  base,  700 
of  convexity,  703 
of  nervous  system,  683 
Nonne's   findings  in   cerebrospinal, 
695 
in  paresis  or  taboparesis, 

695 
in   tabes   without   paresis, 
695 
parenchymatous    types    of    general 

paresis  in,  708 
search  for  organisms  in,  686 
spinal,  753 
treatment  of,  763 

injections  in,  765 

general  scheme  of,  766 

of  mercury,  765 

of  newer  arsenic  products, 

768 
of  salvarsan  and  neosalvar- 
san,  768 


INDEX 


1015 


Syphilis,  treatment  of,  injections  in,  of 
solutions,  767 
intensive,  plan  of,  772 
inunction  in,  764 
iodides  in,  771 
mercury  by  mouth  in,  771 
oleate  of  mercury  in,  764 
unguentum  hydrargyri  in,  765 
with  third  and  fourth  nerve  palsies, 
702 
SyphiUtic  leptomeningitis,  704,  730 
meningitis,  619,  630,  706 

causes  of  interstitial  peripheral 

neuritis,  301 
chiasm  changes  in,  305 
peripheral  palsies  in,  310 
meningomyelitis,  753 

hypertrophic    cervical    pachy- 
meningitis, 757 
meningeal  syndromes  in,  754 
myelitic  syndromes  in,  756 
primary  lateral  sclerosis,  757 
root  sjudromes  in,  755 
symptoms  of,  753 
system  sjTidromes  in,  756 
pachymeningitis,  730 
psychoses,  729 
forms  of,  729 

epileptic,  733 
"hereditary    luetic    mental 

disturbances,  733 
mental    disorder    due    to 
syphilis     as    a 
psychical    neu- 
rasthenia,    729 
as  a  psychic  trau- 
ma, 733 
paranoid,    combined   with 
tabes,  733 
•with     tabetic     symp- 
toms, 733 
pseudoparesis,  731 
psychotic  disturbances  as- 
sociated with  syphiUtic 
cardiac  disease,  733 
resembling    manic-depres- 
sive psychosis,  733 
short    hallucinatory    con- 

fvised  states,  733 
simple  luetic  weakness  of 
mind,  731 
retinitis,  297 
symptoms,  29 
types  of  defectives,  980 
vascular  diseases,  704 
Syphilomata  of  brain,  667 
Syrmgobulbia,  499,  501 
SyringoencephalomyeUa,  492 
bulbar  types  of,  501 
cervical  types  of,  501 
characteristic  groupings  of,  502 
course  and  progress  of,  501 
differential  diagnosis  of,  502 
dorsolumbar  type  of,  501 
etiology,  492 
history  of,  492 


Syringoencephalomyelia,  muscular  atro- 
phies and  other  trophic  changes 
in,  495 
pathogeny  of,  504 
pathology  of,  502 
reflexes  of,  499 
sacrolumbar  types  of,  502 
sensory  dissociation  of,  494 
symptoms  of,  494 
bulbar,  499 
mental,  501 
.  visceral,  499 
treatment  of,  504 
trophic  disturbances  of,  497 
Syringomyelia,  328,  492 

hemifacial  atrophy,  328 
Morvan's  disease,  328 
schematic  representation  of,  503 
SyringomyeUas,  501 
Syringomyelic  cavity,  493 


Tabes,  734 

ataxia  in,  742 

gait  disturbances  in,  742 

bilateral  ptosis  in,  58 

blood  serum  and  cerebrospinal  fluid 
in,  745 

course  and  variation  of,  748 

cranial  nerve  involvement  in,  743 
acoustic,  744 
Argyll-Robertson  phe- 
nomenon, 743 
facial  palsy,  744 
ocular  palsies,  744 
optic   nerve   changes, 

744 
trigeminus,  744 
vagus  and  accessory, 
744 

crises  of,  739 

diagnosis  of,  749 

dorsalis  and  neuralgic  pains  in,  374, 
376 

etiology  and  occurrence  of,  737 

external  rectus  palsy  in,  320 

forms  of,  748 

liistology  of,  750 

history  of,  734 

juvenile,  748 

late,  748 

lesions,  location  in,  752 

muscular  atrophies  in,  745 

optic  atrophy  in,  303 

pains  in,  739 

pathogenesis  of,  750,  753 

pathology  of,  750 

pupillan,'  reflexes  in,  743 

rapid,  749 

Romberg's  sign  in,  743 

sensory  involvement  in,  740 
loss  in,  radicular,  755 

slow,  749 

stationary  or  benign,  749 


1016 


INDEX 


Tabes,  symptoms  of,  738 

tendon  reflexes  in,  743 

trophic,  745 

vegetative  system  in,  753 

visceral,  745 

visual  field,  303 
Tabetic  arthropathies,  746 

cord,  737 

changes  in,  751 

tjrpe  of  neural  atrophies,  469 
Taboparesis,  727 
Taste  apparatus,  disorders  of,  347 

fibers,  pathways,  355 

test  of,  73 
Tay-Sachs  disease,  969 
Tear  glands,  142 

Tectospinal  oculorotary  pathway,  319 
Teeth,  examination  of  trophic  changes 

in,  41 
Tegmentum,  48 

medullary,  pontine,  peduncular  por- 
tions, 318 
Tendon  reflexes  in  multiple  sclerosis,  516 

in  tabes,  743 
Tenth  nerve,  test  of,  75 
Teratomata  of  brain,  687 
Testicles,  non-nervous  glands,  122 
Testicular  neuralgia,  393 
Tetanic  spasm,  238 

produced,  239 
Tetanus,  551 
Tetany,  238 

course  of,  237 

diagnosis  of,  239 

etiology  of,  236 

incidence  of,  235 

pathology  of,  237 

prognosis  of,  239 

strumipriva,  239 

symptoms  of,  235,  237 

treatment  of,  240 
Thalamic  syndrome,  642,  652 

sensory  disturbance  in,  650 
Thalamus,  48 

disease  of,  305 
Thermal  sensation,  test  for,  98 
Third  nerve,  309 

palsies,  310 

chronic  progressive  eye,  315 
syndromes,  312 
paralysis,  314 
test  of,  53 
Thomsen's  disease,  269 
Thoracic  lumbar  portion  of  the  vegeta- 
tive nervous  system,  123 

nerve,  long,  427 
Thrombosis,  637 
Thrombotic  softening,  962 
Thumb-sucking,  29 
Thymus,  263 

examination  of,  41 

non-nervous  glands,  122 
Thyreoses,  204 

etiology  and  pathology  of,  230 

forms  and  diagnosis  of,  231 
Thyroid,  204 


Thyroid,  examination  of,  41 

product,  pseudoepiphysis,  222 
states,  mild  toxic,  226 
therapy,  223 

effects  of,  on  skeleton,  208,  209 
in  infantile  myxedema,  207 
in  sporadic  cretinism,  214,  215 
in  tetany,  240 
Thyroids,  endocrinous  glands,  122 
Tic  douloureux,  325,  379 
Tics,  97 

in  compulsion  neurosis,  802 
cortical  and  psychogenic,  353 
facial  psychogenic,  33 1 
Tinnitus,  64,  336 
Toe  extension,  93 

movements,  91 
Tongue,  atrophy  of,  356 
Tonic  hyperemias,  161 
Torticollis,  spasmodic,  353 
Touch,  Ught,  100 
Toxemias,  122 

occupation,  30 
Toxic  psychoses,  925 

alcoholism,  925.     See  Alcohol- 
ism, 
bromides,  935 
carbon  monoxide,  936 
cocaine,  935 
diabetes  meUitus,  937 
gastro-intestinal  diseases,  937 
general  considerations  of,  936 
lead,  936 
mercury,  936 

miscellaneous  intoxicants,  935 
opium,  934 
pellagra,  938 
uremia,  937 
Transference  in  psychoanalysis,  823 

a  universal  phenomenon,  825 
Trapezius,  test  of,  80 
Traumatic  types  of  defectives,  979 
Traumatism,  940 

effects  of,  on  nervous  system,  942 
insulation,  943 
ophthalmic,  943 
postoperative,  943 
post-traumatic  constitution,  942 
psychoses,  941 

direct    post-traumatic    deliria, 
942 
shell  shock  cases,  943 
trauma  contributing  factor,  943 
traumatic  defect  conditions,  943 

from  injuries  not  di- 
rectly affecting  the 
head,  943 
Tremor  in  exophthalmic  goiter,  228 

in  paralysis  agitans,  561 
Tremors,  97 

locomotor,  97 
static,  97 
of  tongue,  73 
Treponema  palUda,  685,  709,  712 
in  ovum,  760 
in  tabes,  737 


INDEX 


loi: 


Triceps,  test  of,  80 
Triceps-jerk,  91 

Trident  hand,  achondroplasia,  282 
Trigeminal  disturbances,  etiology,  locali- 
zations, clinical,  327 
herpes  zoster,  328 
nerve,  322 

diseases  of  motor  part,  322 

of  sensorj'  part,  324 
trophic  disturbances  of,  328 
neuralgia,  379 
nevu-itis,  325 

oculorotary  pathway,  central,  319 
Trigeminus,  60 

alternate  hemianesthesia,  326 
central  paths  of  motor,  330 
motor  test  of,  60 
nuclear  disease  of,  324 
pathways,  intracerebral,  325 
sensory  test,  61 

symptoms  in  multiple  sclerosis,  512 
Trochlearis  palsy,  317 
Trophic  changes  in  blood,  41 
chemoregulatory,  41 
in  dermatoses,  191 
in  hair  and  nails,  41 
in  lens,  41 

in  ligaments,  muscles,  bones,  41 
in  mucous  membranes,  41 
in  skin,  40 

in  sjTingoencephalomyeha,  497 
in  teeth,  41 
disturbances  in  migraine,  176 
in  paralysis  agitans,  562 
test  for,  103 
in  thyroid,  test  for,  41 
signs  in  multiple  sclerosis,  516 
Tropisms,  20,  21 
Trousseau's  points,  371,  380 
Trunk  lesions,  438 

muscles,  test  of,  88 
Tuberculomata  of  brain,  667 
Tuberculosis,  causing  cord  compression, 
485 
heredity  of,  27 
Tuberculous  meningitis,  617 
Tuberous  sclerosis  of  brain,  970 
Tumors  of  brain,  665 

actinomycosis,  667 
aneurismal,  668 
carcinomata,  667 
cardiac  and  respiratory*  signs  of, 

671 
central  convolutions,  675 
chloromata,  666 
chordoma  of  base,  667 
chordomata,  666 
corpus  callosum,  680 
cysticercus,  668 
diagnosis  of,  681 
endothehomata,  666 
etiology  of,  665 
fibromata,  666 
frontal  lobe,  673 
gliomata,  665 
gumma,  667,  668 


Tumors  of  brain,  headache  in,  670 
infectious,  667 

tuberculomata,  667 
meningeal,  679 
mental  signs  in,  671 

localizing  value  of,  674 
metabolic  disturbances  in,  671 
motor  phenomena  in,  673 
nausea,  vomiting  and  dizziness 

in,  670 
neurofibromata,  666 
occipital  lobe,  679 
optic  nerve  changes  in,  672 

thalamus  in,  681 
parasitic  cystic,  667 
parietal  lobes,  677 
prognosis  of,  681 
sarcomata,  666 
symptoms  of,  669 

focal  or  local,  673. 
general,  669 
syphilomata,  667 
temporal  lobes,  677,  678 
treatment  of,  682 
true,  665 
cerebellar,  595,  596,  597 
cerebellopontine  angle,  603 
cerebral  inequality  of  pupils  in,  56, 

320 
of  fourth  ventricle,  601 
of  hypophysis,  250 
of  kidney,  970 
of  pineal,  251 

with  atrophy  of  cerebellum,  680 
Turmschadel,  283 
Twelfth  nerve,  354 
test  of,  75 


Ulnar  nerve,  427 
Unconscious,  20,  774 

material  from  repression,  777 

methods,  115 
Upper  extremities,  examination  of,  77 
Uremia,  937 
Uremic  coma,  658 
Uterus,  non-nerv'ous  gland,  122 


Vagoparalytic  drugs,  134 
Vagospastic  drugs,  134 
Vagotonia,  38 
Vagotonic  constitution,  38 

contraction  of  esojihagus,  146 
drugs,  134,  135 
reaction,  33,  140 
Vagus  nerve,  130 

chief  representative  of  autono- 
mic system,  134 
structure  of,  146 
test  of,  75 
Valleix's  points,  371,  380 


1018 


INDEX 


Van  Prey's  hairs,  100 
Vascular  apparatus,  159 

disturbances  of  brain  disease,  633 
instability  of  cerebral  vessels,  635 
types  of  syphilis,  705 
Vasomotor    disturbances    in    paralysis 
agitans,  562 
test  for,  103 
irritability  group,  184 
neuroses,  19,  160 
signs  in  multiple  sclerosis,  516 
Vasomotors,  innervation  of,  158 
Vasospastic  drugs,  134 
Vegetative  activity,  18 

cell-columns,  characteristics  of,  127 
lateral,  125 
medial,  126 
nervous  system,  19 

central     connections     and 
peripheral    distribution, 
125 
cortical  representation  of, 
132 
Bechterew,  132 
Pawlow  and  Miil- 
ler,  133 
and  cutaneous  reflex  zones, 

103,  104 
distribution    of    divisions, 

135 
examination  of,  38 

circulatory  signs,  39 
cranial  autonomic 

signs,  39 
cutaneous  signs,  39 
digestive  signs,  39 
genito-urinary     signs, 

39 
metaboUc  signs,  39 
respiratory  signs,  39 
fimction  of,  19 
reaction  to  mental  stimuU, 

122 
receptors,  123 
reflex  paths  in  cord,  126 
relation  to  edemas,  189 
relationship   to  affectivity 
and      ambivalency, 
137 
to   anaphylactic   phe- 
nomena, 188 
with  cortex,  123 
remnant  of  gangMonic    or 

metameric  system,  123 
in  tuberculosis,  157 
neurology,  19,  121 
organs  of  eye,  139 
pathway,  cortical  stations  of,  132 
skin  syndromes,  191 
Ventricle,  tumors  of  foiu"th,  601 
Vertical  associated  palsy,  317 
Vertigo,  67 

in  anxiety  neurosis,  809 
in  cerebellar  disorder,  588 
Vertigoes,  vestibular,  342 
Vestibular,  cerebral  paths  of,  71,  344 


Vestibular,      disturbances    in    multiple 
sclerosis,  513 
nerve,  339 

test  of,  63 
nystagmus,  388 
oculorotary  pathway,  319 
pathways,  diseases  of,  336 
vertigoes,  342 

Bonnier's  syndrome,  344 
treatment  of,  346 

of  seasickness,  346 
ViriUsm,  suprarenal,  257 
Viscera,  cortical  areas  for,  124 

of  pelvis,  innervation  of,  123 
Visceral  neurology,  19,  121 
Viscosity  distm-bance,  633 
Vision,  color,  42 

binocular,  proved,  51 
distiirbance  of,  by  bullet  wound, 

52,  307 
field  of,  test  of,  43 
Visual  apparatus,  diseases  of,  293 
field  in  tabes,  303 

with  diffuse  neuritis,  303 
fields  in  acromegaly,  247 
in  axial  neuritis,  301 
in  interstitial  peripheral  optic 

neuritis,  302 
oculorotary   pathway,  319 


W 


Wahnsinn,  depressive,  951 
War  injiuies,  peripheral  nerve  palsies,  418 
Wassermann  reaction,  687,  694 
in  tabes,  745 
reactions,  congenital,  27 
test,  importance  of  technic  in,  30 
Weber  compass,  100 
Weber-Gubler  syndrome,  601 
Weber's  syndrome,  542,  544 

test,  65 
Werner's  "artificial  memory,"  54 
Wernicke's  hemianopic  pupils,  56 

pohoencephaUtis,  627,  716 
WiUis,  circle  of,  524,  633,  634 
Wish,  unit  of  psychological  experience, 

774 
Word  bhndness,  367 
cortical,  367 
subcortical  or  pure,  367 
deafness,  366 
Wovmds  of  the  cerebellum,  399 
Writing  disturbances,  367 

in  multiple  sclerosis,  514 


Ziehen,  mental  examination.  111 
Zona,  395 
Zoster,  dorsal,  397 

ophthalmic,  397 

symptomatic,  395 


DATE  DUE 

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